Hematology — MCQs

A 40-year-old man with a 10-year history of diabetes, currently on medication, presents with abdominal pain, weakness, lightheadedness, palpitations, and shortness of breath. On examination, he has glossitis, hyperpigmentation of the skin on the dorsum of his hands and feet, and abnormal hair pigmentation. His family reports increased irritability over the past year. Laboratory findings include Hb: 9 gm%, MCV: 110 fl, MCH: 36 Pg, MCHC: 34 gm/dl, Reticulocyte count: 0.1 x 10^9/L, LDH: 600 U/L, Indirect bilirubin: 1 mg/dL, S. iron: Normal, S. Ferritin: Normal, WBC: 2 x 10^9/L, and Platelet count: 90 x 10^9/L. Peripheral blood smear and bone marrow aspiration findings are also noted. Which of the following drugs is the patient most likely taking?

Q539Medium

Splenomegaly and reticulocytosis are typically absent in which type of anaemia?

Q540Hard

A patient presents with microcytic hypochromic anemia, Hb of 9 g/dL, serum iron of 20 µg/dL, ferritin level of 800 ng/mL, and transferrin saturation of 64%. What is the most likely diagnosis?

Hematology Indian Medical PG Practice Questions and MCQs

Question 531: What is a common cause of acute chest syndrome?

A. Sickle cell disease (Correct Answer)
B. Pneumonia
C. Acute myocardial infarction
D. Penetrating chest trauma

Explanation: **Explanation:** **Acute Chest Syndrome (ACS)** is a life-threatening pulmonary complication defined by a new radiologic infiltrate (involving at least one complete lung segment) combined with chest pain, fever, tachypnea, or wheezing. **Why Sickle Cell Disease (SCD) is correct:** ACS is the leading cause of death in patients with **Sickle Cell Disease**. [1] The underlying pathophysiology involves a "vicious cycle" of microvascular occlusion. It is primarily triggered by **pulmonary fat embolism** (from bone marrow necrosis during a vaso-occlusive crisis) [1] or **intrapulmonary sickling**, which leads to lung ischemia, inflammation, and further sickling due to hypoxia. **Analysis of Incorrect Options:** * **B. Pneumonia:** While infection (e.g., *Chlamydia pneumoniae*, *Mycoplasma*) can *trigger* ACS in a sickle cell patient, pneumonia itself is a localized infection. ACS is a specific systemic-pulmonary syndrome unique to the SCD population. * **C. Acute Myocardial Infarction:** This causes ischemic chest pain but does not typically present with the characteristic pulmonary infiltrates or the specific hematologic mechanism seen in ACS. * **D. Penetrating Chest Trauma:** This leads to conditions like pneumothorax or hemothorax, which are distinct clinical entities from the vaso-occlusive nature of ACS. **High-Yield Clinical Pearls for NEET-PG:** * **Management:** Treatment includes aggressive pain control, supplemental oxygen, broad-spectrum antibiotics (covering atypicals), and **exchange transfusion** (indicated if $PaO_2 < 60$ mmHg or rapidly progressing symptoms). * **Prevention:** **Hydroxyurea** is the drug of choice to reduce the frequency of ACS episodes by increasing Fetal Hemoglobin (HbF). * **Radiology:** The chest X-ray often lags behind clinical symptoms; a clear X-ray initially does not rule out ACS.

Question 532: What is the first test to become positive in patients with Hemochromatosis?

A. Increased Serum Iron
B. Increased Serum Ferritin
C. Increased Transferrin Saturation (Correct Answer)
D. Increased Liver Enzymes

