Cram The Pance - S1E40 50 High Yield Hematology Questions

Episode Date: November 16, 2021

50 High Yield Hematology Questions to help you prepare for your Pance, Panre, and Eor's.►Paypal Donation Link: https://bit.ly/3dxmTql (Thank you!)--- Support this podcast: https://anchor.fm/scott--shapiro/supportIncluded in this review:Hodgkin & Non Hodgkin Lymphoma review Hemophilia A & B, Von Willebrand Disease, Factor V Leiden, Protein C & S Deficiency review Iron deficiency anemia review Thalassemia review Sideroblastic anemia review G6PD deficiency review B12 deficiency review Folate deficiency review Anemia of chronic disease review Beta thalassemia Alpha thalassemia Sickle cell anemia review Spherocytosis review Immune Thrombocytopenic Purpura PANCE review Disseminated Intravascular Coagulation PANCE review Hemolytic Uremic Syndrome PANCE review Thrombotic Thrombocytopenia Purpura PANCE review Heparin Induced Thrombocytopenia PANCE review Leukemia Pance review, CML Pance review, AML Pance review, ALL Pance review, CLL Pance review, Chronic Myeloid Leukemia review, Chronic Lymphocytic Leukemia review, Acute Lymphoblastic Leukemia, Acute Myeloid Leukemia reviewBecome a supporter of this podcast: https://www.spreaker.com/podcast/cram-the-pance--5520744/support.

Okay, so this is another 50 high yield question series. This is going to be on hematology. This is your first time doing one of these, the way that I do it. As I go through the NCCA blueprint for the pants on that specific section, I pick a few questions from each individual topic. So it's a really good way to get like a well-rounded review on a specific exam topic before you have maybe an exam on hematology. Or if you're taking the pants, if you're weak in hematology, it's a good way just to get

a quick review in for that. So let's go ahead and get started with the questions. As always, I just wanted to thank you for the support, the comments. I really do appreciate each and every one of those. So thank you so much for that. Let's go ahead and get started with question number one. 39-year-old female presents at the ER with lower leg pain and swelling. A Doppler of the lower extremity is performed, which reveals a DVT.

She admits this is her third DVT in the last few years. She also has a history of several miscarriages and recalls her mother had similar problems with blood clots. Genetic testing in this patient would likely reveal a mutation in which clotting factor. That's going to be factor 5. So this is factor 5, Liden, which is the most common, cause of inherited hyper-coagulable state. So you should always be thinking factor 5 Leiden before anything else that may have a similar presentation like protein C rest efficiency. In this patient, we have multiple DVTs. We know we have a family history of DVTs plus miscarriages,

which we know is another potential problem in factor 5 Liden. So we know the most likely cause of this problem in this patient is having a mutation in factor 5, which leads to resistance to being broken down by protein C. Question 2, 59-year-old female who was recently started on warfare and for DVT prophylaxis begins to develop necrotic lesions on her legs and feet, a deficiency in which vitamin K-dependent plasma protein likely led to this presentation. So that's going to be protein C. So this patient has warfarin-induced skin necrosis from protein C deficiency. So remember with warfarin, the reason why we bridge warfarin with heparin or low molecular

weight heparin for the first few days in our high-risk patients is due to the transient prothrombic state we see in warfarin for the first few days after initiation, where protein C, which is our our body's natural anticoagulant get shut down in the first few hours, but many of the other clotting factors don't shut down for a number of days, which leads to the increased possibility of clots, which can cause infarctin necrosis like we see in this patient. So always be aware of warfare and induced skin necrosis from protein C deficiency. Question three, a 68-year-old male of the history of rheumatoid arthritis presents to the office to review his recent lab results from his annual physical. Hemoglobin, hematocrit, and red blood cells are all decreased. Iron studies show

decreased T-I-B-C, decreased serum iron, and elevated ferritin. What type of anemia does this patient likely have? So that's going to be anemia of chronic disease. So first we see he has rheumatoid arthritis. We already know he has a chronic inflammatory disease. Then his lab show anemia with a decreased H&H, decreased red blood cells, and his iron studies show decreased iron, decreased T-IBC, and increased ferretin. So remember, the body in anemia of chronic disease, the chronic is the body's trying to hide and decrease absorption of iron because the body sees this inflammation and it assumes it's infectious. It obviously doesn't have to be, but the body's making the assumption that it is. And it tries to hide iron because bacteria can't survive without iron.

So low serum iron in the body, low T IBC, which is our total iron binding capacity because the body doesn't want to bind to any new iron. And then increase ferretin, which is our stored iron because the body is storing and hiding iron in this type of anemia. So again, remember anemia of chronic disease. you're going to see decreased serum iron, elevated ferretin, and then decrease T-IBC. The body does not want anything to do with any more iron. Question four, a 47-year-old female presents to the office due to some fatigue and tingling in her lower legs. Patient has a history of hypertension, hyperlipidemia, Crohn's disease, and denies any past surgical history. She denies alcohol use and states she is fairly healthy, eats plenty of fruits, veggies, and lean proteins.

Lab work is obtained, which shows an MCV, mean corpuscular volume of 111. decreased hemoglobin and increased homocysteine and increased methamelonic acid. What is likely diagnosis and etiology of the diagnosis in this patient? So what is the diagnosis and the etiology of the diagnosis? So the diagnosis is going to be a B12 deficiency and the etiology is going to be Crohn's disease. So I have a 47 year old with macracitic anemia. Remember that MCV, which is over 100, in this case was 111, low hemoglobin, and then the key, which is the elevation in both methamelonic acid, also known as MMS.

