Hematology Practice Questions

Q: The use of desmopressin is best indicated for therapy in which of the following bleeding disorders?

Von Willebrand disease (VWD). **Explanation:** **Desmopressin (DDAVP)** is a synthetic analogue of vasopressin that stimulates the release of **von Willebrand factor (vWF)** and **Factor VIII** from endothelial storage sites (Weibel-Palade bodies) [2]. 1. **Why Option C is Correct:** In **Von Willebrand Disease (VWD)**, particularly Type 1 (the most common form), there is a quantitative deficiency of vWF [2]. DDAVP effectively increases endogenous plasma levels of vWF and Factor VIII, making it the treatment of choice for minor bleeding episodes or surgical prophylaxis in these patients [2]. 2. **Why Other Options are Incorrect:** * **Severe Hemophilia A (Option A):** DDAVP can be used in *mild* Hemophilia A. However, in *severe* cases (Factor VIII <1%), there are no endogenous stores to release; therefore, exogenous Factor VIII replacement is mandatory. [1] * **Severe Hemophilia B (Option B):** Hemophilia B is a deficiency of Factor IX. DDAVP has no effect on Factor IX levels; these patients require Factor IX concentrates. * **Glanzmann Thrombasthenia (Option D):** This is a qualitative platelet disorder (deficiency of GpIIb/IIIa) [3]. DDAVP does not correct this receptor defect; treatment usually involves platelet transfusions or recombinant Factor VIIa. **NEET-PG High-Yield Pearls:** * **Route:** DDAVP can be administered IV, SC, or via a highly concentrated nasal spray (Stimate). * **Side Effects:** The most critical side effect is **hyponatremia** (due to the antidiuretic effect), which can lead to seizures. Patients should be advised on fluid restriction. * **Tachyphylaxis:** Repeated doses lead to a diminishing response as endothelial stores become exhausted. * **Contraindication:** Avoid DDAVP in **VWD Type 2B**, as it can cause paradoxical thrombocytopenia due to the release of abnormal vWF that induces platelet aggregation.

Q: Which drug is used for the treatment of Acute Myeloid Leukemia (AML) with FLT-3 mutation?

Gilteritinib. **Explanation:** **Acute Myeloid Leukemia (AML)** management has evolved with the identification of specific molecular markers. The **FLT3 (Fms-like tyrosine kinase 3)** mutation is one of the most common genetic alterations in AML (seen in ~30% of cases) and is associated with a poor prognosis and high relapse rates. * **Why Gilteritinib is correct:** **Gilteritinib** is a highly potent, selective **second-generation FLT3 inhibitor**. It targets both FLT3-ITD (Internal Tandem Duplication) and FLT3-TKD (Tyrosine Kinase Domain) mutations. It is specifically FDA-approved for the treatment of adult patients with **relapsed or refractory AML** with a FLT3 mutation. Other FLT3 inhibitors used in AML include Midostaurin (used in induction) and Quizartinib. **Analysis of Incorrect Options:** * **A. Crizotinib:** An ALK (Anaplastic Lymphoma Kinase) and ROS1 inhibitor primarily used in **Non-Small Cell Lung Cancer (NSCLC)** and ALK-positive Anaplastic Large Cell Lymphoma. * **C. Acalabrutinib:** A second-generation **Bruton Tyrosine Kinase (BTK) inhibitor** used in Chronic Lymphocytic Leukemia (CLL) and Mantle Cell Lymphoma. * **D. Ibrutinib:** The first-generation **BTK inhibitor** used for CLL, Small Lymphocytic Lymphoma (SLL), and Waldenström Macroglobulinemia. **High-Yield Clinical Pearls for NEET-PG:** * **FLT3-ITD** mutation is a marker of **poor prognosis** in AML [1]. * **Midostaurin** is the first-generation FLT3 inhibitor added to standard "7+3" chemotherapy for newly diagnosed FLT3+ AML. * **All-trans retinoic acid (ATRA)** and **Arsenic Trioxide** remain the drugs of choice for the M3 subtype (APML) characterized by t(15,17) [1]. * **Venetoclax** (BCL-2 inhibitor) is another high-yield drug now used in AML for elderly patients unfit for intensive chemotherapy.

