Hematology Practice Questions

Q: A 23-year-old female presented with jaundice and pallor for 2 months. Her peripheral blood smear shows the presence of spherocytes. What is the most relevant investigation to arrive at a diagnosis?

Coombs test. **Explanation:** The presence of **spherocytes** on a peripheral blood smear in a patient with jaundice and pallor indicates extravascular hemolysis [1]. The two primary differential diagnoses for spherocytosis are **Hereditary Spherocytosis (HS)** and **Autoimmune Hemolytic Anemia (AIHA)** [1]. 1. **Why Coombs Test is the correct answer:** The most critical step in evaluating spherocytosis is to differentiate between an inherited membrane defect (HS) and an acquired immune-mediated process (AIHA). The **Direct Antiglobulin Test (Coombs test)** is the first-line investigation [1]. A positive result confirms AIHA, while a negative result points toward HS. Given the patient's age and presentation, ruling out an immune cause is the clinical priority [1]. 2. **Why other options are incorrect:** * **Osmotic Fragility Test:** While this test is classically associated with HS (showing increased fragility), it is **not specific**. Spherocytes in AIHA will also show increased osmotic fragility. Furthermore, it has been largely replaced by the Eosin-5-maleimide (EMA) binding test. * **Tests for PNH:** PNH typically presents with intravascular hemolysis and cytopenias [1]; the characteristic finding is "schistocytes" or normal morphology, not spherocytes. * **Reticulocyte Count:** This helps confirm that hemolysis is occurring (showing marrow response) but does not provide a specific diagnosis for the cause of spherocytosis. **Clinical Pearls for NEET-PG:** * **Spherocytes** lack central pallor because they have the lowest surface-area-to-volume ratio. * **MCHC** (Mean Corpuscular Hemoglobin Concentration) is typically **increased** (>36 g/dL) in spherocytosis [1]. * If the Coombs test is negative, the most sensitive modern test for Hereditary Spherocytosis is the **EMA Binding Test** (Flow cytometry).

Q: What is the most common site of thrombosis in paroxysmal nocturnal hemoglobinuria?

Intra-abdominal veins. **Explanation:** **Paroxysmal Nocturnal Hemoglobinuria (PNH)** is an acquired clonal hematopoietic stem cell disorder characterized by a deficiency of glycosylphosphatidylinositol (GPI)-anchored proteins (like CD55 and CD59) due to a mutation in the **PIGA gene**. This deficiency leads to uncontrolled complement-mediated hemolysis and a profound prothrombotic state. **Why Intra-abdominal veins are correct:** Thrombosis is the leading cause of mortality in PNH (accounting for ~40% of deaths). Unlike the general population, where deep vein thrombosis (DVT) of the legs is most common, PNH has a unique predilection for **atypical sites**. The **intra-abdominal veins** (specifically the **hepatic vein**, leading to Budd-Chiari syndrome) are the most common sites of thrombosis. Other frequent sites include the portal, splenic, and mesenteric veins, as well as cerebral dural sinuses. **Why other options are incorrect:** * **A & D (Femoral and Saphenous veins):** While DVT can occur in PNH, these are the "typical" sites for thrombosis in the general population (e.g., post-surgery or immobilization). They are not the *most characteristic* or most common sites specific to the PNH disease process. * **B (Median cubital vein):** This is a superficial vein of the upper limb, usually associated with phlebitis due to IV cannulation, not the systemic hypercoagulable state of PNH. **High-Yield Clinical Pearls for NEET-PG:** * **Gold Standard Diagnosis:** Flow cytometry (shows absence of CD55/CD59). * **Classic Triad:** Hemolytic anemia, Pancytopenia, and Venous thrombosis. * **Treatment of Choice:** Eculizumab (a monoclonal antibody against C5 complement). * **Screening:** Always screen for PNH in patients presenting with unexplained Budd-Chiari syndrome or mesenteric ischemia.

Q: Which of the following factors can differentiate primary polycythemia from secondary polycythemia?

