Hematology Practice Questions

Q: Which of the following types of multiple myeloma is most commonly associated with an increased risk of hyperviscosity syndrome?

IgM. ### Explanation **Correct Option: A (IgM)** Hyperviscosity syndrome (HVS) is most frequently associated with **IgM paraproteinemia** [1]. This is due to the unique structural properties of IgM: it is a large **pentameric** molecule with a high molecular weight (approx. 900,000 Daltons). Because of its size and high intrinsic viscosity, it significantly increases blood resistance to flow even at relatively low concentrations. While IgM is the hallmark of Waldenström Macroglobulinemia, "IgM Myeloma" is a rare but distinct subtype of Multiple Myeloma that carries the highest risk of HVS among all myeloma types. **Incorrect Options:** * **B (IgG):** This is the most common subtype of Multiple Myeloma [2]. However, IgG is a small monomer; HVS usually only occurs if IgG levels exceed 10 g/dL, making it much less common than with IgM. * **C (IgD):** This is a rare subtype often associated with light chain excretion (Bence-Jones proteinuria) and renal failure, but not typically with hyperviscosity. * **D (IgA):** IgA is the second most common subtype. It can form polymers (dimers or trimers), which increases the risk of HVS more than IgG, but it still lacks the massive pentameric structure and viscosity potential of IgM. **NEET-PG High-Yield Pearls:** 1. **Clinical Triad of HVS:** Mucosal bleeding (epistaxis), visual disturbances ("sausage-link" or "box-car" appearance of retinal veins), and neurological symptoms (headache, vertigo, coma) [1]. 2. **Sia Water Test:** A bedside test where a drop of patient serum added to distilled water forms a precipitate (positive in Waldenström/IgM Myeloma). 3. **Treatment:** The immediate management of symptomatic hyperviscosity is **Plasmapheresis** to rapidly reduce the protein load [1]. 4. **Most Common Myeloma:** IgG > IgA > Light Chain > IgD.

Q: Autosplenectomy is a feature of which of the following conditions?

Sickle cell disease. **Explanation:** **1. Why Sickle Cell Disease (SCD) is correct:** Autosplenectomy is a hallmark of **Sickle Cell Anemia (HbSS)**. The underlying mechanism involves the repeated sickling of red blood cells in the hypoxic, acidic, and slow-flow environment of the splenic sinusoids [1]. These rigid, sickle-shaped cells cause microvascular occlusion (vaso-occlusion), leading to recurrent **splenic infarctions**. Over time, the splenic tissue is replaced by fibrous scar tissue, causing the spleen to shrink and become non-functional (fibrotic nubbin) [1]. This process is usually complete by age 5–8 in HbSS patients. **2. Why other options are incorrect:** * **Beta Thalassemia:** Unlike SCD, Beta Thalassemia typically presents with **massive splenomegaly**. This occurs due to extramedullary hematopoiesis and the constant clearance of abnormal RBCs (excess alpha chains) by the splenic macrophages. * **G6PD Deficiency:** This condition presents as episodic hemolysis triggered by oxidative stress (e.g., fava beans, drugs). It does not cause chronic vaso-occlusion or splenic infarction; therefore, the spleen size usually remains normal or slightly enlarged during acute episodes. **3. Clinical Pearls for NEET-PG:** * **Howell-Jolly Bodies:** The presence of these nuclear remnants on a peripheral smear is a classic sign of functional asplenia/autosplenectomy. * **Infection Risk:** Autosplenectomy increases susceptibility to **encapsulated organisms** (*S. pneumoniae, H. influenzae, N. meningitidis*). Prophylactic penicillin and vaccinations are mandatory. * **SCD vs. SC Disease:** While HbSS leads to autosplenectomy early, patients with **HbSC disease** or Sickle-Thalassemia may maintain an enlarged spleen into adulthood. * **Radiology:** On X-ray or CT, an autosplenectomized spleen may appear as a small, shrunken, and sometimes **calcified** mass in the left upper quadrant.

Q: Priapism can be due to which of the following conditions?