M.A. And elevation in homocysteine. So for the sake of a vignette, this is pretty clear-cut B-12 deficiency. But what's causing it? While she denies surgery, so we can rule out a gastric bypass and things like that, she admits to eating protein, so it's not dietary related. And then finally we see her past medical history. She mentions Crohn's disease. Remember B-12, is absorbed at the ilium. And where does Crohn's disease most commonly affect the terminal ilium? That's what's leading to the B-12 deficiency in this patient. Final note about the methamalonic acid in the homocysteine. So you're going to see these measurements. mentioned in vignettes about either B12 or folate deficiency. What you need to remember is that

homocysteine is not specific. It's elevated in both folate and B12 deficiency. So your key is the methamelonic acid, the MMA. It's only going to be elevated in B12 deficiency, and often that's the only way you can tell the difference between the two. So the way that I used to remember that is methamelonic acid, also known as MMA. I used to remember you have to be 12 years old to enjoy watching MMA like mixed martial arts. Just the way that you used to remember. You just the way that that I used to kind of remember that, no offense, anybody that watches MMA. So you have to be 12 years old to enjoy watching MMA. And then that helps you remember MMA methamelonic acid is only elevated in B12 deficiency.

Question five, sickle cell disease is due to a point mutation where glutamic acid is substituted by which amino acid on the beta chain. So that is going to be valine. So in sickle cell, you have this little switch in the beta-globin gene, something known as valine, bumps out glutamic acid. This creates this mutated hemoglobin called HB. which is susceptible to sickling. I used to remember Val kicks butt with her big glutes. That helped me remember valine kicks out glutamic acid and sickle cell. So Val kicks butt with her big glutes.

Question six, a 57-year-old man who is in the ICU being treated for sepsis begins to develop excessive bleeding from his fully catheter and IV site. Labs are drawn which show thrombocytopenia, elevated PT, elevated PTT, and elevated D-dimer, as well as a peripheral smear that displays schistocytes. What diagnosis should be suspected in this patient? So that's going to be DIC, disseminated intravascular coagulation. So how do we know this is DIC? Well, the way that I used to remember what you need to know in DIC is instead of remembering DIC,

I used to remember the mnemonic D-DIC. So you added D-DIC to it, so D-DIC. And then you remember D-Dimer, dripping, ill, and clotting. All right. So in this patient, first, D-D, is D-Dimers elevated. It's the only thrombinia that's going to have an elevated D-dimer, as well as your other clotting factors, your PT, your PTT being elevated as we see in this patient. The second DRIpping.

He's dripping from his fully catheter, his IV. Patients with DIC are always going to see them described as dripping or oozing from somewhere. Vina puncture sites, fully catheters, wherever it is, you're always going to see them saying dripping or oozing. The I stands for ill. This patient is ill. He's septic, remember? Patients with DIC, they're always going to be ill. They're always going to be sick.

This doesn't happen in a healthy patient. So whether they're septic, it's in a trauma case. malignancy, they're always going to be ill. And then finally, the last letter, which is C, which stands for clotting, and that's the underlying process going on in the body. We have this widespread microthrombi, bodies unnecessarily making clots all over the body, which leads to a deficiency in clotting factor. So when we actually need a clot, like during abenop puncture, folicathor, whatever it is,

there's no clotting factors left in these patients just ooze and bleed. Question seven, what pentat of clinical manifestations can potentially be seen in a patient with thrombotic, thrombocytopinic purpura. So that is going to be fever, anemia, thrombocytopinia, renal failure, and neurologic symptoms. So TTP has a pentad. Pentat consists of, like I said, fever, renal failure, anemia, specifically micro-angiopathic hemolytic anemia, thrombocytoppenia and neurologic changes. Most patients will not have the entire pentat.

It's very rare, less than 5%. But for the sake of the exam, remember the pentad. And then you can remember the pentide by remembering the mnemonic fat RN, F. A T, RN, and the fat stands for fever, anemia, thrombocytopinia, RN stands for renal failure and neurologic symptoms. So fat RN, that helps you remember the pentad that can be seen. Question eight, hemophilia A is a deficiency in which coagulation factor. So that is going to be factor eight.

So remember when you say the number eight, like factor eight, you say the letter A first. So you say factor eight. So you see what I'm saying? So that always hope you remember, hemophilia A is a factor. 8 deficiency. Question 9. What is the most common inherited bleeding disorder? That is going to be Von Willebrand disease.

It's seen up to 1% of the population. Question 10, a 47-year-old woman who presents with mucocutaneous bleeding as lab drawn would have labs drawn, which displays isolated thromocetopinia with a platelet count of 28,000 and normal coagulation studies. She's later diagnosed with immune thrombocitinic purpura. What is the first line choice for treatment in this patient? with mild symptoms. So that is going to be glucocorticoids.

Remember, this is an autoimmune process. Virtually all autoimmune conditions, steroids are going to be our first line. ITP is no different. Now, this patient had severe bleeding, very low platelet count. Then we have the option of also giving them IVIG, which is intravenous immunoglobulin in addition to the steroids. But remember, our mild cases, steroids are generally going to be our treatment that we use first.