Alveolar oxygen tension <90% on room air. The fundamental difference between primary and secondary polycythemia lies in the **erythropoietin (EPO) drive**. **Primary Polycythemia (Polycythemia Vera)** is a myeloproliferative neoplasm (usually due to a JAK2 mutation) where the bone marrow produces excess red cells autonomously, independent of EPO levels [1]. In fact, EPO levels are typically low. **Secondary Polycythemia** is a physiological or pathological response to **hypoxia**. When arterial oxygen saturation drops (e.g., chronic lung disease, high altitude, or cyanotic heart disease), the kidneys sense the hypoxia and increase EPO production to stimulate erythropoiesis [1]. Therefore, **Alveolar oxygen tension (SaO2) <92% (or <90% in some clinical contexts)** is a hallmark of secondary polycythemia caused by hypoxemia. **Analysis of Incorrect Options:** * **A. Increased red cell mass:** This is the definition of absolute polycythemia and is present in **both** primary and secondary forms; hence, it cannot differentiate them [1]. * **C. Pneumonitis:** While lung inflammation can lead to hypoxia, it is a specific clinical condition rather than a diagnostic parameter used to classify polycythemia. * **D. Hypertension:** Hypertension is a common finding in Polycythemia Vera (due to hyperviscosity) but can also be seen in patients with secondary causes like Obstructive Sleep Apnea (OSA) or renal artery stenosis [1]. It is not a specific differentiating factor. **NEET-PG High-Yield Pearls:** * **JAK2 V617F mutation:** Present in >95% of Polycythemia Vera cases [1]. * **EPO Levels:** Low in Primary; High in Secondary polycythemia. * **Splenomegaly:** Highly suggestive of Primary Polycythemia (PV) [1]. * **Gaisbock Syndrome:** "Stress polycythemia" where red cell mass is normal but plasma volume is decreased (Relative Polycythemia) [1].

Q: What is the most likely subtype of leukemia associated with the provided finding?

AML-M3. ***AML-M3*** - The finding shows **faggot cells** (promyelocytes with multiple bundled **Auer rods**), which is **pathognomonic** for AML-M3 (Acute Promyelocytic Leukemia). - Associated with **t(15;17) chromosomal translocation** and **PML-RARA fusion gene**, making it highly responsive to **all-trans retinoic acid (ATRA)** therapy. *B cell ALL* - Characterized by **lymphoblasts** with high nuclear-to-cytoplasmic ratio and **absent Auer rods**. - Typically affects **children** and presents with **lymphadenopathy** and **hepatosplenomegaly**. *AML-M4* - Shows **myeloblasts** and **monoblasts** with single Auer rods, not the characteristic **bundled Auer rods** seen in faggot cells. - Associated with **inv(16)** or **t(16;16)** chromosomal abnormalities and **eosinophilia**. *AML-M5* - Predominantly contains **monoblasts** and **promonocytes** with **folded nuclei** and abundant cytoplasm. - Auer rods are **rare or absent**, and cells lack the distinctive **faggot cell** morphology of AML-M3.

Q: Plasmapheresis is recommended in all of the following conditions except?

Chronic active hepatitis. Plasmapheresis (Therapeutic Plasma Exchange) is a procedure used to remove high-molecular-weight substances, such as autoantibodies, immune complexes, or paraproteins, from the plasma. **Why Chronic Active Hepatitis is the correct answer:** Chronic active hepatitis is primarily an inflammatory and fibrotic liver disease managed with immunosuppressants (like corticosteroids) [1] or antiviral therapy [2]. Plasmapheresis has no established therapeutic role in its management as the pathology is not driven by circulating macromolecules that require mechanical removal. **Analysis of other options:** * **Hyperviscosity Syndrome:** This is a classic indication. Plasmapheresis rapidly reduces blood viscosity by removing excess proteins (usually IgM), preventing complications like stroke or retinal hemorrhage. * **Macroglobulinaemia (Waldenström’s):** This condition involves high levels of monoclonal IgM. Because IgM is large and mostly intravascular (80%), plasmapheresis is highly effective at removing it to treat associated hyperviscosity. * **Immune Complex Glomerulonephritis:** Conditions like Goodpasture’s syndrome or rapidly progressive glomerulonephritis (RPGN) utilize plasmapheresis to physically remove circulating nephrotoxic autoantibodies or immune complexes to prevent irreversible renal damage. **NEET-PG High-Yield Pearls:** * **ASFA Category I Indications (First-line):** Guillain-Barré Syndrome (GBS), Myasthenia Gravis (crisis), TTP (Thrombotic Thrombocytopenic Purpura), and Goodpasture’s Syndrome. * **TTP Fact:** Plasmapheresis is the treatment of choice for TTP; it removes ADAMTS13 inhibitors and replenishes the enzyme. * **IgM vs. IgG:** Plasmapheresis is more efficient for IgM-mediated diseases (like Waldenström’s) because IgM is primarily intravascular, whereas IgG is distributed in the extravascular space.

