Hematology Practice Questions

Q: Life-threatening intravascular hemolysis occurs with sepsis due to which organism?

Clostridium perfringens. **Explanation:** The correct answer is **Clostridium perfringens**. **1. Why Clostridium perfringens is correct:** Sepsis caused by *Clostridium perfringens* (often associated with septic abortion, cholecystitis, or gas gangrene) is a notorious cause of **massive, life-threatening intravascular hemolysis** [1]. The primary virulence factor responsible is the **Alpha-toxin (Lecithinase)**. This toxin acts as a phospholipase that hydrolyzes lecithin and sphingomyelin in the red blood cell (RBC) membranes, leading to membrane instability, rapid lysis, and the formation of **spherocytes**. This can result in "mahogany-colored" urine (hemoglobinuria), acute renal failure, and DIC. **2. Why the other options are incorrect:** * **Mycoplasma pneumoniae:** While it causes hemolysis, it is typically **extravascular** and mediated by **Cold Agglutinins (IgM)**. It is rarely life-threatening or acutely intravascular. * **Pseudomonas & Klebsiella:** These Gram-negative organisms are common causes of septic shock and DIC, but they do not produce specific toxins that cause direct, massive intravascular destruction of RBCs as a primary clinical feature. **3. High-Yield Clinical Pearls for NEET-PG:** * **The "Double Zone of Hemolysis":** On blood agar, *C. perfringens* produces a characteristic double zone (inner clear zone due to theta-toxin, outer fuzzy zone due to alpha-toxin). * **Nagler’s Reaction:** Used to identify the lecithinase activity of the Alpha-toxin. * **Peripheral Smear:** Look for **marked microspherocytosis** and a lack of organisms (as the hemolysis is toxin-mediated, not necessarily due to direct bacterial invasion of RBCs). * **Treatment:** High-dose Penicillin G and surgical debridement are the mainstays of management.

Q: Which of the following is characteristic of Felty syndrome?

Splenomegaly and neutropenia. **Explanation:** **Felty Syndrome** is a rare but serious extra-articular manifestation of long-standing, seropositive Rheumatoid Arthritis (RA) [1]. It is classically defined by a clinical triad: 1. **Rheumatoid Arthritis** (usually severe, erosive, and chronic). 2. **Splenomegaly** [1]. 3. **Neutropenia** (Absolute Neutrophil Count <2000/mm³). The correct answer is **A** because the hallmark of the syndrome is the immune-mediated destruction of neutrophils and their sequestration in the enlarged spleen, leading to an increased risk of recurrent bacterial infections. **Analysis of Incorrect Options:** * **Option B:** Describes **Caplan Syndrome**, which is the combination of Rheumatoid Arthritis and coal worker's pneumoconiosis, characterized by intrapulmonary nodules. * **Option C:** Refers to **Rheumatoid Vasculitis**, a complication involving small and medium-sized vessels, leading to skin ulcers and digital ischemia [1]. * **Option D:** While multiple finger deformities (e.g., swan-neck, boutonniere) are characteristic of RA itself, they are not the defining features of Felty Syndrome. **High-Yield Clinical Pearls for NEET-PG:** * **Genetics:** Strongly associated with the **HLA-DR4** serotype. * **Risk Factors:** More common in Caucasians, females, and patients with high titers of Rheumatoid Factor (RF) and anti-CCP [1]. * **Complication:** Patients have an increased risk of **Non-Hodgkin Lymphoma** and skin cancers. * **Treatment:** The primary goal is treating the underlying RA, typically with **Methotrexate**. Granulocyte colony-stimulating factor (G-CSF) may be used for severe neutropenia.

Q: A 46-year-old lady presents with pallor. You suspect iron deficiency anemia. Which of the following blood investigation results is NOT consistent with this provisional diagnosis?

Decreased level of red cell protoporphyrin. In Iron Deficiency Anemia (IDA), the body lacks sufficient iron to complete the final step of heme synthesis. Under normal conditions, the enzyme **ferrochelatase** inserts ferrous iron into a precursor molecule called **protoporphyrin IX** to form heme. **Why Option D is the Correct Answer:** When iron is deficient, protoporphyrin has no iron to bind with. Consequently, it accumulates within the red blood cells. Therefore, in IDA, we see an **increased** level of **Free Erythrocyte Protoporphyrin (FEP)**, not a decreased level. This makes Option D inconsistent with the diagnosis. **Analysis of Incorrect Options:** * **A. Low plasma ferritin:** Ferritin is the storage form of iron [1]. A low serum ferritin is the **most specific** initial laboratory finding in IDA, reflecting depleted body stores [2]. * **B. Decreased saturation of transferrin:** As serum iron drops and Total Iron Binding Capacity (TIBC) increases, the percentage of transferrin saturated with iron falls (typically <16% in IDA) [2]. * **C. Decreased hemoglobin:** This is the hallmark of anemia [1]. In IDA, hemoglobin synthesis is impaired, leading to microcytic hypochromic anemia [2]. **NEET-PG High-Yield Pearls:** * **Earliest sign of IDA:** Decreased serum ferritin (depletion of stores) [2]. * **Earliest functional sign:** Increase in Red Cell Distribution Width (RDW) [2]. * **Gold Standard Investigation:** Bone marrow aspiration (Prussian blue staining) showing absent haemosiderin (though rarely done clinically) [2]. * **Mentzer Index:** (MCV/RBC count) 13 suggests IDA.

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: In lymphoplasmacytoid lymphoma, which of the following monoclonal immunoglobulins is most commonly seen?