Question 11. The clinical manifestation seen in thrombotic thrombocytopinic purpura are due to increased numbers of Von Willa Brand factor, increasing platelet adhesion, and causing small vessel thrombosis. The increased numbers of Von Willa Brand factor is due to a deficiency in which enzyme. So that is going to be the atom TS13 enzymes. So remember, von Willa brand factor, I always like to call him Willie, just made it easier for me.

He's like sticky paper for platelets helping to form a platelet plug, which helps us stop bleeding, which is good in moderation. But Willie doesn't have a stop button, and that's why we have added. Adam. So Adam TS-13 helps make sure Willie doesn't get out of control. But in TTP, we have a deficiency in atom TS-13. So no one to stop Willie. Willie's out of control leading to small vessel thrombosis and a number of other clinical manifestations. So again, TTP deficiency in Adam-TS-13 leading to increased numbers of Willie or Von Willa-Billibrand factor. Okay, question 12. A patient with iron deficiency anemia that had recent blood work done

will likely show what levels in their serum iron, ferretin, and T-IBC, also known as transferin. So that's going to be decreased serum iron, decreased ferretin, and increased T-IBC. So this is iron deficiency anemia, so of course our serum iron is going to be low. Ferritin, which is our iron storage. Well, this is decreased because we don't have the luxury of storing iron at this time. We're deficient in it. It needs to be out in the body being used up. So the ferritin is going to be low.

And then finally, increased T-I-B-C, which is our iron bone. binding capacity. This is the body's willingness or desire for iron. This will be increased because the body's low in iron, so it desires to have more so its binding capacity will increase to snatch up more iron. Question 13, a 57-year-old female presents to the office to review the results of her recent imaging test she had done after being diagnosed with non-hodgkin lymphoma. The results of the PET CT reveal that the lymphoma has spread. Which area of the body will likely show extra nodal involvement on the PET CT? So that is going to be the GI tract. That's the most common site of extranodal disease in non-Hodgkin lymphoma is the GI tract.

Remember, extranoidal disease is much more common in non-Hodgkin lymphoma compared to Hodgkin lymphoma where extra-nodal spread is rare. Question 14, a 66-year-old woman, six days post-op begins to develop shortness of breath. It is discovered on CT that she has developed a pulmonary embolism and is started on low molecular weight heparin. Five days later, necrotic areas of discoloration are noted on her lower extremities, and her labs show her platelet count is down to 70,000, which is a significant decrease from her platelet count of 275,000 on admission. What treatment should be initiated in this patient for the likely diagnosis?

So the treatment would be to stop heparin and start a non-heparin agent like Argatroban. So this is heparin-induced thrombocytopinia. She's on heparin. All of a sudden we see a big drop in our platelets. 50% drop in platelet count is usually what you'll see in these patients. So look for that in the vignette. If you see they had a, you see they met. mention a patient has this 50% or greater dropping platelets and they recently started heparin right away. That should just be common sense that this is HIT. It's the first thing that should come to mind. They also mentioned she has necotic lesions on our low extremities, which is caused from the thrombosis. These are all signs of heparin-induced thrombocytopenia. So what's the

treatment? Well, it's the heparin that caused this. So we stop the heparin and we start a non-heparin agent. This is usually going to be a direct thrombid inhibitor like our gacharban, which is one of the more popular ones to use. Question 15, a 57-year-old male presents to the office today, complaining of fatigue and weakness over the past few months. On exam, splenomaglia is noted. A subsequent CBC and bone marrow biopsy are performed, and it is discovered that he has chronic myelogynous leukemia, CML. What class of medication will this patient likely be started on? So that is going to be tyrosine kinase inhibitors like a matinip. So in CML, we have a tyrosine kinase that's over expressing and causing cancer. And CML is the first targeted therapy we came up with back in

the 90s. We developed this class of drugs and amatinib was the first. So tyrosin kinase inhibitor, like I said, amatinib was the first one that we developed that targeted and decreased this overexpression that we see in CML. So remember in CML, your tyrosin kinase inhibitors like amatinib will usually be your first line therapy. So the way that I used to remember the important things, but the things that I feel are important for CML is I used to remember this little story. So the story goes, Tyrone was a country music lover and was excited to go to a country music concert in Philadelphia on 9-11. Unfortunately, tragedy struck that day and the concert was postponed 11 days later to 922. So what do those things stand for?

So Tyrone helps you remember Tyrocyne Kinesis Inhibitor, which is your first line therapy, was a country music lover, CML, which is going to be your chronic myelogenist leukemia. And then he was excited to go to a country music concert in Philadelphia. Philadelphia, Philadelphia chromosome, as we see in CML, on 9-11. Unfortunately, tragedy struck that day and the concert was postponed 11 days later to 922, which is the 922 translocation that we see in CML. All right, question 16, 49-year-old female with history of diabetes presents to the office for a routine physical. She states she doesn't feel terrible, but she's had some minor aches and pains in her joints. On physical exam, you note a hyperpigmentation of her skin as well as hepatomagaly.