Explanation: **Explanation:** In Hereditary Hemochromatosis (HH), the primary defect is a mutation in the **HFE gene**, leading to decreased **Hepcidin** levels. Low hepcidin causes unregulated iron absorption in the duodenum and excessive release of iron from macrophages. **1. Why Transferrin Saturation is the correct answer:** Transferrin saturation (TSAT) is calculated as (Serum Iron / Total Iron Binding Capacity) × 100. Because iron is being absorbed at an abnormally high rate and entering the plasma, the available transferrin molecules become saturated very early in the disease process, often before significant tissue damage or massive storage occurs. It is the **most sensitive screening test** and the first biochemical abnormality to appear. A TSAT >45% is highly suggestive of HH. **2. Why the other options are incorrect:** * **Serum Iron:** While serum iron increases, it is subject to significant daily fluctuations and is not as reliable or early an indicator as the saturation percentage. * **Serum Ferritin:** Ferritin reflects total body iron stores. While it is used to assess the severity of iron overload, it rises **after** transferrin saturation has already increased. It is also an acute-phase reactant, making it less specific. * **Liver Enzymes:** Elevation of ALT/AST occurs only after significant iron deposition leads to structural liver damage (cirrhosis/fibrosis). This is a late manifestation of the disease. **NEET-PG High-Yield Pearls:** * **Gold Standard Diagnosis:** Genetic testing for **C282Y mutation** of the HFE gene. * **Most Accurate Test for Iron Overload:** MRI (T2*) or Liver Biopsy (Perls' Prussian Blue stain). * **Classic Triad (Bronze Diabetes):** Cirrhosis, Diabetes Mellitus, and Skin Hyperpigmentation. * **Treatment of Choice:** Therapeutic Phlebotomy (Target Ferritin: 50–100 ng/mL).

Question 533: What is the most common plasma cell tumor?

A. Plasmacytoma
B. Waldenstrom's macroglobulinemia
C. Multiple myeloma (Correct Answer)
D. Primary amyloidosis

Explanation: **Explanation:** **Multiple Myeloma (MM)** is the most common primary malignancy of the bone marrow and the most common plasma cell dyscrasia [1]. It is characterized by the neoplastic proliferation of a single clone of plasma cells (M-protein) that produce monoclonal immunoglobulins. It typically presents in older adults with the classic **CRAB** features: Hyper**C**alcemia, **R**enal insufficiency, **A**nemia, and **B**one lesions (lytic) [2]. **Analysis of Incorrect Options:** * **Plasmacytoma:** This refers to a localized collection of neoplastic plasma cells. It can be solitary (bone or extramedullary) but is significantly less common than the systemic involvement seen in Multiple Myeloma. * **Waldenstrom’s Macroglobulinemia:** This is a lymphoproliferative disorder characterized by **IgM** monoclonal gammopathy [1]. It involves lymphoplasmacytic cells rather than pure plasma cells and is clinically distinguished by hyperviscosity and lymphadenopathy rather than bone lesions. * **Primary Amyloidosis (AL):** While associated with plasma cell dyscrasias (deposition of light chain fragments), it is a systemic metabolic complication rather than the most common primary tumor itself. **High-Yield Clinical Pearls for NEET-PG:** * **Most common symptom:** Bone pain (usually involving the back/ribs). * **Diagnostic Hallmark:** Presence of >10% clonal plasma cells in bone marrow [2]. * **Peripheral Smear:** **Rouleaux formation** (due to decreased zeta potential from high globulins). * **Urine:** Bence-Jones proteins (detected by sulfosalicylic acid test, not by standard dipstick). * **Radiology:** "Punched-out" lytic lesions; **Skull X-ray** is a classic board favorite [2]. * **Note:** Bone scans are often negative in MM because they detect osteoblastic activity, whereas MM is primarily osteolytic. Use a Skeletal Survey or MRI instead.

Question 534: Which of the following conditions is treated with the ABVD chemotherapy regimen?

A. Chronic lymphocytic leukemia
B. Acute lymphoblastic leukemia
C. Non-Hodgkin's lymphoma
D. Hodgkin's disease (Correct Answer)