Q: All of the following conditions are acquired causes of iron overload, EXCEPT:

Hypothyroidism. **Explanation:** The correct answer is **Hypothyroidism**. Iron overload is characterized by an excess of iron in the body, which can be either hereditary (Primary Hemochromatosis) or acquired (Secondary Hemochromatosis) [1]. **Why Hypothyroidism is the correct answer:** Hypothyroidism is **not** a cause of iron overload. In fact, it is frequently associated with **iron deficiency anemia** due to decreased gastrointestinal absorption of iron, achlorhydria, or heavy menstrual bleeding (menorrhagia) in women [2]. While endocrine disorders like Diabetes Mellitus are *complications* of iron overload, hypothyroidism does not cause it [1]. **Analysis of Incorrect Options:** * **Sideroblastic Anemia:** This is a classic cause of acquired iron overload. Ineffective erythropoiesis leads to increased iron absorption and frequent blood transfusions, resulting in iron deposition in tissues (hemosiderosis). * **Chronic Hepatitis C:** Chronic liver inflammation can lead to elevated ferritin levels and mild-to-moderate hepatic iron overload. It often involves the downregulation of hepcidin or the release of iron from necrotic hepatocytes. * **Nonalcoholic Steatohepatitis (NASH):** Also known as "Dysmetabolic Iron Overload Syndrome," NASH is frequently associated with increased hepatic iron stores, which can further exacerbate oxidative stress and liver fibrosis. **Clinical Pearls for NEET-PG:** * **Gold Standard Diagnosis:** Liver biopsy with Prussian Blue staining (to calculate the Hepatic Iron Index) is the definitive test, though MRI (T2*) is now the preferred non-invasive method. * **Porphyria Cutanea Tarda (PCT):** This condition is strongly associated with iron overload and Hepatitis C [3]. * **Treatment of Choice:** Therapeutic phlebotomy is the mainstay for most iron overload states; however, in anemias like Sideroblastic anemia, **iron chelators** (e.g., Deferasirox, Deferoxamine) are used instead.

Q: Which of the following statements is FALSE regarding hemochromatosis?

Prone to Mycobacterium enteric infections. The correct answer is **D**. Hemochromatosis is a disorder of iron overload, and certain pathogens thrive in high-iron environments. While patients are highly susceptible to specific "siderophilic" (iron-loving) bacteria, **Mycobacterium** species are not typically associated with increased enteric infection risk specifically due to iron overload in hemochromatosis. **Why the other options are true:** * **Vibrio vulnificus (Option A & C):** This is a high-yield association. *Vibrio* requires free iron for rapid growth and virulence. Patients with hemochromatosis are at a significantly higher risk of fatal septicemia from *Vibrio vulnificus* after consuming **raw seafood/shellfish** or through wound exposure to seawater. * **Yersinia enterocolitica (Option B):** *Yersinia* is a siderophilic organism that cannot produce its own siderophores (iron-binding molecules) efficiently. In an iron-overloaded host, the excess iron facilitates its proliferation, leading to severe enterocolitis or sepsis. * **Listeria monocytogenes:** Though not listed, it is another siderophilic organism to remember. **Clinical Pearls for NEET-PG:** * **The "Bronze Diabetes" Triad:** Skin hyperpigmentation, Diabetes Mellitus, and Cirrhosis [1]. * **Most Common Cause of Death:** Historically cirrhosis; however, currently, **Hepatocellular Carcinoma (HCC)** is the leading cause of death in treated patients [1]. * **Cardiac Involvement:** Most common manifestation is **Restrictive Cardiomyopathy**, though dilated cardiomyopathy can also occur. * **Joints:** Characterized by "hook-like" osteophytes in the 2nd and 3rd MCP joints and pseudogout (CPPD). * **Screening:** Best initial test is **Transferrin Saturation** (>45%) [3]; Gold standard for diagnosis is **HFE gene analysis** [1],[2].

Q: Which of the following is preferred in an elderly woman with macrocytic anemia and early signs of neurological deficit?