Labs are drawn which show an increase in serum iron, increased ferretin, and increased serum transferin saturation. What is the mainstay of treatment for the likely diagnosis in this patient? So that is going to be phlebotomy. So this is hemacromatosis, which is a disease of iron accumulation and the first line treatment for hemacromatosis is phlebotomy. So why should you have suspected hemachromatosis in this patient? So first, anytime you see diabetes and hyperpigmentation of the skin mentioned in a vignette,

as is in this vignette, you should right away be thinking hemochromatosis. Hemacromatosis has the nickname of bronze diabetes, which is due to the bronzing of the skin seen of these patients and the increased risk of diabetes from pancreatic beta cell damage from the iron. And if that wasn't enough, the lab show elevated transfarin, ferretin, and iron, all obviously seen in hemacromatosis, as well as hepatomagli on exam, arthralogas, these are all common findings, and then we know phlebotomy is the mainstay of treatment in these patients

to remove the excess iron. Question 17, a specimen is sent to the lab for analysis. When the specimen is placed on the slide and a cover slip is applied, the cells appear to be smudged or damaged. Which type of leukemia most commonly displays this type of abnormality? So that is going to be chronic lymphocytic leukemia. So smudge cells are a hallmark finding in CLL. All smudge cells are a lab artifact.

They're lymphocytes smeared out in the process of being spread on the little little glass slide with microscopy when they put the little cover slip over the top. And this is just because the CLL cells are more fragile than normal lymphocytes. The way that I used to remember that is CLL, chronic lymphocytic leukemia. That CLL stands for crushed little lymphocytes. That helps you remember that the smudge cells are the hallmark finding in CLL, crushed little lymphocytes. Question 18, the Philadelphia chromosome is caused from a translocation between what two chromosomes, So that is going to be 9 and 22.

If you remember the story about Tyrone in Philadelphia, the concert getting pushed back 11 days, helped you remember the 9 and 22 is the translocation for the Philadelphia chromosome. Question 19. The presence of hour rods and myeloporoxidase positivity should raise suspicion for which acute myelogynous leukemia subtype. So that is going to be acute promylacitic leukemia. The way that I used to remember hour rods were associated with acute promylochitis. acidic leukemia is that hour rods instead of remembering hour rods I just used to remember a rod so if you've heard of Alex Rodriguez he played for the New York Yankees they call him a rod and he played in New York in the big apple for the New York Yankees so I used to remember a rod our rods or Alex Rodriguez played for the New York Yankees in the big apple and Apple is spelled APL and APL stands for acute pro myelicytic leukemia so that helps you remember that the hour rods are seen in acute pro myelocytic leukemia. Question 20, a 32-year-old male presents to the

office today with pain and swelling in his jaw for the past few months. Upon obtaining history from the patient, you learn he recently moved here from South Africa about six months ago. On physical exam, non-tender, submandibular and axillary lymph nodes are palpated and a large mass is visualized on the right lower side of the mandible. The lymph nodes are biopsied, which display large, bright histocytes scattered throughout a back. background of the darker basophilic tumor cells, almost creating the appearance of stars in a dark sky. What is the likely diagnosis in this patient?

So that is going to be burkitt lymphoma. So you have a patient that recently moved here from Africa, which we know is the endemic form of burkitt lymphoma, where it's located. Next he describes jaw pain. We also see that mass. So we know up to 60% of cases of endemic burkitt lymphoma involve the jaw and facial bones, non-tender lymphadenopathy on exam. a starry sky appearance on biopsy. These are all key findings in Burkitt lymphoma, which is a

subtype of non-hachial lymphoma. So Burkitt comes up a lot in the exams, and I think you should be familiar with those few things because they do come up. So really there's four things that you need to remember about Burkitt lymphoma. And the way that I used to remember that is instead of remembering Burkitt lymphoma, I used to remember burkite lymphoma, like a kite in the sky. So as soon as you see Burkitt lymphoma, think of burkite lymphoma. So when you see burkite lymphoma, you see burkite lymphoma, think of a kite and think of a guy kite surfing in the middle of the ocean. So he's kite surfing in the middle of the ocean and it's the middle of the night. He decided to do kite surfing in the middle of the night and you look out into the sky and there's all these stars up in the sky

and that helps you remember your starry sky appearance on biopsy. Now he's kite surfing. He's holding onto that bar that they hold onto. It's called a control bar. That helps you remember that the burkite lymphoma is commonly associated with Epstein bar, particularly the end endens. variety, the African variety. Now he's kite surfing, middle of the ocean, middle of the night, starry sky holding onto that bar, Epstein bar virus, and he's looking out in the ocean and he sees a shark circling him. It's not just any shark, it's jaws. That helps remember the jaw involvement, the jaw mass in burkitt or burkite lymphoma. And finally, that shark is there because he's actually surfing off the coast of Africa where the most shark attacks occur in the entire world. That helps

Remember, the endemic African version is where you have that increase in Epstein Bar and jaws. Remember, Burkitt lymphoma is Burk kite lymphoma, guy kite surfing, middle of the night, starry sky appearance, holding onto that control bar to control the kite, Epstein Bar virus, being circled by a shark, not just any shark jaws, and he's surfing off the coast of Africa, the African endemic variety. Question 21, what is the most common type of Hodgkin lymphoma? So that is going to be nodular sclerosis. That's the most common type, up to about 70% of the cases. Question 22. 12-year-old African-American boy presents to the emergency department complaining of severe abdominal pain.

