Hematology — MCQs

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?

Q248Medium

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

Q249Easy

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

Q250Medium

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

Hematology Indian Medical PG Practice Questions and MCQs

Question 241: Life-threatening intravascular hemolysis occurs with sepsis due to which organism?

A. Clostridium perfringens (Correct Answer)
B. Mycoplasma pneumoniae
C. Pseudomonas
D. Klebsiella

Explanation: **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.

Question 242: In which of the following conditions is splenectomy not useful?

A. Hereditary spherocytosis
B. Porphyria (Correct Answer)
C. Thalassemia
D. Sickle cell disease with large spleen

Explanation: **Explanation:** The correct answer is **Porphyria**. Splenectomy is a surgical intervention primarily used in hematology to manage conditions involving excessive peripheral destruction of blood cells (extravascular hemolysis) or symptomatic massive splenomegaly. **Why Porphyria is the correct answer:** Porphyrias are a group of metabolic disorders caused by enzyme deficiencies in the heme biosynthetic pathway. The pathology is chemical/metabolic (accumulation of porphyrins), not mechanical or related to splenic sequestration. Therefore, removing the spleen has no physiological basis for treating the underlying enzyme defect or the clinical manifestations (skin photosensitivity or neurological crises). **Analysis of incorrect options:** * **Hereditary Spherocytosis:** This is the **most common** indication for elective splenectomy. Since the spleen is the primary site where spherical, rigid RBCs are destroyed, splenectomy significantly increases RBC lifespan and cures the anemia (though not the membrane defect) [1], [2]. * **Thalassemia:** In Thalassemia major, splenectomy is indicated if there is "hypersplenism" (increasing transfusion requirements) or symptomatic massive splenomegaly. It helps reduce the frequency of blood transfusions. * **Sickle Cell Disease (SCD):** While many SCD patients undergo "autosplenectomy" due to repeated infarcts, some (especially those with HbSC or S-beta thal) develop massive, painful splenomegaly or **splenic sequestration crises**. In these specific cases, splenectomy is life-saving. **NEET-PG High-Yield Pearls:** * **Vaccination Rule:** Post-splenectomy patients are at risk of OPSI (Overwhelming Post-Splenectomy Infection) by encapsulated organisms (*S. pneumoniae, H. influenzae, N. meningitidis*). Vaccinate **2 weeks before** elective surgery or **2 weeks after** emergency surgery. * **Peripheral Smear:** Look for **Howell-Jolly bodies**, Pappenheimer bodies, and Heinz bodies post-splenectomy. * **Platelet count:** Splenectomy often leads to transient thrombocytosis.

Question 243: Warm autoimmune hemolytic anemia may be seen in all of the following conditions except?

A. Systemic lupus erythematosus
B. Alpha-methyl dopa therapy
C. Non-Hodgkin's lymphoma
D. Mycoplasma pneumoniae (Correct Answer)

Explanation: ### Explanation **Correct Answer: D. Mycoplasma pneumoniae** The distinction between Warm and Cold Autoimmune Hemolytic Anemia (AIHA) is a high-yield topic in hematology, based on the thermal amplitude of the antibodies involved [1]. **Why Mycoplasma pneumoniae is the correct answer:** *Mycoplasma pneumoniae* is classically associated with **Cold Agglutinin Disease (Cold AIHA)** [2]. The infection triggers the production of **IgM antibodies** (specifically anti-I antibodies) that bind to red blood cells at low temperatures (optimally at 4°C) [2]. This leads to complement-mediated hemolysis, typically occurring in the peripheral circulation where temperatures are lower [2]. **Why the other options are incorrect:** * **A. Systemic Lupus Erythematosus (SLE):** SLE is the most common autoimmune cause of **Warm AIHA**. It involves **IgG antibodies** that react at body temperature (37°C), leading to extravascular hemolysis in the spleen [1]. * **B. Alpha-methyl dopa therapy:** This drug is a classic cause of drug-induced Warm AIHA. It alters the Rh antigens on the RBC surface, inducing the formation of autoantibodies. * **C. Non-Hodgkin's Lymphoma (NHL):** Lymphoproliferative disorders (like NHL and CLL) are major secondary causes of Warm AIHA due to the production of pathogenic IgG by malignant B-cells. **Clinical Pearls for NEET-PG:** 1. **Warm AIHA:** IgG-mediated, extravascular hemolysis (spleen), associated with SLE, CLL, and drugs (α-methyldopa, Penicillin) [1]. 2. **Cold AIHA:** IgM-mediated, intravascular/extravascular hemolysis (liver), associated with *Mycoplasma pneumoniae* and Infectious Mononucleosis (EBV) [2]. 3. **Direct Coombs Test:** Positive in both, but Warm AIHA usually shows IgG/C3d, while Cold AIHA shows only C3d (as IgM dissociates at warmer temperatures) [1]. 4. **Treatment:** Steroids are the first-line for Warm AIHA but are generally **ineffective** for Cold AIHA (where cold avoidance and Rituximab are preferred) [2].