Hydroxycobalamine. The clinical presentation of **macrocytic anemia** combined with **neurological deficits** (such as paresthesia, loss of vibration/position sense, or ataxia) is a classic hallmark of **Vitamin B12 (Cobalamin) deficiency**. [1] **1. Why Hydroxycobalamine is Correct:** In Vitamin B12 deficiency, neurological symptoms occur due to the failure of myelin synthesis (Subacute Combined Degeneration of the Spinal Cord). **Hydroxycobalamine** is the preferred parenteral form of B12 because it has a longer half-life and better tissue retention compared to cyanocobalamin. In patients with neurological deficits, parenteral administration is mandatory to bypass potential malabsorption (e.g., Pernicious Anemia) and ensure rapid correction to prevent permanent nerve damage. **2. Why Other Options are Incorrect:** * **Folic acid:** While it can correct the megaloblastic anemia, it **cannot** treat the neurological symptoms [2]. In fact, giving folic acid alone in B12 deficiency can "mask" the anemia while allowing the neurological damage to progress irreversibly. * **Erythropoietin:** This is used for anemia of chronic kidney disease or certain malignancies; it has no role in nutritional macrocytic anemia. * **Iron dextran:** This is used for iron-deficiency anemia (microcytic), not macrocytic anemia. **Clinical Pearls for NEET-PG:** * **The "Folate Trap":** Always rule out B12 deficiency before starting Folic acid. * **Earliest Sign:** Hypersegmented neutrophils on a peripheral smear are the earliest sign of megaloblastic anemia. * **Biochemical Markers:** In B12 deficiency, both **Methylmalonic Acid (MMA)** and **Homocysteine** levels are elevated. In Folate deficiency, only Homocysteine is elevated. * **Neurological involvement:** Only B12 deficiency causes neurological symptoms; Folate deficiency does not. [1]

Q: Megaloblastic anemia may be caused by all of the following, except?

Long term aspirin intake. **Explanation:** Megaloblastic anemia is characterized by impaired DNA synthesis, leading to a maturation delay between the nucleus and the cytoplasm (nuclear-cytoplasmic asynchrony). [1] This results in large, nucleated RBC precursors in the bone marrow and macrocytic cells in the peripheral blood. [1] **Why Option D is Correct:** **Long-term aspirin intake** is associated with gastrointestinal bleeding and chronic blood loss, which typically leads to **Iron Deficiency Anemia (Microcytic Hypochromic)**. Aspirin does not interfere with DNA synthesis or the metabolism of Vitamin B12 or Folate; therefore, it does not cause megaloblastic changes. **Why the other options are incorrect:** * **Vitamin B12 and Folic Acid Deficiency (Options B & C):** These are the most common causes. [1] Both are essential cofactors for DNA synthesis. B12 is required for the conversion of homocysteine to methionine, and Folate is required for thymidylate synthesis. [1] Deficiency leads to ineffective erythropoiesis. * **Dilantin (Phenytoin) Toxicity (Option A):** Phenytoin is a well-known cause of drug-induced megaloblastic anemia. It interferes with folate metabolism by inhibiting intestinal folate conjugase (reducing absorption) and increasing the catabolism of folate. **NEET-PG High-Yield Pearls:** 1. **Drug-induced Megaloblastic Anemia:** Common culprits include **Methotrexate** (DHFR inhibitor), **Phenytoin**, **Pyrimethamine**, **Trimethoprim**, and **Zidovudine (AZT)**. 2. **Pernicious Anemia:** The most common cause of B12 deficiency, caused by autoimmune destruction of gastric parietal cells (leading to Intrinsic Factor deficiency). [1] 3. **Peripheral Smear Findings:** Look for **Hypersegmented Neutrophils** (>5 lobes) and **Macro-ovalocytes**. 4. **Biochemical Markers:** Both B12 and Folate deficiency show elevated **Homocysteine**, but only B12 deficiency shows elevated **Methylmalonic Acid (MMA)**.

Q: Cryoprecipitate is a rich source of which of the following?