After obtaining a history from the mother, it has discovered the patient has a history of sickle cell disease, and this is his fourth time being hospitalized in the last six months with the presumed sickle cell crisis. He's not currently on any medications and has no other medical conditions. After the patient is treated for the acute vasoeclusive crisis, which medications should be started in the near future to decrease frequency and severity of future episodes. So that is going to be hydroxyria. So this is the only medication I would memorize for sickle cell. The reason why we give hydroxiaria is because it increases the fetal hemoglobin in the body,

which does not sickle. So this reduces the frequency of future episodes of these painful vasoeclusive crises. It does take a while to start working, though. It can be weeks to up to months. So there's real no value in initiation in an acute episode, but definite value as a preventative therapy. Question 23. What is the most common childhood malignancy? So that is going to be acute lymphoblastic leukemia, also known as acute lymphocytic leukemia. It's the most common childhood malignancy. The way that I used to remember that, I don't know if you've ever heard of this

popular soap opera that used to be around in the 90s and the 2000s. It's called All My Children. So all my children, A-L-L-L-Machial, my children, all acute lymphoblastic leukemia, my children, most common malignancy in childhood. So that used to help me remember that. Question 24, a patient presents to the office today with some questions regarding his recent diagnosis of B12 deficiency and is wondering how he could have wound up being deficient in B12. He states he eats plenty of meat. He's never had a surgery.

And his only medical conditions are type 2 diabetes and asthma. He only takes two medications, metformin for diabetes, and an albuterol inhaler as needed for his asthma. What would you tell the patient the most likely causes for his B12 deficient? C. So you would tell this patient the most likely cause for his B12 deficiency is metformin. So with B12 deficiency, of course, you should focus on your most common problems. It's going to be pernicious anemia, Crohn's disease, your diet, but also be aware of medications. So metformin probably being one of your biggest ones because it's taken by so many people.

So be aware metformin reduces intestinal absorption of vitamin B12 and up to 30% of patients and lower serum vitamin B12 concentrations in up to 10% of patients. So you should always screen your patients that take metformin for B12 deficiency, particularly if they've been taking it a long time, like over 10 years. It becomes more prevalent. Question 25, what is the most common cause of anemia worldwide? That is going to be iron deficiency anemia. Question 26, a seven-year-old boy presents to the office accompanied by his mother.

She states he has been tired for several weeks and has had a persistent fever that just does not seem to dissipate. On exam, ekemosis is noted on both upper and lower extremities, as well as hepatomagally. CBC is performed that reveals anemia, thrombocytopinia, and neutropenia. Subsequent bone marrow biopsy is ordered, which displays over 20% lymphoblasts. What is the most likely diagnosis in this patient? So that is going to be acute lymphoblastic leukemia, aka acute lymphocytic leukemia. So we know that this is AOL because first it's the most common childhood malignancy. So should always be your first thought when you have a child and you suspect in malignancy.

Then we have fever, bruising, which are all common in ALLL, Padomagaly, which is one of the most common physical exam findings. Then we see on labs, anemia, thrombocytopenia, and neutropenia. And then the confirmatory test, which honestly could have just listed this and this would probably give you the diagnosis, is the greater than 20% lymphoblasts on bone marrow biopsy. That pretty much seals the deal. We know we are most likely dealing with an acute lymphoblastic leukemia. Question 27. where is the most common site on the body to have lymphadenopathy and a patient with Hodgkin lymphoma.

So that is going to be the neck cervical, superclivicular area. So in Hodgkin lymphoma, the neck is the most common site for lymphadenopathy, with 60 to 80 percent of patients having enlarged cervical and or superclivicular nose. Remember, this is going to be painless lymphadenopathy in Hodgkin lymphoma, lost they're drinking alcohol, which is a whole other story of that weird thing that happens when Hodgkin lymphoma patients drink alcohol. Question 28, large, by, or multi-nucleated cells sometimes referred to as having an owl-eye appearance on histology. Seen in Hodgki lymphoma are known as, that's going to be Reed Sternberg cells.

Question 29, a 58-year-old male presents to the office complaining of facial flushing, accompanied by headache, blurred vision, and severe itching after he takes a hot shower. CBC displays an increase in both hemoglobin and hematocrypt, a mutation in which gene likely led to this this patient's presentation. So that is going to be the Jack 2 gene, the J-A-K-2 gene. So this patient has polycythemia vera, which is caused for a mutation in the Jack 2 gene, aka the Janus-Kinus, Janus-Kinis-Kinis-2 gene. And a patient with this mutation will commonly present with increased hemoglobin hematocrit due to the primary erythrocytosis. They may have visual changes, headache flushing in the face, all due to the hyper viscosity of the blood. And the one that

always seems to come up on the exams, and it's seen in a around 15% of patients is the pruritis after hot baths or showers. It's also known as aquagenic pruritis. Question 30, the 27-year-old female presents to the office complaining of persistent fatigue. On physical exam, Palora is noted, as well as spooning of the nails and a smooth appearance to her tongue. She is not currently taking any prescription medications and has no medical conditions. Her CBC reveals decreased red blood cells, decreased hemoglobin, and hematicrip.