Question 244: Pawn ball megakaryocytes are characteristic of which condition?

A. Myelodysplastic syndrome (Correct Answer)
B. Idiopathic thrombocytopenic purpura
C. Thrombotic thrombocytopenic purpura
D. Chloramphenicol toxicity

Explanation: **Explanation:** **Pawn ball megakaryocytes** are a hallmark morphological feature of **Myelodysplastic Syndrome (MDS)**. These are small, mononuclear or binuclear megakaryocytes with separated, round nuclei that resemble the shape of a pawn from a chessboard. This phenomenon is a manifestation of **dysmegakaryopoiesis** (disordered platelet production), which is one of the three lineages affected in MDS, alongside dyserythropoiesis and dysmyelopoiesis. **Analysis of Options:** * **Myelodysplastic Syndrome (Correct):** MDS is characterized by ineffective hematopoiesis and peripheral cytopenias. The presence of "pawn ball" nuclei or "micromegakaryocytes" is highly specific for diagnosing MDS, particularly the 5q- syndrome subtype. * **Idiopathic Thrombocytopenic Purpura (ITP):** In ITP, the bone marrow typically shows an *increase* in the number of megakaryocytes (compensatory hyperplasia) to counter peripheral destruction, but they are morphologically normal or slightly larger, not "pawn ball" shaped. * **Thrombotic Thrombocytopenic Purpura (TTP):** TTP is a microangiopathic hemolytic anemia. The bone marrow is generally reactive but does not show the specific dysplastic nuclear changes seen in MDS. * **Chloramphenicol Toxicity:** This typically causes dose-dependent bone marrow suppression or idiosyncratic aplastic anemia, characterized by a hypocellular marrow rather than specific dysplastic megakaryocytes. **High-Yield Clinical Pearls for NEET-PG:** * **Ring Sideroblasts:** Seen in MDS (specifically RARS) due to iron accumulation in mitochondria; visualized with **Prussian Blue stain**. * **Pseudo-Pelger-Huët Anomaly:** Hyposegmented, bilobed neutrophils (pince-nez appearance) seen in MDS. * **5q- Syndrome:** A specific subtype of MDS occurring typically in elderly females, characterized by macrocytic anemia, normal/elevated platelets, and "pawn ball" megakaryocytes. It has a favorable prognosis and responds well to **Lenalidomide**.

Question 245: Which of the following is characteristic of Felty syndrome?

A. Splenomegaly and neutropenia (Correct Answer)
B. Nodules in the upper lobe of the lung and difficulty in breathing
C. Digital ulceration and gangrene
D. Multiple finger deformities

Explanation: **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.

Question 246: 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?

A. Low levels of plasma ferritin
B. Decreased saturation of transferrin
C. Decreased level of hemoglobin
D. Decreased level of red cell protoporphyrin (Correct Answer)

Explanation: 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 Thalassemia trait; >13 suggests IDA.

