Hematology Practice Questions

Q: All of the following disorders are inherited except?

Antiphospholipid antibody syndrome. **Explanation:** The core concept tested here is the classification of **Thrombophilia** into inherited (genetic) and acquired causes. **Why Antiphospholipid Antibody Syndrome (APS) is the correct answer:** APS is an **acquired** autoimmune hypercoagulable state. It is characterized by the presence of clinical features (venous/arterial thrombosis or pregnancy morbidity) and persistent laboratory evidence of antiphospholipid antibodies (Lupus anticoagulant, Anti-cardiolipin, or Anti-̢2 glycoprotein I). Unlike the other options, it is not caused by a single gene mutation passed through families, though it may be associated with other autoimmune diseases like SLE. **Why the other options are incorrect:** * **Factor V Leiden Mutation:** This is the **most common inherited cause** of hypercoagulability. It involves a point mutation (G1691A) that makes Factor V resistant to inactivation by activated Protein C. * **Protein C & Protein S Deficiency:** Both are **inherited** (usually autosomal dominant) deficiencies of natural anticoagulants [1]. Protein C inactivates Factors Va and VIIIa; Protein S acts as a cofactor for Protein C. **High-Yield Clinical Pearls for NEET-PG:** * **Most common inherited thrombophilia:** Factor V Leiden. * **Most common acquired thrombophilia:** Antiphospholipid Antibody Syndrome. * **Warfarin-induced skin necrosis:** Classically seen in patients with **Protein C deficiency** when starting Warfarin without heparin bridging. * **Screening for APS:** Requires two positive lab tests at least 12 weeks apart to confirm "persistence" of antibodies.

Q: A 70-year-old male presents with excessive fatigue, fever, and bleeding from gums while brushing. On examination, he has splenomegaly. CBC shows marked pancytopenia with the presence of blasts. A t(15;17)(q22;q12) cytogenetic rearrangement is found in this patient. Which of the following agents will you use in the management of this patient?

Tretinoin. ### Explanation **Diagnosis: Acute Promyelocytic Leukemia (APL) - AML M3** The clinical presentation of pancytopenia, blasts, and the pathognomonic **t(15;17)** translocation confirms the diagnosis of **Acute Promyelocytic Leukemia (APL)** [1]. This translocation involves the **PML** (Promyelocytic Leukemia) gene on chromosome 15 and the **RAR-$\alpha$** (Retinoic Acid Receptor-alpha) gene on chromosome 17. The resulting fusion protein blocks myeloid differentiation at the promyelocyte stage. **Why Tretinoin (All-Trans Retinoic Acid - ATRA) is correct:** ATRA is the cornerstone of APL management. It acts by binding to the PML-RAR$\alpha$ fusion protein, inducing the **differentiation** of leukemic promyelocytes into mature neutrophils. When combined with Arsenic Trioxide (ATO) or chemotherapy (Anthracyclines), it leads to high cure rates. **Why other options are incorrect:** * **Acyclovir:** An antiviral used for Herpes Simplex or Varicella-Zoster; it has no role in treating leukemia. * **Daunorubicin:** While anthracyclines are used in AML induction, ATRA is the specific targeted therapy for the t(15;17) mutation [1]. In modern protocols (low-risk APL), ATRA + Arsenic is often preferred over cytotoxic chemotherapy. * **Rituximab:** A monoclonal antibody against **CD20**, used in B-cell lymphomas and CLL, not in AML/APL. **High-Yield Clinical Pearls for NEET-PG:** * **DIC Risk:** APL is a medical emergency due to the high risk of **Disseminated Intravascular Coagulation (DIC)** triggered by the release of procoagulants from granules. * **Differentiation Syndrome:** A side effect of ATRA/Arsenic treatment characterized by fever, pulmonary infiltrates, and weight gain. It is managed with **Dexamethasone**. * **Auer Rods:** Classically seen in abundance ("faggot cells") in APL. * **Targeted Therapy:** APL is the first example of "differentiation therapy" in oncology.

Q: In polycythaemia vera, what is the most common postoperative complication following major surgery?

Thrombosis. **Explanation:** **Polycythemia Vera (PV)** is a chronic myeloproliferative neoplasm characterized by the autonomous overproduction of red blood cells, often driven by the **JAK2 V617F mutation** [2]. **Why Thrombosis is the correct answer:** The primary pathophysiology in PV involves a significant increase in red cell mass, leading to **hyperviscosity** and slowed blood flow. Additionally, qualitative abnormalities in platelets and increased interaction between leukocytes and the endothelium create a prothrombotic state. Major surgery further exacerbates this risk due to immobilization and systemic inflammation [1]. Consequently, **thrombosis** (both arterial and venous) is the most common and life-threatening postoperative complication [2]. **Analysis of Incorrect Options:** * **B. Gastric Ulcer:** While PV patients have an increased incidence of peptic ulcer disease (due to increased histamine release from basophils), it is a chronic association rather than a specific postoperative complication. * **C. Diabetes Insipidus:** This is not associated with PV. It typically results from ADH deficiency or resistance, unrelated to hyperviscosity. * **D. Haemorrhage:** Paradoxically, PV patients *are* at risk of bleeding (often due to acquired von Willebrand syndrome at very high platelet counts), but statistically, **thrombotic events** occur more frequently and represent the leading cause of morbidity post-surgery [2]. **High-Yield Clinical Pearls for NEET-PG:** * **Target Hematocrit:** To minimize surgical risk, the hematocrit should be controlled to **<45%** (ideally for several months) prior to elective surgery. * **Pruritus:** Characteristically "aquagenic" (itching after a warm bath) due to mast cell degranulation [2]. * **Spent Phase:** PV can progress to myelofibrosis or transform into Acute Myeloid Leukemia (AML). * **Treatment of choice:** Phlebotomy and low-dose aspirin; Hydroxyurea is used for high-risk patients.