Factor VIII. **Explanation:** Cryoprecipitate is a blood product prepared by thawing one unit of Fresh Frozen Plasma (FFP) at 1–6°C and collecting the insoluble precipitate. It is a concentrated source of specific clotting factors, making it the treatment of choice for specific consumptive coagulopathies [1]. **Why Factor VIII is correct:** Cryoprecipitate is specifically enriched with five key components [1]: 1. **Factor VIII** (Anti-hemophilic factor) 2. **Fibrinogen** (Factor I) – It is the most concentrated source available. 3. **von Willebrand Factor (vWF)** 4. **Factor XIII** (Fibrin stabilizing factor) 5. **Fibronectin** **Why the other options are incorrect:** * **Option A (Thromboplastin):** Also known as Factor III or Tissue Factor, it is not a plasma-derived component found in cryoprecipitate; it is released from damaged tissues to initiate the extrinsic pathway. * **Option C (Factor X) and Option D (Factor VII):** These are vitamin K-dependent clotting factors. Along with Factors II and IX, these remain in the supernatant (the "cryo-poor" plasma) after the cryoprecipitate is removed [1]. To replace these, one would use FFP or Prothrombin Complex Concentrate (PCC). **High-Yield Clinical Pearls for NEET-PG:** * **Primary Indication:** Hypofibrinogenemia (e.g., in DIC or massive hemorrhage). * **Dosage:** One bag of cryoprecipitate typically raises the fibrinogen level by 5–10 mg/dL. * **Storage:** It is stored at -18°C or colder and has a shelf life of 1 year. Once thawed, it must be used within 6 hours (or 4 hours if pooled). * **Note on Hemophilia A:** While it contains Factor VIII, recombinant or plasma-derived Factor VIII concentrates are now preferred over cryoprecipitate to reduce the risk of transfusion-transmitted infections.

Question 1

A 22-year-old woman from a large Italian family is screened for a familial blood disorder. Laboratory studies reveal a hemoglobin of 9.5 g/dL and a peripheral smear showing mild microcytosis, hypochromia, and a few target cells. Hemoglobin electrophoresis demonstrates a mild increase in hemoglobin A2 (7.5%). What is the most likely diagnosis?

Accepted Answer

Heterozygous beta-thalassemia

Answer

Anemia of chronic disease

Answer

G6PD deficiency

Answer

Homozygous beta-thalassemia

Question 2

The use of desmopressin is best indicated for therapy in which of the following bleeding disorders?

Accepted Answer

Von Willebrand disease (VWD)

Answer

Severe hemophilia A

Answer

Severe hemophilia B

Answer

Glanzmann thrombasthenia

Question 3

Which drug is used for the treatment of Acute Myeloid Leukemia (AML) with FLT-3 mutation?

Accepted Answer

Gilteritinib

Answer

Crizotinib

Answer

Acalabrutinib

Answer

Ibrutinib

Question 4

A 58-year-old woman presents with a 6-month history of backache and recurrent chest infections. She develops sudden weakness of the legs and urinary retention. Investigations reveal a hemoglobin of 7.3 g/dL, serum calcium of 12.6 mg/dL, phosphate of 2.5 mg/dL, alkaline phosphatase of 100 U/L, serum albumin of 3 g/dL, globulin of 7.1 g/dL, and urea of 178 mg/dL. What is the most likely diagnosis?

Accepted Answer

Multiple myeloma

Answer

Lung cancer

Answer

Disseminated tuberculosis

Answer

Osteoporosis

Question 5

All of the following conditions are acquired causes of iron overload, EXCEPT:

Accepted Answer

Hypothyroidism

Answer

Sideroblastic anemia

Answer

Chronic Hepatitis C

Answer

Nonalcoholic steatohepatitis

Question 6

Which of the following statements is FALSE regarding hemochromatosis?

Accepted Answer

Prone to Mycobacterium enteric infections

Answer

Prone to fatal Vibrio enteric infections

Answer

Prone to fatal Yersinia enteric infections

Answer

Should avoid raw fish

Question 7

Which of the following is preferred in an elderly woman with macrocytic anemia and early signs of neurological deficit?

Accepted Answer

Hydroxycobalamine

Answer

Folic acid

Answer

Erythropoietin

Answer

Iron dextran

Question 8

Megaloblastic anemia may be caused by all of the following, except?

Accepted Answer

Long term aspirin intake

Answer

Dilantin toxicity

Answer

Vitamin B12 deficiency

Answer

Folic acid deficiency

Question 9

Cryoprecipitate is a rich source of which of the following?

Accepted Answer

Factor VIII

Answer

Thromboplastin

Answer

Factor X

Answer

Factor VII

Question 10

What is true about Thrombocytopenic purpura?

Accepted Answer

Thrombosis in cerebral blood vessels

Answer

Hemolysis is extravascular

Answer

Normal renal function tests

Answer

Immediate cure following plasmapheresis

Hematology — MCQs

Hematology — MCQs

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