Hematology Practice Questions

Q: What is the treatment of choice in hairy cell leukemia?

Cladribine. Explanation: **Hairy Cell Leukemia (HCL)** is a rare B-cell lymphoproliferative disorder characterized by pancytopenia, massive splenomegaly, and "hairy" cytoplasmic projections on peripheral smear. **Why Cladribine is the Correct Answer:** The treatment of choice for HCL is **Cladribine (2-Chlorodeoxyadenosine or 2-CdA)**. It is a purine nucleoside analog that is resistant to adenosine deaminase, leading to the accumulation of toxic nucleotides in B-cells. A single 7-day course of Cladribine induces complete remission in over 80-90% of patients, making it the gold standard first-line therapy. **Analysis of Incorrect Options:** * **Steroids (A):** Unlike in CLL or autoimmune hemolytic anemias, steroids have no significant role in treating the underlying malignancy in HCL and may increase the risk of opportunistic infections. * **Splenectomy (C):** Historically used to manage cytopenias and massive splenomegaly, it is now reserved for refractory cases or emergencies (e.g., splenic rupture). It is no longer the first-line treatment. * **Pentostatin (D):** Also a purine analog (adenosine deaminase inhibitor) and highly effective in HCL. However, **Cladribine** is preferred due to its shorter treatment duration and slightly better safety profile. **High-Yield Clinical Pearls for NEET-PG:** * **Marker of choice:** CD103 (most specific), CD11c, CD25, and Annexin A1. * **Genetic Mutation:** **BRAF V600E** mutation is present in nearly 100% of cases. * **Bone Marrow:** Often results in a **"Dry Tap"** due to increased reticulin fibrosis. * **Stain:** Tartrate-Resistant Acid Phosphatase (**TRAP**) positive. * **Clinical Hint:** Look for a patient with massive splenomegaly but **absent lymphadenopathy** (a classic HCL feature).

Q: A 55-year-old man complains of pain in his back, fatigue, and occasional confusion. He admits to polyuria and polydipsia. An X-ray examination reveals numerous lytic lesions in the lumbar vertebral bodies. Laboratory studies disclose hypoalbuminemia, mild anemia, and thrombocytopenia. A monoclonal Igk peak is demonstrated by serum electrophoresis. Urinalysis shows 4+ proteinuria. A bone marrow biopsy discloses foci of plasma cells, which account for 18% of all hematopoietic cells. What is the appropriate diagnosis?

Multiple myeloma. **Explanation:** The clinical presentation is a classic case of **Multiple Myeloma (MM)**, a neoplastic proliferation of plasma cells [1]. The diagnosis is confirmed based on the **CRAB criteria** and laboratory findings: 1. **C (Calcium elevation):** Confusion, polyuria, and polydipsia are signs of hypercalcemia. 2. **R (Renal insufficiency):** 4+ proteinuria (likely Bence-Jones proteins/light chains). 3. **A (Anemia):** Present in this patient, along with thrombocytopenia due to marrow infiltration. 4. **B (Bone lesions):** X-ray shows characteristic "punched-out" lytic lesions in the vertebrae [1]. The presence of **>10% clonal plasma cells** in the bone marrow (18% here) and a **monoclonal (M) protein peak** on electrophoresis are definitive diagnostic markers for MM [1]. **Incorrect Options:** * **A & B (ALL/CLL):** These are lymphoid malignancies. While they cause cytopenias, they do not typically present with lytic bone lesions, hypercalcemia, or a monoclonal IgK peak. * **C (Extramedullary plasmacytoma):** This refers to a plasma cell tumor occurring outside the bone marrow (e.g., in the upper respiratory tract). The presence of 18% marrow involvement and systemic CRAB features points to systemic Multiple Myeloma rather than a localized soft tissue tumor. **NEET-PG High-Yield Pearls:** * **Most common symptom:** Bone pain (back/ribs). * **M-Spike:** Usually IgG (most common), followed by IgA. * **Peripheral Smear:** Characterized by **Rouleaux formation** (due to high ESR/globulins). * **Urinalysis:** Standard dipsticks often miss Bence-Jones proteins; Sulfosalicylic acid (SSA) test or urine electrophoresis is required. * **Gold Standard Diagnosis:** Bone marrow aspiration and biopsy showing >10% plasma cells [1]. [2]

Q: Salmonellosis is most common in which of the following conditions?