What additional lab test should be older to control? confirm the diagnosis in this patient. So that would be an iron panel or iron studies. So this patient has a classic presentation of iron deficiency anemia, pallor fatigue, quinloinichia, which is the spooning of the nails, atrophic lusitis with a loss of the tongue papilli, which is why she has that smooth appearance to her tongue. And then finally, the decreased H&H, decreased red blood cells, we just need an iron panel to confirm, which is likely going to show decreased serum iron, decreased ferretin, and

increased T IBC. Question 31, B-12 deficiency and folate deficiency are common types of microcytic, normalcytic, or macrocytic anemia. So B-12 and folate are going to be macro-cetic anemia. That's going to be an MCV above 100. So remember in B-12 and folate deficiency, the red blood cells are commonly going to be larger with an MCV or mean corpuscular volume over 100. Question 32, a 67-year-old African-American male presents to the office complaining of lower back pain for the last six months. He has tried over the counter analgesics, warm compresses, all with minimal improvement in his pain. He denies any trauma to the area, and the pain is increasing over the weeks, and he is feeling very tired lately. Routine lab work is ordered, which reveal

elevated calcium and elevated creatine levels, and follow-up urine electrophoresis is positive for Benz-Jone proteins. What diagnosis should be suspected in this patient? So that is going to be multiple myeloma. Okay, so why would, why should we suspect multiple myeloma in this patient. So first, demographics. This is more common in African-American patients and also more common in men. Patient fits that criteria. Next, bone pain, particularly in the back, involving the vertebrates. One of the most common clinical manifestations, fatigue is another one. It's always non-specific, though. And then we have labs which show renal insufficiency and hypercalcemia. Those are both common findings in multiple myeloma. And then finally, on urine protein electrophoresis,

we see those Benz-Jones proteins, which are normally composed of Kappa. and Lambda light chain. This combined with the clinical presentation leads us to the most likely diagnosis, which is multiple myeloma. Question 33, a 22-year-old female presents to the office today complaining of persistent fever and unexplained weight loss. On physical exam, non-tender lymphadenopathy is noted. Biopsy is obtained of the affected lymph nodes revealing Reed-Sternberg cells. What is the most likely diagnosis in this patient? So that is going to be Hodgkin lymphoma. So that's evident by the non-tender lymphadenopathy, the bee symptoms like the fever and weight loss, and then, of course, the Reed-Sternberg cells, which are pathognomonic for Hodgkin lymphoma, and that could have honestly

been the only thing I said, and you probably would have known that that was the likely diagnosis. So the mnemonic that I have for Hodgkin lymphoma is, remember, Hodgkin lymphoma is all about the B cells, B as in boy, Hodgkin Lymphoma is all about the B cells. So remember B cells for Hodgkin Lymphoma, and remember everything's about bees. So B-cell malignancy. Hachka lymphoma is a B-cell malignancy originating the lymphatic symptoms. So that's your first B. Second B, B symptoms, common in advanced disease, so fever, night sweats, weight loss. Third B is for EBV infection, which can be associated. Hachkin lymphoma can be associated with Epstein-Barr virus.

Next B is bin-nucleated cells. Remember your reed-sternberg cells are going to be bi-nobledged. Next B is for bimodal distribution. You have a peak in late adolescence and in older adults in Hanchkin. the last B stands for bound in place because Hodgkin lymphoma is usually localized, extra nodal spread like we see in non-Hodgkin is rare. So bound in place. Again, B-cell malignancy, B-s symptoms, eBV infection, binucleated cells, bimodal distribution, and bound in place. Question 34, hemolytic Euremic syndrome is a clinical syndrome characterized by a triad of what three clinical manifestations. That is going to be hemolytic anemia, specifically microangopathic, thrombocytopinia and acute kidney injury or renal dysfunction.

So it's one of the main causes of acute kidney injury in children, and it's a toxic, mediated immune reaction predominantly seen in children after a GI infection. That's your HUS or hemolytic uremic syndrome. Question 35, a 37-year-old female presents to the office complaining of easy bruising, frequent nosebleeds for the last few weeks. She has a history of hypothyroidism, type 2 diabetes, and was recently diagnosed with epilepsy. Her medications include metformin, levitisitis. and caromazepine, which was started a few weeks ago.

Labs are shown which reveal anemia, neutropenia, and thrombocytopinia. What is the likely cause of this patient's presentation? So that is going to be carbamazepine. So this patient has aplastic anemia, easy bruising, mucocutaneous bleeding, and then the pancytopenia, which is seen on the labs, so decrease in all cell lines, platelets, red blood cells, white blood cells. And the cause, although sometimes it can be idiopathic, and this patient, she was recently started on carbamazepine, which is one of the few. medications that can cause aplastic anemia and definitely one of the ones that you should remember.

It's one of the more common ones and the ones that they usually like to ask about and test questions. Question 36. In addition to measuring the common coagulation pathway, PTT, also known as partial thromoplastin time, also measures which additional pathway of the clotting cascade. So PTT, in addition to measuring the common coagulation pathway, also measures the intrinsic pathway. The way that I used to remember this, is so PTT measures the intrinsic pathway. PT, which is pro thermon in time, measures the extrinsic pathway. So what I used to remember is that PTT, the T and the T are right next to each other in the word PTT, and that's because T and T are in love.

They're almost like the top of the T's, it's almost like they're holding hands and they're in love. So T and T are in love, and that helps you remember PTT measures the intrinsic pathway. And then with PT, the T is gone. T is no longer there. That's because T and T are broad. broken up and they're X's. They are X's and that's because PT measures the extrinsic pathway. All right. Question 37. Which phase of hemostasis involves platelets forming a platelet plug at the

site of vascular injury? So that is going to be primary hemostasis. So primary hemostasis, it's the reason why when you get like a little scrape or a blood draw done that you stop bleeding within a minute or two. It's your platelets that swoop in, form a platelet plug and quickly stop the bleeding. So later on, your secondary hemostasis, kicks in which involves your clotting factors to incorporate fiber in and form a clot. But initially it's your primary hemostasis with that little platelet plug and that's what's involved in the primary hemostasis. Question 38, a 56-year-old homeless male is brought into the ER today by fire rescue after the