Q: A 60-year-old man presents with a 6-month history of increasing fatigue. Physical examination reveals marked pallor, and a CBC shows a macrocytic anemia. Which of the following is the most likely cause of anemia in this patient?

Alcoholism. ### Explanation **Correct Option: A. Alcoholism** The key to this question lies in the **MCV (Mean Corpuscular Volume)**. The patient presents with **macrocytic anemia** (MCV >100 fL). Alcoholism is one of the most common causes of non-megaloblastic macrocytosis [1]. Alcohol has a direct toxic effect on the bone marrow (interfering with erythrocyte maturation) and is often associated with poor nutrition, leading to secondary **folate deficiency** [1]. In chronic alcoholics, macrocytosis can occur even in the absence of anemia or liver disease [3]. **Why Incorrect Options are Wrong:** * **B. Chronic Disease:** Anemia of Chronic Disease (ACD) is typically **normocytic, normochromic**, though it can become microcytic in long-standing cases [2], [4]. It is never macrocytic. * **C. Iron Deficiency:** This is the most common cause of **microcytic, hypochromic** anemia (MCV <80 fL) due to impaired hemoglobin synthesis [2]. * **D. Renal Disease:** Anemia in chronic kidney disease (CKD) is primarily due to decreased erythropoietin production [2]. It is characteristically **normocytic and normochromic**. **NEET-PG High-Yield Pearls:** 1. **Macrocytic Anemia Classification:** * **Megaloblastic:** (Hypersegmented neutrophils present) – Vitamin B12 and Folate deficiency [1], [5]. * **Non-megaloblastic:** Alcoholism, Liver disease, Hypothyroidism, and Myelodysplastic Syndrome (MDS). 2. **Alcohol & MCV:** An unexplained increase in MCV is often an early biochemical marker of occult alcohol abuse. 3. **Round vs. Oval:** Macrocytes in alcoholism/liver disease are usually **round**, whereas in B12/Folate deficiency, they are **oval** (macro-ovalocytes).

Q: What is the commonest acute presentation of sickle cell anemia?

Bone pain. **Explanation:** **Correct Answer: B. Bone pain** **Why Bone Pain is Correct:** The hallmark of Sickle Cell Anemia (SCA) is the **Vaso-occlusive Crisis (VOC)**. Under conditions of hypoxia, acidosis, or dehydration, HbS polymerizes, causing RBCs to become sickle-shaped [2]. These rigid cells obstruct microvasculature, leading to tissue ischemia and infarction [2]. The **most common** site for these crises is the bone marrow, manifesting as severe, acute bone pain [1]. In infants, this often presents as **Dactylitis** (Hand-foot syndrome), while in older children and adults, it typically affects long bones, ribs, and the spine. **Analysis of Incorrect Options:** * **A. Priapism:** While a well-known and serious complication of SCA due to venous stasis in the corpora cavernosa, it is far less frequent than bone pain. * **C. Fever:** Fever is a common *trigger* or associated symptom of a crisis (often due to underlying infection), but it is not the primary presentation of the sickle cell process itself [2, 3]. * **D. Splenomegaly:** In early childhood, the spleen may be enlarged. However, due to repeated infarctions, the spleen eventually undergoes fibrosis and shrinkage, a process known as **Autosplenectomy**. This is a chronic sequela rather than a common acute presentation. **High-Yield Clinical Pearls for NEET-PG:** * **Most common cause of death (Adults):** Acute Chest Syndrome [1]. * **Most common cause of death (Children):** *Streptococcus pneumoniae* sepsis (due to functional asplenia). * **Osteomyelitis in SCA:** While *Staphylococcus aureus* is the most common cause of osteomyelitis generally, **Salmonella** is uniquely associated with SCA. * **Radiology:** "Hair-on-end" appearance on skull X-ray and "H-shaped" vertebrae (Codfish vertebrae) due to central endplate infarction.