Explanation: **Explanation:** The **ABVD regimen** is the gold standard first-line chemotherapy for **Hodgkin’s Lymphoma (HL)** [1]. It was developed to provide high cure rates while minimizing the long-term risks (such as secondary leukemia and infertility) associated with older regimens like MOPP. The regimen consists of: * **A:** **A**driamycin (Doxorubicin) – An anthracycline (Side effect: Cardiotoxicity). * **B:** **B**leomycin – A cytotoxic antibiotic (Side effect: Pulmonary fibrosis). * **V:** **V**inblastine – A microtubule inhibitor (Side effect: Bone marrow suppression). * **D:** **D**acarbazine – An alkylating agent (Side effect: Highly emetogenic). **Analysis of Incorrect Options:** * **Chronic Lymphocytic Leukemia (CLL):** Typically treated with targeted therapies like Ibrutinib or chemo-immunotherapy regimens such as **FCR** (Fludarabine, Cyclophosphamide, Rituximab) [2]. * **Acute Lymphoblastic Leukemia (ALL):** Requires a complex multi-phase protocol (Induction, Consolidation, Maintenance) involving drugs like Vincristine, Prednisolone, L-Asparaginase, and Daunorubicin. * **Non-Hodgkin’s Lymphoma (NHL):** The standard treatment for the most common subtype (DLBCL) is **R-CHOP** (Rituximab, Cyclophosphamide, Hydroxydaunorubicin, Oncovin/Vincristine, and Prednisolone) [2]. **High-Yield Clinical Pearls for NEET-PG:** * **Stanford V** and **BEACOPP** are other regimens used in advanced or refractory Hodgkin’s Disease. * **Brentuximab Vedotin** (anti-CD30 antibody-drug conjugate) is used in relapsed HL. * **Reed-Sternberg cells** (Owl-eye appearance) are the hallmark of Hodgkin’s Disease; they are typically **CD15+ and CD30+** [1]. * Monitoring: Patients on ABVD should undergo **Pulmonary Function Tests (PFTs)** for Bleomycin toxicity and **Echocardiography** for Doxorubicin cardiotoxicity.

Question 535: Which of the following conditions is not characterized by palpable purpura?

A. Henoch-Schonlein purpura (HSP)
B. Scurvy
C. Mixed cryoglobulinemia
D. Immune thrombocytopenic purpura (ITP) (Correct Answer)

Explanation: ### Explanation The key to answering this question lies in distinguishing between **vasculitic purpura** and **thrombocytopenic purpura**. **1. Why ITP is the correct answer:** Immune Thrombocytopenic Purpura (ITP) is characterized by a low platelet count due to peripheral destruction [1]. In ITP, the skin lesions are **non-palpable (flat)** petechiae or ecchymoses. Because the underlying pathology is a deficiency in platelets rather than inflammation of the blood vessel wall, there is no structural thickening or inflammatory infiltrate to make the lesion feel raised or "palpable." **2. Analysis of Incorrect Options:** * **Henoch-Schönlein Purpura (HSP):** This is an IgA-mediated small-vessel vasculitis. The inflammation and leukocytoclasis (vessel wall damage) lead to localized edema and extravasation, resulting in the classic "palpable purpura," typically on the lower extremities [1]. * **Mixed Cryoglobulinemia:** This is a systemic vasculitis often associated with Hepatitis C. It involves immune complex deposition in small vessels, causing inflammatory damage that manifests as palpable purpura. * **Scurvy (Vitamin C deficiency):** While often associated with perifollicular hemorrhages, Scurvy can present with palpable purpuric lesions. This is due to defective collagen synthesis leading to weakened vascular walls and secondary perifollicular inflammation. **Clinical Pearls for NEET-PG:** * **Palpable Purpura = Vasculitis:** Always think of small-vessel vasculitis (e.g., HSP, Microscopic polyangiitis, Churg-Strauss) when you see this term [1]. * **Non-palpable Purpura = Hematologic/Platelet issues:** Think of ITP, TTP, or bone marrow suppression [1]. * **HSP Tetrad:** Palpable purpura, arthralgia, abdominal pain, and renal involvement (hematuria). * **Rule of Thumb:** If the purpura is itchy or painful and raised, it is likely inflammatory (vasculitic). If it is flat and painless, it is likely a clotting or platelet defect.

Question 536: Which condition is associated with ADAMS13 mutation?