patient was found unconscious behind a local department store. By the time the patient is brought into the ER, he is awake and alert and you're able to obtain a history and physical. States that he doesn't know of any medical conditions. He's not taking any medication, but he admits he. his diet is poor with most of his caloric intake coming from alcohol. Neuro exam is normal, and he states besides feeling tired, he's otherwise feeling well. Routine labs are obtained which show a decreased hemoglobin and hematocrit, decreased red blood cells,

and an MCV of 110. Homo cystine and methamelonic acid levels were obtained, demonstrating an increase in homocysteine, but normal level of methamelonic acid. What is the most likely diagnosis in this patient? So that's going to be a folate deficiency. So we have a homeless patient who has a poor diet, getting most of his daily calories from alcohol, which is one of the most common causes of folate deficiency, is poor nutrition. Then we have the labs, which indicate decreased H&H in addition to an increased MCV.

So we know we have a macracidic anemia. Then we just have to decide is this B12 or folate deficiency. The two keys in the vignette are the normal neuroexam. So remember B12 often has neurosyptoms, but folate deficiency normally does not. And then the increased homocysteine but normal methamelonic acid. Remember, the normal methamelonic acid is the key because it's only going to be elevated and be 12 deficiency. And remember, you have to be 12 years old to enjoy watching MMA. So that helps you remember that again.

Question 39, a 7-year-old boy presents to the office today with swelling over his right knee. Mother states that they were at the playground and he bumped his knee on the slide as he was coming down. She states he's always bruised very easy and often complains of. of joint pain. Coagulation studies show a prolonged PTT and plasma factor 8 assay shows decreased levels of factor 8. What would be the first line pharmacologic agent to administer in this patient with mild symptoms? So that would be Desmopressin, also known as DDAVP. So this patient has a clear presentation of hemophilia A. It's male gender. Remember this is an X-linked recessive disorder. I used to remember hemophilia and I used to remember Dr. Phil. Dr. Phil is obviously a guy.

and as soon as I see hemophilia, I'd remember this is an excellent recessive disorder, and this should be a male in the vignette. Next, hemarthrosis, which is actually the most common manifestation in your ambulatory patients with hemophilia, prolonged PTT, and then decreased factor 8. So we know this is hemophilia A, but how do we treat mild cases of hemophilia A? That's what the DDAVP, also known as Desmopressin, which increases the release of endogenous von Willebran factor. And what Von Willa Brin factor does is stabilizes factor 8 in the body.

If this patient had a severe bleeding episode, then you can use factor 8 infusions. But in your mild cases, you can use DDAVP. Question 40, a patient with a history of iron deficiency anemia describes a constant urge to chew on ice. This craving for ice is known as, that's going to be pegophagia. So patients with iron deficiency anemia may have what's known as PICA, PICA. So pica refers to a desire or compulsion to eat substances not fit as food. So it can be toilet paper chalk, baby powder. But pegophasia is specifically pika for ice.

And it's considered actually very specific for iron deficiency anemia. It sounds ridiculous, but it's actually really specific to iron deficiency anemia. And once you treat these patients, it actually resolves very quickly once the anemia is treated. It does come up a lot on exam questions. So it doesn't seem that important, but you should be familiar with. that in case it does come up. Question 41. Describe the appearance of red blood cells on a peripheral smear in a patient with multiple myeloma. So they're going to have something known as a Rulot formation, which is a stack of coins formation. So Rulot formation is seen in over 50% of patients with multiple

myeloma. And it's when the red blood cells take on an appearance of a stack of coins in the suspension of blood. And it's due to the elevated serum protein levels in these patients. So the way that I used to remember this is if you actually look at a roulo formation, the red blood cells stacked on top of each other to me look like M&M stacked on top of each other, like little red M&M stacked on top of each other. So as soon as I would see multiple myeloma, MM, M&M, I would think of M&Ms and I would think of M&Ms being stacked and that always helped me remember the Rurloaf formation that can be seen in multiple myeloma, the stack of coins, the red blood cells stacking on each other that look like stacked red M&Ms. question 42 what is the name of the x-link recessive disorder caused from a deficiency in factor nine sometimes referred to as christmas disease so that is going to be hemophilia b he's so hemophilia b is a factor nine deficiency um sometimes you'll hear it called christmas disease and that's actually named after the first patient that was ever diagnosed with the disorder named uh his name was

stephen christmas um the way that i used remember that is hemophilia b i used remember hemophilia b9 like the tumor is B9. So hemophilia B is a factor 9 deficiency. The tumor is B9. That just used to help me remember it. Question 43, a 47-year-old female presents to the office complaining of gingerville bleeding and epistasis for two days.

She's recovering from an upper respiratory infection, but otherwise feels well. She's not currently taking any prescription medication and denies past medical history. Labs are obtained including CBC, CMP, peripheral smear, and coagulation panel, which are all normal, with the exception of a platelet count of 22,000. Remember, normal platelet count is 150 to 450,000. What is the most likely diagnosis in this patient? So that is going to be ITP immune thromocytopinic perpura. So we have a patient with isolated thromocytopinia. All labs are otherwise normal. So even if there was nothing else in the vignette, right away, isolated thrombocitone, you should be thinking of ITP. Now remember, in the other thrombocitia is like TTP, you're going to be, you're going to see signs of hemolytic anemia on this smear. So schistocytes, et cetera.