Sickle cell anemia. **Explanation:** **Sickle Cell Anemia (SCA)** is the correct answer because patients with this condition are uniquely predisposed to invasive infections by *Salmonella* species, particularly **Salmonella osteomyelitis**. This susceptibility is due to several pathophysiological factors: 1. **Functional Hyposplenism:** Recurrent splenic infarcts lead to "autosplenectomy," impairing the clearance of encapsulated organisms and intracellular pathogens like *Salmonella*. 2. **Bowel Infarcts:** Microvascular occlusion in the gut wall allows *Salmonella* (normal flora or transient) to translocate into the bloodstream. 3. **Expanded Bone Marrow:** Chronic hemolysis leads to expanded, sluggish medullary blood flow and areas of bone necrosis (infarcts), providing an ideal nidus for bacterial seeding. **Analysis of Incorrect Options:** * **B. Thalassemia:** While these patients are prone to infections due to iron overload (siderophilic bacteria like *Yersinia*) and splenectomy, *Salmonella* is not specifically characteristic of Thalassemia compared to SCA. * **C. Hemophilia:** This is a coagulation disorder. Patients are at risk for transfusion-transmitted infections (HIV, Hepatitis), but not specifically *Salmonella*. * **D. Cystic Fibrosis:** These patients are primarily predisposed to respiratory infections, most notably *Pseudomonas aeruginosa* and *Staphylococcus aureus*. **High-Yield Clinical Pearls for NEET-PG:** * **Most common cause of Osteomyelitis in SCA:** *Salmonella* (unique to SCA), though *Staph. aureus* remains a frequent cause globally. * **Most common cause of Osteomyelitis in the general population:** *Staphylococcus aureus*. * **Most common cause of death in SCA children:** Sepsis due to *Streptococcus pneumoniae*. * **Prophylaxis:** Daily Oral Penicillin is recommended for children with SCA until age 5 to prevent pneumococcal sepsis.

Q: All are true about the clinical features of hereditary spherocytosis, EXCEPT?

Severe anemia during childhood. Hereditary Spherocytosis (HS) is the most common inherited erythrocyte membrane defect, typically caused by mutations in proteins like Ankyrin (most common), Spectrin, or Band 3 [2]. This leads to a loss of membrane surface area, resulting in spherical, rigid RBCs that are prematurely destroyed in the splenic sinusoids [1]. **Why Option B is the correct answer:** In HS, the anemia is typically mild to moderate, not severe [2]. The bone marrow is usually able to compensate for the extravascular hemolysis by increasing erythropoiesis. Severe anemia in HS is rare and usually only occurs during "crises" (e.g., Aplastic crisis triggered by Parvovirus B19 or Megaloblastic crisis due to folate deficiency). **Analysis of Incorrect Options:** * **A. Gallstones:** Chronic extravascular hemolysis [3] leads to increased production of indirect bilirubin. This results in the formation of pigment (calcium bilirubinate) gallstones, which can be seen even in young children. * **C. Splenomegaly:** Since the spleen is the primary site of destruction for the rigid spherocytes [1], it undergoes work hypertrophy, making splenomegaly a hallmark clinical feature. * **D. Jaundice:** Intermittent unconjugated hyperbilirubinemia (acholuric jaundice) is common due to the continuous breakdown of RBCs. **NEET-PG High-Yield Pearls:** * **Inheritance:** Most cases are Autosomal Dominant. * **Diagnosis:** The gold standard is the **EMA Binding Test** (Flow cytometry). The Osmotic Fragility Test is also used but is less specific. * **Peripheral Smear:** Shows spherocytes (small, dark cells lacking central pallor) and reticulocytosis. * **Lab Marker:** Characteristically high **MCHC** (>36 g/dL) [1]. * **Treatment:** Splenectomy is the definitive treatment for symptomatic cases (usually deferred until age 6 to reduce sepsis risk) [2].

Q: Serum alkaline phosphatase is normal in which of the following conditions?

Multiple myeloma. **Explanation:** The correct answer is **Multiple Myeloma**. **1. Why Multiple Myeloma is the correct answer:** In Multiple Myeloma (MM), bone destruction is primarily mediated by **osteoclast-activating factors** (like RANKL and IL-6), which lead to purely **lytic lesions** [1]. Unlike other bone pathologies, there is a characteristic **lack of osteoblastic activity** (bone formation). Since Serum Alkaline Phosphatase (ALP) is a marker of osteoblastic activity, it remains **normal** in MM despite extensive bone destruction [2]. This is a classic "negative" finding used to differentiate MM from other bone-metastasizing cancers. **2. Why the other options are incorrect:** * **Osteosarcoma:** This is a bone-forming tumor. Increased osteoblastic activity leads to significantly **elevated** ALP levels, which also serve as a prognostic marker. * **Osteomalacia:** Characterized by defective mineralization of the osteoid [3]. The body attempts to compensate for weak bones by increasing osteoblastic activity, resulting in **elevated** ALP [4]. * **Malnutrition:** Severe malnutrition, specifically Vitamin D or Calcium deficiency, leads to secondary hyperparathyroidism and increased bone turnover, typically causing an **elevation** in ALP [4]. **Clinical Pearls for NEET-PG:** * **The "Punch-out" Rule:** MM presents with "punched-out" lytic lesions on X-ray [2]. Because there is no reactive bone formation, a **Bone Scan** (which relies on osteoblastic activity) is often **falsely negative** in MM; Skeletal Surveys or MRI are preferred. * **Hypercalcemia in MM:** High serum calcium with normal ALP is a strong diagnostic clue for Multiple Myeloma. * **ALP in Healing:** ALP is also elevated during the healing phase of fractures and in Paget’s disease (where it is markedly high).