IgM. **Explanation:** **Lymphoplasmacytoid Lymphoma (LPL)** is a mature B-cell neoplasm characterized by a proliferation of small B-lymphocytes, plasmacytoid lymphocytes, and plasma cells. 1. **Why IgM is Correct:** The defining clinical feature of LPL is the secretion of a monoclonal protein. In the vast majority of cases (approximately 90-95%), this protein is **IgM** [1]. When LPL involves the bone marrow and is associated with a monoclonal IgM gammopathy of any size, it is clinically termed **Waldenström Macroglobulinemia (WM)** [1]. The large size of the IgM pentamer leads to the classic clinical presentation of hyperviscosity syndrome [1]. 2. **Why Other Options are Incorrect:** * **IgG and IgA:** While rare variants of LPL can secrete IgG or IgA, these are much more commonly associated with **Multiple Myeloma** or Monoclonal Gammopathy of Undetermined Significance (MGUS) [1]. * **IgD:** This is extremely rare and is typically associated with a specific, aggressive subtype of Multiple Myeloma, not LPL. **High-Yield Clinical Pearls for NEET-PG:** * **Genetic Marker:** Over 90% of LPL/WM cases harbor the **MYD88 L265P mutation**, which is a key diagnostic marker. * **Clinical Triad:** Look for anemia, hepatosplenomegaly/lymphadenopathy, and hyperviscosity (visual disturbances, mucosal bleeding, neurological symptoms) [1]. * **Morphology:** Bone marrow biopsy shows a "lymphoplasmacytoid" infiltrate and increased mast cells [1]. * **Differentiator:** Unlike Multiple Myeloma, LPL/WM typically does **not** cause lytic bone lesions or hypercalcemia (No "CRAB" features).

Q: What is the best treatment for a patient with paroxysmal nocturnal hemoglobinuria (PNH) presenting with black-colored urine?

Prednisolone. **Explanation:** The clinical presentation of "black-colored urine" in a patient with Paroxysmal Nocturnal Hemoglobinuria (PNH) signifies an **acute hemolytic crisis**. **1. Why Prednisolone is Correct:** While Eculizumab is the definitive long-term management for PNH, **Corticosteroids (Prednisolone)** are the traditional first-line treatment for managing **acute episodes of hemolysis**. They work by stabilizing the red cell membrane and reducing complement-mediated destruction. In the context of an acute presentation (black urine), steroids help reduce the severity and duration of the hemolytic paroxysm. **2. Why the other options are incorrect:** * **Eculizumab (Option A):** This is a monoclonal antibody against protein C5. It is the **treatment of choice for long-term control** and prevention of thrombosis in PNH. However, in many exam scenarios (and resource-limited settings), steroids are prioritized for the immediate management of a sudden hemolytic flare. * **Rituximab (Option B):** This is an anti-CD20 monoclonal antibody used in B-cell lymphomas and Autoimmune Hemolytic Anemia (AIHA). It has no role in PNH, as PNH is a stem cell defect, not an antibody-mediated process [1]. * **Infliximab (Option C):** This is an anti-TNF-alpha agent used in Crohn’s disease and Rheumatoid Arthritis; it has no role in PNH management. **Clinical Pearls for NEET-PG:** * **Gold Standard Diagnosis:** Flow cytometry (shows absence of CD55 and CD59). * **Definitive Cure:** Allogeneic Bone Marrow Transplantation. * **Most Common Cause of Death:** Thrombosis (often in unusual sites like the hepatic vein—Budd-Chiari Syndrome). * **Triad of PNH:** Hemolytic anemia, Pancytopenia, and Venous thrombosis.

Question 1

Life-threatening intravascular hemolysis occurs with sepsis due to which organism?

Accepted Answer

Clostridium perfringens

Answer

Mycoplasma pneumoniae

Answer

Pseudomonas

Answer

Klebsiella

Question 2

In which of the following conditions is splenectomy not useful?

Accepted Answer

Porphyria

Answer

Hereditary spherocytosis

Answer

Thalassemia

Answer

Sickle cell disease with large spleen

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

Pawn ball megakaryocytes are characteristic of which condition?

Accepted Answer

Myelodysplastic syndrome

Answer

Idiopathic thrombocytopenic purpura

Answer

Thrombotic thrombocytopenic purpura

Answer

Chloramphenicol toxicity

Question 5

Which of the following is characteristic of Felty syndrome?

Accepted Answer

Splenomegaly and neutropenia

Answer

Nodules in the upper lobe of the lung and difficulty in breathing

Answer

Digital ulceration and gangrene

Answer

Multiple finger deformities

Question 6

A 46-year-old lady presents with pallor. You suspect iron deficiency anemia. Which of the following blood investigation results is NOT consistent with this provisional diagnosis?

Accepted Answer

Decreased level of red cell protoporphyrin

Answer

Low levels of plasma ferritin

Answer

Decreased saturation of transferrin

Answer

Decreased level of hemoglobin

Question 7

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 8

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 9

In lymphoplasmacytoid lymphoma, which of the following monoclonal immunoglobulins is most commonly seen?

Accepted Answer

IgM

Answer

IgA

Answer

IgD

Answer

IgG

Question 10

What is the best treatment for a patient with paroxysmal nocturnal hemoglobinuria (PNH) presenting with black-colored urine?

Accepted Answer

Prednisolone

Answer

Eculizumab

Answer

Rituximab

Answer

Infliximab

Hematology — MCQs

Hematology — MCQs

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