Chronic Myeloid Leukemia (CML). **Explanation:** **1. Why Chronic Myeloid Leukemia (CML) is correct:** Priapism (a prolonged, painful erection lasting >4 hours) is a known complication of CML, occurring in approximately 1–5% of male patients. The underlying mechanism is **Hyperleukocytosis** (Leukostasis). In CML, the massive elevation of the White Blood Cell (WBC) count—often exceeding 100,000/µL—increases blood viscosity. This leads to the sludging of leukemic cells within the *corpora cavernosa*, obstructing venous outflow and resulting in an ischemic, low-flow state. **2. Why the other options are incorrect:** * **Myelofibrosis:** While it is a myeloproliferative neoplasm, it is characterized by bone marrow fibrosis and cytopenias (or modest leukocytosis) rather than the extreme hyperleukocytosis seen in CML. Thus, it rarely causes leukostasis-induced priapism. * **Autoimmune Hemolytic Anemia (AIHA):** This involves the destruction of RBCs by antibodies. While Sickle Cell Anemia is a classic cause of priapism (due to sickled RBCs obstructing flow), AIHA does not cause the mechanical vascular obstruction required to trigger priapism. * **Thrombocytopenia:** A low platelet count leads to bleeding tendencies (epistaxis, petechiae), not the thrombotic or obstructive events that cause priapism. **Clinical Pearls for NEET-PG:** * **High-Yield Association:** Priapism is most commonly associated with **CML** among leukemias and **Sickle Cell Anemia** among hemoglobinopathies. * **Management:** In CML-induced priapism, the priority is reducing the WBC count via **Leukapheresis** and cytoreduction (e.g., Hydroxyurea) [1], alongside local urological intervention (aspiration/shunts). * **Leukostasis triad:** Often presents with CNS symptoms (headache/confusion), Respiratory distress (hypoxemia), and Priapism.

Q: A 30-year-old epileptic female on phenytoin presents with weakness and fatigue. Blood examination reveals Hb = 4.6 gm/dL, MCV = 102 fL, and MCH = 40 pg/dL. What is the most probable diagnosis?

Megaloblastic anemia. **Explanation:** The clinical presentation and laboratory findings point directly to **Megaloblastic Anemia**. [1] **1. Why the correct answer is right:** The patient has severe anemia (Hb 4.6 g/dL) with an elevated **Mean Corpuscular Volume (MCV = 102 fL)** and **Mean Corpuscular Hemoglobin (MCH = 40 pg/dL)**. An MCV >100 fL indicates **macrocytic anemia**. Phenytoin is a well-known cause of folate deficiency because it interferes with intestinal folate absorption and increases its catabolism. [1] Folate deficiency impairs DNA synthesis, leading to the characteristic "megaloblastic" changes in the bone marrow and macrocytosis in the peripheral blood. **2. Why the incorrect options are wrong:** * **A. Heart failure:** While severe anemia can lead to high-output heart failure, it is a complication, not the primary diagnosis for the hematological findings. [2] * **B. Iron deficiency anemia:** This typically presents as **microcytic hypochromic** anemia (MCV <80 fL, MCH <27 pg), which contradicts the macrocytosis seen here. [2] * **C. Phenytoin-induced agranulocytosis:** This refers to a severe reduction in white blood cell counts (neutrophils), not a macrocytic reduction in hemoglobin. **3. NEET-PG High-Yield Pearls:** * **Drug-induced Megaloblastic Anemia:** Common culprits include **Phenytoin**, Methotrexate, Trimethoprim, and Zidovudine. [1] * **Peripheral Smear:** Look for **hypersegmented neutrophils** (≥ 5 lobes) as the earliest sign of megaloblastic changes. * **Phenytoin Side Effects (Mnemonic: PHENYTOIN):** **P**-P450 inducer, **H**-Hyperplasia of gums, **E**-Erythroblasts (Megaloblastic anemia), **N**-Neuropathy, **Y**-Yellow-brown skin (pigmentation), **T**-Teratogenicity (Fetal Hydantoin Syndrome), **O**-Osteomalacia, **I**-Interference with B12/Folate, **N**-Nystagmus.

Q: A 30-year-old female presents with mucosal bleeding upon minor trauma and a history of menorrhagia. Both bleeding time and prothrombin time are elevated. The ristocetin assay is positive. What is the most likely diagnosis?

Von Willebrand disease. The correct answer is **Von Willebrand Disease (vWD)**. **1. Why Von Willebrand Disease is correct:** vWD is the most common inherited bleeding disorder. Von Willebrand Factor (vWF) has two primary roles: it acts as a bridge for platelet adhesion to subendothelial collagen (via GpIb) and serves as a carrier protein to stabilize **Factor VIII** [1]. * **Bleeding Time (BT):** Elevated because deficient vWF impairs primary hemostasis (platelet plug formation) [3]. * **Prothrombin Time (PT) / aPTT:** While the question mentions elevated PT, in classic vWD, the **aPTT** is more commonly prolonged due to low Factor VIII levels [1]. However, mucosal bleeding and a **positive Ristocetin assay** (which induces vWF-mediated platelet agglutination) are pathognomonic for vWD [1]. [2] **2. Why the other options are incorrect:** * **ITP (Option A):** Characterized by isolated thrombocytopenia. While BT is increased, PT and aPTT are strictly normal. * **Hemophilia A & B (Options B & C):** These are X-linked recessive disorders affecting the intrinsic pathway (Factor VIII and IX respectively). They typically present with deep-seated bleeds (hemarthrosis) rather than mucosal bleeding. In Hemophilia, BT is normal because platelet function is unaffected. **3. NEET-PG High-Yield Pearls:** * **Most common type:** Type 1 (Quantitative deficiency; Autosomal Dominant). * **Ristocetin Cofactor Activity:** The gold standard diagnostic test [1]. * **Treatment of choice:** **Desmopressin (DDAVP)**, which releases stored vWF from Weibel-Palade bodies in endothelial cells [1]. * **Clinical Clue:** Always suspect vWD in a female with menorrhagia and a normal platelet count but prolonged bleeding time [2].