Q: Cryoglobulinemia is associated with which of the following viruses?

Hepatitis C Virus (HCV). Cryoglobulinemia refers to the presence of abnormal proteins (immunoglobulins) in the blood that precipitate at temperatures below 37°C and dissolve upon rewarming. **Why Hepatitis C Virus (HCV) is the Correct Answer:** Hepatitis C is the most common cause of **Mixed Cryoglobulinemia (Type II and III)**. Chronic HCV infection leads to persistent antigenic stimulation, causing B-cell proliferation and the production of monoclonal or polyclonal IgM antibodies with rheumatoid factor activity [1]. These antibodies bind to IgG and HCV RNA to form immune complexes that deposit in small-to-medium vessels, leading to vasculitis. Up to 80-90% of mixed cryoglobulinemia cases are associated with chronic HCV infection [1]. **Why Other Options are Incorrect:** * **Hepatitis A (HAV):** This is an acute, self-limiting infection and does not cause the chronic immune stimulation required for cryoglobulin production. * **Hepatitis B (HBV):** While HBV is strongly associated with **Polyarteritis Nodosa (PAN)**, it is a much less common cause of cryoglobulinemia compared to HCV. * **Hepatitis D (HDV):** HDV requires HBV for replication; it is associated with severe liver disease but not specifically with cryoglobulinemic vasculitis. **High-Yield Clinical Pearls for NEET-PG:** * **Meltzer’s Triad:** The classic clinical presentation of cryoglobulinemia includes **Purpura, Arthralgia, and Myasthenia (weakness)**. * **Complement Levels:** Characteristically shows **low C4** levels with normal or slightly low C3. * **Renal Involvement:** Most commonly presents as Membranoproliferative Glomerulonephritis (MPGN). * **Treatment:** The primary goal is treating the underlying HCV infection (e.g., Direct-Acting Antivirals). Plasmapheresis and Rituximab are used in severe vasculitic crises.

Q: A 60-year-old person presents with a history of angina and shortness of breath for the past week. Blood withdrawn shows a thick brownish-red color. What is the most likely diagnosis?

Methemoglobinemia. ### Explanation **Correct Answer: C. Methemoglobinemia** **Medical Concept:** Methemoglobinemia occurs when the iron in hemoglobin is oxidized from the **ferrous state (Fe²⁺)** to the **ferric state (Fe³⁺)**. Ferric iron cannot bind oxygen, and its presence shifts the oxygen dissociation curve to the left, hindering oxygen release to tissues [1]. This leads to functional anemia and cellular hypoxia, manifesting as **angina and shortness of breath**, even with normal PaO₂ levels. The classic physical hallmark is the **"chocolate-colored" or brownish-red appearance of blood**, which does not change color even when exposed to 100% oxygen (unlike deoxygenated blood in cyanosis). **Why Incorrect Options are Wrong:** * **A & B (Sickle cell & Hemolytic anemia):** While these cause anemia and shortness of breath, the blood remains bright red when oxygenated. Hemolysis may show icteric plasma, but not brownish-red whole blood. * **D (G-6-PD deficiency):** This leads to episodic hemolysis (often triggered by drugs or fava beans) [2]. While G-6-PD deficiency can coexist with drug-induced methemoglobinemia (e.g., from Primaquine), the specific "brownish-red blood" is the pathognomonic sign of methemoglobinemia itself. **NEET-PG High-Yield Pearls:** * **Clinical Sign:** "Chocolate-colored blood" + Central cyanosis that does not improve with oxygen [1]. * **Diagnosis:** A "Saturation Gap" (difference >5% between oxygen saturation measured by pulse oximetry and that calculated by ABG). * **Antidote:** **Methylene Blue** (Treatment of choice). Note: Methylene blue is contraindicated in patients with G-6-PD deficiency (use Vitamin C instead). * **Common Triggers:** Nitrites, Benzocaine, Dapsone, and Sulfonamides [1].

Q: What is the difference between hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP)?