In HUS, you're going to have increased BUN-C creatine. In your DIC, you're going to have the coag panel being off. But in this patient, just the platelets are low. So that, in addition to the presentation with mucocutaneous bleeding, history of a recent infection with a preceding viral infection is one of the more common causes of primary ITP. Not taking any meds, we can roll that out. And this patient, the most likely cause is going to be immune thrombocytic penic perpera. Question 44.

What would be the initial treatment of choice for a patient with thrombotic, thrombocytopinic perpura? So that is going to be plasma exchange, also known as plasmapheresis. So plasma exchange is the mainstay of treatment for TTP and all patients with TTP are treating with plasma exchange until the platelet count normalizes. Prior to plasma exchange therapy, the mortality rate for patients with TTP was close to 90%. So this therapy is really crucial in treating these patients. In 145, a 42-year-old male presents to the office complaining of tea-colored urine he notices in the middle of the night and in the early morning that usually clears up by midday.

In addition, he feels tired. He's been experiencing some mild abdominal pain. Labs reveal decreased red blood cells, low hemoglobin and hematocrit, leukopenia, and thrombocytopenia. What diagnosis should be suspected in this patient? So you should suspect proxysmal, nocturnal hemoglobinuria. So anytime you see Coca-Cola or tea-colored urine mentioned, and they specifically say it happens in the early morning or at night, but it clears up as the day goes on. Right away, your first diagnosis that should come to mind is going to be proxysmal nocturnal hemoglobinuria. And that's from the hemolytic anemia that these patients have, which leads to this.

We also see, he has some fatigue from the anemia, some abdominal pain, and that's because patients with PNH can have smooth muscle dystonia. That may be one of the first presenting symptoms. It manifests as an esophageal spasm, abdominal pain. And then finally, the labs, which reveal pancytoppenia, which is another manifestation of PNH. The only thing missing in this trial would be the appearance of venous thrombosis, which is another possible finding in PNH. Question 46 in hereditary hemochromatosis.

A mutation in the HFE protein leads to a decrease in which iron-regulating peptide hormone that results in an increase in iron absorption from the intestinal tromotosis. tract. So that would be hepcidin. So HFE mutation leads to inappropriately low levels of hepcidin. Low hepcidin levels result in increased intestinal iron absorption. So the role of hepcidin is basically to decide whether or not the body has had enough iron, tells the gut to stop absorbing it when we've had a sufficient amount of iron. But without hepcidin around, the iron just keeps getting absorbed nonstop. There's no off switch. And that leads to the clinical manifestations we see in hemachromatosis. Question four.

47 in hemophilia A and B, where's the most common site for bleeding in your ambulatory patients representing up to 80% of hemorrhages? So I mentioned this before, but that is going to be the joint space hemarthrosis. So look for hemarthrosis in a vignette about hemophilia. It's definitely going to be there. That's your most common presentation in your ambulatory ambulatory patients. Question 48, the patient presents to the office today with questions about his recent lab work. He states on the report it said he has hypersegmental. neutrophils and he was wondering what would cause this.

His past medical history includes chronic lymphocytic leukemia, B-12 deficiency, type 2 diabetes, and a surgical history includes an appendectomy, splenectomy, and colon resection. What would you tell the patient the most likely causes for the hyper segmented neutrophils? So that would be the B-12 deficiency. So B-12 as well as Foley deficiency can both lead to hyper-segmented neutrophils. So normal neutrophils normally contain three to four low. But in both vitamin B12 deficiency and folate deficiency, the neutrophils can continue up to five to six lobes. So if you see hypersegmented neutrophils in a vignette, be thinking macracitic anemia like B12 or folate deficiency.

Question 49. Hereditary sphero cytosis is caused by a defect in which part of the red blood cell. So that is going to be the red blood cell membrane and cytoskeleton. So in hereditary sphero cytosis, you have an intrinsic defect in the red blood cell membrane proteins and cytoskeleton. which leads to both the increased fragility of red blood cells and the alteration of the bi-concave shape of the cell into a sphere shape. Question 50. Last one.

In a patient with type 1 von Willebrand disease, what is the treatment of choice to treat mild to moderate bleeding? So that is going to be DDAVP Desmopressin. So DDAVP, it's effective in the majority of type 1 von Willa brand disease patients with minor bleeding or used in prophylaxis for minor procedures. Now, patients with type 3 and patients with severe types of 1 and 2 von Willowbran disease, they're not going to respond to DDAVP and usually will require Von Willowbran factor concentrates. That's because in type 3 and severe types of one and two, there's a near complete depletion of von Willa brand factor.

And remember what DDAVP does, DDAVP promotes the release of endogenous von Willa brand factor. But if you have no Von Willa brand factor, there's no willy floating around. DDAVP is not going to do anything. So type 3 or type 1 and 2, there's just not enough willy around. So it doesn't matter if you encourage this release with DDAVP. It's not around to respond. So really this is best going to be used in patients with type 1 that have a mild to moderate bleeding. All right.

So that was a 50 high yield question series for hematology. I hope that was helpful. If it is, please let me know in the comments. And please let me know if there's anything you want me to cover in the upcoming podcasts and YouTube videos. If you haven't checked out the YouTube channel yet, please do. Good visuals to go along with the audio. Thank you so much for listening to the podcast.

And good luck in PA school. Good luck on your pants, your panor, your EORs. And thank you again for listening.