Q: Pure red cell aplasia is associated with which of the following conditions?

Thymoma. **Explanation:** **Pure Red Cell Aplasia (PRCA)** is a rare hematological syndrome characterized by a severe reduction in erythroid precursors in the bone marrow, leading to isolated anemia with normal white cell and platelet counts [1]. **Why Thymoma is the Correct Answer:** The strongest clinical association with acquired PRCA is **Thymoma** (seen in approximately 10–15% of thymoma patients). The underlying mechanism is **autoimmune-mediated destruction** of erythroid progenitor cells. It is believed that T-cells or IgG antibodies directed against erythropoietin or erythroblasts are produced due to the loss of self-tolerance associated with thymic tumors. Surgical removal of the thymoma (thymectomy) can lead to remission of PRCA in many cases. **Why Other Options are Incorrect:** * **Renal Cell Carcinoma (RCC) & Hepatocellular Carcinoma (HCC):** These tumors are classically associated with **Paraneoplastic Erythrocytosis** (Polycythemia), not aplasia. They often secrete ectopic Erythropoietin (EPO), which stimulates red blood cell production. * **Prostate Carcinoma:** While it can cause anemia of chronic disease or leucoerythroblastic anemia (due to bone marrow metastasis), it has no specific association with PRCA. **High-Yield Clinical Pearls for NEET-PG:** * **Other Associations of PRCA:** Parvovirus B19 infection (especially in patients with underlying hemolytic anemias like Sickle Cell), SLE, and drugs (e.g., Phenytoin, Isoniazid) [1]. * **Diamond-Blackfan Anemia:** This is the **congenital** form of PRCA, often presenting in infancy with craniofacial anomalies and thumb deformities [1]. * **Diagnostic Hallmark:** Bone marrow biopsy showing <0.5% erythroblasts but normal granulopoiesis and megakaryopoiesis. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 595-596.

Q: Waldenstrom's macroglobulinemia is seen in which of the following conditions?

Plasmacytic Lymphoma. **Explanation:** **Waldenström’s Macroglobulinemia (WM)** is a distinct clinical syndrome defined by the presence of a monoclonal IgM protein (macroglobulinemia) in the setting of bone marrow infiltration by **Lymphoplasmacytic Lymphoma (LPL)** [1]. 1. **Why Option B is Correct:** According to the WHO classification, Waldenström’s Macroglobulinemia is the clinical manifestation of Lymphoplasmacytic Lymphoma (LPL). LPL is a B-cell neoplasm characterized by a mixture of small B-lymphocytes, plasmacytoid lymphocytes, and plasma cells. These cells secrete large amounts of **monoclonal IgM**, leading to the hallmark hyperviscosity syndrome [1]. 2. **Why Other Options are Incorrect:** * **Option A (Non-Hodgkin’s Lymphoma):** While LPL is technically a subtype of indolent NHL, the question asks for the specific underlying condition. "NHL" is too broad a category, whereas Plasmacytic (Lymphoplasmacytic) Lymphoma is the definitive pathological diagnosis. * **Option C (Chronic Lymphocytic Leukemia):** CLL is a malignancy of mature B-cells that typically expresses CD5 and CD23. While CLL can occasionally produce a monoclonal protein (M-spike), it is usually IgG or IgA, and it does not define Waldenström’s. **High-Yield Clinical Pearls for NEET-PG:** * **Hallmark Symptom:** Hyperviscosity syndrome (visual disturbances, mucosal bleeding, and neurological symptoms like "sausage-link" appearance of retinal veins) [1]. * **Genetic Marker:** Over 90% of patients possess the **MYD88 L265P mutation**. * **Diagnosis:** Bone marrow biopsy showing >10% infiltration by lymphoplasmacytic cells + any size IgM monoclonal spike [1]. * **Distinction from Multiple Myeloma:** Unlike Myeloma, WM rarely causes lytic bone lesions or hypercalcemia; it is primarily a disease of organomegaly (hepatosplenomegaly and lymphadenopathy).