Central nervous system manifestations are dominant in TTP.. **Explanation:** Both **Thrombotic Thrombocytopenic Purpura (TTP)** and **Hemolytic Uremic Syndrome (HUS)** belong to the spectrum of Microangiopathic Hemolytic Anemias (MAHA) [1]. While they share many clinical features, their primary target organs differ due to their distinct pathophysiologies. **1. Why Option B is Correct:** TTP is primarily caused by a deficiency in the **ADAMTS13** enzyme (a vWF-cleaving protease), leading to large vWF multimers that cause widespread microthrombi. While multi-systemic, TTP classically presents with **dominant Central Nervous System (CNS) manifestations** (confusion, seizures, focal deficits). In contrast, HUS—typically caused by Shiga toxin-producing *E. coli* (O157:H7)—is characterized by **dominant Renal Failure** [1], [2]. **2. Why the other options are incorrect:** * **Option A:** Thrombocytopenia is a hallmark of **both** conditions due to the consumption of platelets in microthrombi formation [2]. * **Option C:** MAHA (evidenced by schistocytes on a peripheral smear) is the **defining feature of both** TTP and HUS [1]. * **Option D:** This is the reverse of clinical reality. Renal failure is the **predominant** feature of HUS, whereas it is usually mild or secondary in TTP [2]. **High-Yield Clinical Pearls for NEET-PG:** * **The TTP Pentad (FAT RN):** **F**ever, **A**nemia (MAHA), **T**hrombocytopenia, **R**enal failure, **N**eurological symptoms. (Note: The full pentad is seen in <25% of patients). * **The HUS Triad:** MAHA, Thrombocytopenia, and Acute Renal Failure [2]. * **Treatment:** **Plasmapheresis (Plasma Exchange)** is the gold standard for TTP. Antibiotics and anti-motility agents are generally avoided in Shiga-toxin HUS as they may worsen toxin release. * **Lab Clue:** Coagulation profiles (PT/aPTT) are typically **normal** in TTP/HUS, distinguishing them from DIC.

Question 1

All of the following disorders are inherited except?

Accepted Answer

Antiphospholipid antibody syndrome

Answer

Protein S deficiency

Answer

Protein C deficiency

Answer

Factor V Leiden mutation

Question 2

A 70-year-old male presents with excessive fatigue, fever, and bleeding from gums while brushing. On examination, he has splenomegaly. CBC shows marked pancytopenia with the presence of blasts. A t(15;17)(q22;q12) cytogenetic rearrangement is found in this patient. Which of the following agents will you use in the management of this patient?

Accepted Answer

Tretinoin

Answer

Acyclovir

Answer

Daunorubicin

Answer

Rituximab

Question 3

Purpura is a feature of which of the following conditions?

Accepted Answer

Disseminated Intravascular Coagulation

Answer

Henoch Schonlein Purpura

Answer

Meningococcemia

Answer

Schamberg's disease

Question 4

Which of the following statements regarding polycythemia vera are true?

Accepted Answer

a) The JAK2 gene is located on the short arm of chromosome 9; b) Splenomegaly is an early presenting sign in PV; c) Venous and arterial thrombosis may be the presenting manifestation of PV; d) Red cell mass and plasma volume determinations are mandatory to establish the presence of an absolute erythrocytosis; e) A bone marrow aspirate and biopsy provide specific diagnostic information.

Question 5

In polycythaemia vera, what is the most common postoperative complication following major surgery?

Accepted Answer

Thrombosis

Answer

Gastric ulcer

Answer

Diabetes insipidus

Answer

Haemorrhage

Question 6

Cryoglobulinemia is associated with which of the following viruses?

Accepted Answer

Hepatitis C Virus (HCV)

Answer

Hepatitis A Virus (HAV)

Answer

Hepatitis B Virus (HBV)

Answer

Hepatitis D Virus (HDV)

Question 7

A 60-year-old person presents with a history of angina and shortness of breath for the past week. Blood withdrawn shows a thick brownish-red color. What is the most likely diagnosis?

Accepted Answer

Methemoglobinemia

Answer

Sickle cell anemia

Answer

Hemolytic anemia

Answer

G-6-PD deficiency

Question 8

Regarding oral iron supplements used for iron deficiency anemia, which of the following statements is correct?

Accepted Answer

A tolerable dose will deliver 40 to 60 mg of elemental iron per day.

Answer

The mass of the total salt is important in determining the daily dose.

Answer

Treatment should be stopped as soon as normal hemoglobin levels are reached.

Answer

The desired rate of hemoglobin improvement is 0.5 g/dL per week.

Question 9

What is the difference between hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP)?

Accepted Answer

Central nervous system manifestations are dominant in TTP.

Answer

Thrombocytopenia is seen in TTP.

Answer

Microangiopathic hemolytic anemia is present in both.

Answer

Renal failure is more prominent in TTP.

Question 10

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 gm/dL, serum calcium of 12.6 mg/dL, phosphate of 2.5 mg/dL, alkaline phosphatase of 100 U/L, serum albumin of 3 gm/dL, globulin of 7.1 gm/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

Hematology — MCQs

Hematology — MCQs

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