Q: A patient presented with skin pigmentation, hyperglycemia, and a serum ferritin level of 900 mcg/L. What is the possible diagnosis?

Atransferrinemia. The clinical triad of **skin pigmentation** (bronzing), **hyperglycemia** (diabetes mellitus), and **elevated serum ferritin** (iron overload) is characteristic of **Secondary Hemochromatosis**. [1] ### **Explanation of the Correct Answer** **Atransferrinemia** is a rare autosomal recessive disorder characterized by a deficiency of transferrin. Without transferrin to transport iron to the bone marrow for erythropoiesis, iron remains "free" in the plasma. This leads to: 1. **Iron Overload:** Excess iron deposits in parenchymal organs (liver, pancreas, skin), causing the "Bronze Diabetes" presentation described in the question. [1], [2] 2. **Microcytic Anemia:** Despite high systemic iron, the bone marrow lacks iron for hemoglobin synthesis. ### **Why Other Options are Incorrect** * **Hereditary Spherocytosis:** This is a red cell membrane defect (e.g., ankyrin deficiency) leading to extravascular hemolysis. While chronic hemolysis can cause mild iron elevation, it does not typically present with the full triad of bronze diabetes unless the patient is heavily transfused. * **Thalassemia:** While Thalassemia Major causes significant iron overload (due to ineffective erythropoiesis and repeated transfusions), the primary presentation in NEET-PG vignettes usually emphasizes severe anemia, hepatosplenomegaly, and "chipmunk facies." Atransferrinemia is a more specific cause of "transferrin-deficient" iron overload. ### **NEET-PG High-Yield Pearls** * **Bronze Diabetes:** Refers to the combination of skin hyperpigmentation and diabetes mellitus due to iron deposition in the pancreas. [1] * **Classic Triad of Hemochromatosis:** Cirrhosis, Diabetes, and Skin Pigmentation. * **Diagnosis:** The most sensitive initial test for iron overload is **Transferrin Saturation** (>45-50%), while **Serum Ferritin** reflects total body iron stores. [3] * **Treatment of Atransferrinemia:** Plasma infusions (to provide transferrin) and iron chelation.

Question 1

What is the treatment of choice in hairy cell leukemia?

Accepted Answer

Cladribine

Answer

Steroid

Answer

Splenectomy

Answer

Pentostatin

Question 2

Which is the most common preceding infection in bone marrow failure syndromes?

Accepted Answer

Hepatitis virus

Answer

Parvovirus B19

Answer

Epstein-Barr virus

Answer

Human Immunodeficiency Virus

Question 3

Warm autoimmune hemolytic anemia may be seen in all of the following conditions except?

Accepted Answer

Mycoplasma pneumoniae

Answer

Systemic lupus erythematosus

Answer

Alpha-methyl dopa therapy

Answer

Non-Hodgkin's lymphoma

Question 4

A 55-year-old man complains of pain in his back, fatigue, and occasional confusion. He admits to polyuria and polydipsia. An X-ray examination reveals numerous lytic lesions in the lumbar vertebral bodies. Laboratory studies disclose hypoalbuminemia, mild anemia, and thrombocytopenia. A monoclonal Igk peak is demonstrated by serum electrophoresis. Urinalysis shows 4+ proteinuria. A bone marrow biopsy discloses foci of plasma cells, which account for 18% of all hematopoietic cells. What is the appropriate diagnosis?

Accepted Answer

Multiple myeloma

Answer

Acute lymphoblastic leukemia

Answer

Chronic lymphocytic leukemia

Answer

Extramedullary plasmacytoma

Question 5

Which of the following statements about Sickle Cell Disease is true?

Accepted Answer

Symptoms are ameliorated by HbF

Answer

Mutation in the alpha chain

Answer

Veno-occlusive crises are the cause of morbidity

Answer

Bone pain is a presenting feature

Question 6

Which of the following is NOT true about Heparin-Induced Thrombocytopenia (HIT) syndrome?

Accepted Answer

It is more common with unfractionated heparin than fractionated heparin

Answer

Low molecular weight heparin (LMWH) should not be used for treatment

Answer

It causes both arterial and venous thrombosis

Answer

It commonly occurs about a week after starting heparin therapy

Question 7

Salmonellosis is most common in which of the following conditions?

Accepted Answer

Sickle cell anemia

Answer

Thalassemia

Answer

Hemophilia

Answer

Cystic fibrosis

Question 8

All are true about the clinical features of hereditary spherocytosis, EXCEPT?

Accepted Answer

Severe anemia during childhood

Answer

Gallstones

Answer

Splenomegaly

Answer

Jaundice

Question 9

Serum alkaline phosphatase is normal in which of the following conditions?

Accepted Answer

Multiple myeloma

Answer

Osteosarcoma

Answer

Osteomalacia

Answer

Malnutrition

Question 10

A patient presented with skin pigmentation, hyperglycemia, and a serum ferritin level of 900 mcg/L. What is the possible diagnosis?

Accepted Answer

Atransferrinemia

Answer

Hereditary spherocytosis

Answer

Thalassemia

Hematology — MCQs

Hematology — MCQs

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