Q: Bone marrow transplantation is used as a treatment modality in all of the following conditions except:

Hemochromatosis. **Explanation:** Bone marrow transplantation (BMT), or Hematopoietic Stem Cell Transplantation (HSCT), is used to treat conditions where the underlying pathology resides in the hematopoietic system or where donor cells can provide a missing enzyme or functional cell type [1]. **Why Hemochromatosis is the Correct Answer:** Hereditary Hemochromatosis is a disorder of **iron metabolism** caused primarily by mutations in the *HFE* gene, leading to excessive intestinal iron absorption. The defect lies in the liver (hepcidin regulation) and the gut, not the bone marrow. Therefore, BMT has no role in its management. The standard treatment remains **therapeutic phlebotomy** or iron chelation. **Analysis of Other Options:** * **Adrenoleukodystrophy (ALD):** This is a peroxisomal disorder. HSCT is the standard of care in the early stages of childhood cerebral ALD as it provides donor-derived microglial cells that can process very-long-chain fatty acids (VLCFA), halting neurodegeneration. * **Hurler’s Syndrome (MPS I):** A lysosomal storage disorder caused by alpha-L-iduronidase deficiency. BMT provides donor cells that secrete the functional enzyme, which is then taken up by the patient's cells (cross-correction), preventing progressive organ damage and cognitive decline. * **Osteopetrosis:** The "marble bone disease" is caused by **defective osteoclasts** (which are derived from the monocyte-macrophage lineage in the bone marrow). BMT provides functional donor-derived osteoclasts that can perform normal bone resorption. **NEET-PG High-Yield Pearls:** * **Osteopetrosis** is a classic "non-malignant" indication for BMT often tested in exams. * **Gaucher disease** and **Niemann-Pick** are other storage disorders where BMT may be considered. * In **Thalassemia and Sickle Cell Anemia**, BMT is currently the only curative treatment [2].

Question 1

A 58-year-old woman presents with a six-month history of backache and recurrent chest infections. She develops sudden leg weakness 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

Question 2

Which of the following types of multiple myeloma is most commonly associated with an increased risk of hyperviscosity syndrome?

Accepted Answer

IgM

Answer

IgG

Answer

IgD

Answer

IgA

Question 3

Autosplenectomy is a feature of which of the following conditions?

Accepted Answer

Sickle cell disease

Answer

Beta thalassemia

Answer

G6PD deficiency

Answer

All of the above

Question 4

Priapism can be due to which of the following conditions?

Accepted Answer

Chronic Myeloid Leukemia (CML)

Answer

Myelofibrosis

Answer

Autoimmune Hemolytic Anemia (AIHA)

Answer

Thrombocytopenia

Question 5

A 30-year-old epileptic female on phenytoin presents with weakness and fatigue. Blood examination reveals Hb = 4.6 gm/dL, MCV = 102 fL, and MCH = 40 pg/dL. What is the most probable diagnosis?

Accepted Answer

Megaloblastic anemia

Answer

Heart failure

Answer

Iron deficiency anemia

Answer

Phenytoin-induced agranulocytosis

Question 6

A 30-year-old female presents with mucosal bleeding upon minor trauma and a history of menorrhagia. Both bleeding time and prothrombin time are elevated. The ristocetin assay is positive. What is the most likely diagnosis?

Accepted Answer

Von Willebrand disease

Answer

Immune thrombocytopenic purpura (ITP)

Answer

Hemophilia A

Answer

Hemophilia B

Question 7

Pure red cell aplasia is associated with which of the following conditions?

Accepted Answer

Thymoma

Answer

Renal cell carcinoma

Answer

Hepatocellular carcinoma

Answer

Prostate carcinoma

Question 8

A 14-year-old presented with recurrent epistaxis along with characteristic skin and mucous membrane lesions. The lesions were seen at the dorsum of the tongue, oral cavity, buccal mucosa, lips, palate, and nasal mucosa. Lab findings revealed a normal coagulation profile. Which of the following drugs has been recently approved for this condition?

Accepted Answer

Bevacizumab

Answer

Ranibizumab

Answer

Transtuzumab

Answer

Sorafenib

Question 9

Waldenstrom's macroglobulinemia is seen in which of the following conditions?

Accepted Answer

Plasmacytic Lymphoma

Answer

Non-Hodgkin's Lymphoma (NHL)

Answer

Chronic Lymphocytic Leukemia (CLL)

Answer

None of the above

Question 10

Bone marrow transplantation is used as a treatment modality in all of the following conditions except:

Accepted Answer

Hemochromatosis

Answer

Adrenoleukodystrophy

Answer

Hurler's Syndrome

Answer

Osteopetrosis

Hematology — MCQs

Hematology — MCQs

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