Question 247: 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?

A. Acute lymphoblastic leukemia
B. Chronic lymphocytic leukemia
C. Extramedullary plasmacytoma
D. Multiple myeloma (Correct Answer)

Explanation: **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]

Question 248: Which of the following statements about Sickle Cell Disease is true?

A. Mutation in the alpha chain
B. Symptoms are ameliorated by HbF (Correct Answer)
C. Veno-occlusive crises are the cause of morbidity
D. Bone pain is a presenting feature

Explanation: ### Explanation **Correct Option: B. Symptoms are ameliorated by HbF** The pathophysiology of Sickle Cell Disease (SCD) involves the polymerization of deoxygenated Hemoglobin S (HbS), leading to red cell sickling and vaso-occlusion. **Fetal Hemoglobin (HbF)** acts as a potent inhibitor of HbS polymerization because it does not co-polymerize with HbS [2]. Higher levels of HbF dilute the concentration of HbS and increase the overall oxygen affinity, thereby reducing sickling episodes and clinical severity. This is the pharmacological basis for using **Hydroxyurea**, which induces HbF production to manage SCD [2]. **Analysis of Incorrect Options:** * **A. Mutation in the alpha chain:** SCD is caused by a point mutation in the **$\beta$-globin gene** on chromosome 11, where glutamic acid is replaced by valine at the 6th position ($\beta^6 Glu \to Val$) [4]. * **C. Veno-occlusive crises are the cause of morbidity:** While common, the primary cause of morbidity and mortality in SCD is **Vaso-occlusive crises (VOC)** involving small *arterioles and capillaries*, leading to organ damage (e.g., Acute Chest Syndrome, Stroke). "Veno-occlusive" refers to a different pathology (e.g., Budd-Chiari or hepatic sinusoidal obstruction) [3]. * **D. Bone pain is a presenting feature:** While bone pain (dactylitis) is common, it is typically a **complication** or a feature of a crisis rather than the "presenting feature" at birth. Infants are asymptomatic for the first 6 months due to the protective presence of HbF. **NEET-PG High-Yield Pearls:** * **Dactylitis (Hand-foot syndrome):** Often the first clinical manifestation of SCD in infants as HbF levels drop. * **Autosplenectomy:** Repeated splenic infarctions lead to a shrunken, fibrotic spleen by adulthood, increasing risk from encapsulated organisms (*S. pneumoniae*, *H. influenzae*) [3]. * **Salmonella Osteomyelitis:** SCD patients have a unique predisposition to *Salmonella* infections of the bone. * **Diagnosis:** **Hb Electrophoresis** is the gold standard (shows HbS, no HbA1) [1]. Screening is done via the **Solubility test** or Sickling test.

Question 249: In lymphoplasmacytoid lymphoma, which of the following monoclonal immunoglobulins is most commonly seen?

A. IgA
B. IgD
C. IgG
D. IgM (Correct Answer)

Explanation: **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).

Question 250: What is the best treatment for a patient with paroxysmal nocturnal hemoglobinuria (PNH) presenting with black-colored urine?

A. Eculizumab
B. Rituximab
C. Infliximab
D. Prednisolone (Correct Answer)

Explanation: **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.

Practice by Chapter

Anemia Evaluation and Management

Practice Questions

Hemoglobinopathies

Practice Questions

Thalassemias

Practice Questions

Platelet Disorders

Practice Questions

Coagulation Disorders

Practice Questions

Thrombotic Disorders

Practice Questions

Leukemias

Practice Questions

Lymphomas

Practice Questions

Multiple Myeloma and Plasma Cell Disorders

Practice Questions

Myeloproliferative Neoplasms

Practice Questions

Transfusion Medicine

Practice Questions

Hematopoietic Stem Cell Transplantation

Practice Questions

Want unlimited practice?

Get full access to all questions, explanations, and performance tracking.

Start For Free

Hematology — MCQs

Hematology — MCQs

On this page

Practice by Chapter

Want unlimited practice?