Question 1

A 23-year-old female presented with jaundice and pallor for 2 months. Her peripheral blood smear shows the presence of spherocytes. What is the most relevant investigation to arrive at a diagnosis?

Accepted Answer

Coombs test

Answer

Tests for paroxysmal nocturnal hemoglobinuria (PNH)

Answer

Osmotic fragility test

Answer

Reticulocyte count

Question 2

What is the most common site of thrombosis in paroxysmal nocturnal hemoglobinuria?

Accepted Answer

Intra-abdominal veins

Answer

Femoral vein

Answer

Median cubital vein

Answer

Saphenous vein

Question 3

A 67-year-old man presents with 5 months of increasing weakness, fatigue, and weight loss. He has experienced decreasing vision in both eyes, headaches, and dizziness. His hands are sensitive to cold. Physical examination reveals generalized lymphadenopathy and hepatosplenomegaly. Laboratory studies show a serum protein level of 15.5 g/dL and an albumin concentration of 3.2 g/dL. A bone marrow biopsy shows infiltration by numerous small plasmacytoid lymphoid cells with Russell bodies in the cytoplasm. Which of the following additional laboratory findings is most likely to be reported for this patient?

Accepted Answer

Monoclonal IgM spike in serum

Answer

Bence Jones proteinuria

Answer

Hypercalcemia

Answer

Karyotype with t(14;18) translocation

Question 4

Which of the following factors can differentiate primary polycythemia from secondary polycythemia?

Accepted Answer

Alveolar oxygen tension <90% on room air

Answer

Increased red cell mass

Answer

Pneumonitis

Answer

Hypertension

Question 5

What is the most likely subtype of leukemia associated with the provided finding?

Accepted Answer

AML-M3

Answer

B cell ALL

Answer

AML-M4

Answer

AML-M5

Question 6

Plasmapheresis is recommended in all of the following conditions except?

Accepted Answer

Chronic active hepatitis

Answer

Hyperviscosity syndrome

Answer

Macroglobulinaemia

Answer

Immune complex glomerulonephritis

Question 7

A 60-year-old man presents with a 6-month history of increasing fatigue. Physical examination reveals marked pallor, and a CBC shows a macrocytic anemia. Which of the following is the most likely cause of anemia in this patient?

Accepted Answer

Alcoholism

Answer

Chronic disease

Answer

Iron deficiency

Answer

Renal disease

Question 8

What is the commonest acute presentation of sickle cell anemia?

Accepted Answer

Bone pain

Answer

Priapism

Answer

Fever

Answer

Splenomegaly

Question 9

Which of the following conditions has the least impact on life expectancy since the point of diagnosis?

Accepted Answer

Chronic lymphoid leukemia

Answer

Chronic myeloid leukemia

Answer

Acute myeloid leukemia

Answer

Acute lymphoid leukemia

Question 10

A 48-year-old woman presents with a 3-week history of fatigue, yellow skin, and yellow sclerae. Physical examination reveals mild jaundice. Her serum bilirubin level is 3.7 mg/dL, predominantly unconjugated. Liver function tests, including serum AST, ALT, and alkaline phosphatase, are normal. Hemoglobin level is 6.0 g/dL. Jaundice resolves after corticosteroid administration. What is the most likely cause of hyperbilirubinemia in this patient?

Accepted Answer

Autoimmune hemolytic anemia

Answer

Acute hepatitis B infection

Answer

Gallstone in the common bile duct

Answer

Primary biliary cirrhosis

Hematology — MCQs

Hematology — MCQs

On this page

Practice by Chapter

Want unlimited practice?