A. Thrombotic thrombocytopenic purpura (TTP) (Correct Answer)
B. Hemolytic uremic syndrome (HUS)
C. Disseminated intravascular coagulation (DIC)
D. Immune thrombocytopenic purpura (ITP)

Explanation: **Thrombotic Thrombocytopenic Purpura (TTP)** is the correct answer because its pathophysiology is directly linked to a deficiency or dysfunction of the enzyme **ADAMTS13** (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13). ADAMTS13 is responsible for cleaving large **von Willebrand Factor (vWF) multimers**. When ADAMTS13 is deficient (due to an acquired autoantibody or a rare congenital mutation), these "ultra-large" multimers persist, causing spontaneous platelet aggregation and microthrombi formation, leading to microangiopathic hemolytic anemia (MAHA) and thrombocytopenia [2]. **Why other options are incorrect:** * **Hemolytic Uremic Syndrome (HUS):** While clinically similar to TTP, HUS is typically caused by **Shiga toxin** (from *E. coli* O157:H7) or mutations in **complement regulatory proteins** (Atypical HUS), not ADAMTS13 [2]. * **Disseminated Intravascular Coagulation (DIC):** This is a consumption coagulopathy triggered by systemic inflammation or sepsis [1]. It is characterized by abnormal coagulation profiles (prolonged PT/aPTT and low fibrinogen), which are usually normal in TTP [1]. * **Immune Thrombocytopenic Purpura (ITP):** This is caused by anti-platelet antibodies leading to splenic destruction of platelets [3]. It does not involve ADAMTS13 or microangiopathic hemolysis (schistocytes). **High-Yield Clinical Pearls for NEET-PG:** * **The Classic Pentad of TTP:** (FAT RN) **F**ever, **A**nemia (MAHA), **T**hrombocytopenia, **R**enal failure, and **N**eurological symptoms. * **Diagnosis:** ADAMTS13 activity levels **<10%** are diagnostic. * **Treatment:** Emergency **Plasmapheresis (Plasma Exchange)** is the gold standard. Never delay treatment for lab confirmation. * **Contraindication:** Platelet transfusion is generally contraindicated as it may "fuel the fire" of thrombus formation.

Question 537: A patient presents with abundant spherocytes in the peripheral blood and a positive family history of anemia and splenectomy. All of the following statements regarding his condition are true, except:

A. Coombs test should be done to exclude an immunological cause.
B. Cholecystectomy may be required in the future.
C. The most common mode of inheritance is autosomal recessive. (Correct Answer)
D. Splenectomy may be required.

Explanation: ### **Explanation** The clinical presentation of **abundant spherocytes** and a **positive family history** of anemia and splenectomy strongly points toward **Hereditary Spherocytosis (HS)**. **1. Why Option C is the Correct Answer (The False Statement):** The most common mode of inheritance for Hereditary Spherocytosis is **Autosomal Dominant (75% of cases)**, involving mutations in genes encoding membrane proteins like **Ankyrin (most common)**, Spectrin, or Band 3 [2]. While autosomal recessive forms exist, they are less common and typically present with more severe hemolysis. **2. Analysis of Other Options:** * **Option A (Coombs Test):** Spherocytes are also seen in **Autoimmune Hemolytic Anemia (AIHA)**. A negative Direct Coombs test is essential to differentiate the inherited membrane defect (HS) from an acquired immunological cause. * **Option B (Cholecystectomy):** Chronic extravascular hemolysis leads to increased bilirubin production, resulting in **pigment (calcium bilirubinate) gallstones**. Cholecystectomy is often performed concurrently with splenectomy if stones are symptomatic. * **Option D (Splenectomy):** The spleen is the primary site of destruction for spherocytes [3]. Splenectomy is the **definitive treatment** for moderate to severe HS as it increases red cell lifespan, though it does not correct the underlying membrane defect [2]. ### **NEET-PG High-Yield Pearls:** * **Most common molecular defect:** Ankyrin mutation [2]. * **Pathophysiology:** Deficiency of membrane proteins → loss of surface area → formation of spherical cells → trapped in splenic cords [1], [2]. * **Diagnostic Tests:** Increased **MCHC** (highly characteristic) [1], increased osmotic fragility, and the **EMA Binding test** (Gold Standard/Most sensitive). * **Peripheral Smear:** Spherocytes (small, dark red cells lacking central pallor) and polychromasia (reticulocytosis). * **Complication:** Aplastic crisis triggered by **Parvovirus B19** infection.

Question 538: A 40-year-old man with a 10-year history of diabetes, currently on medication, presents with abdominal pain, weakness, lightheadedness, palpitations, and shortness of breath. On examination, he has glossitis, hyperpigmentation of the skin on the dorsum of his hands and feet, and abnormal hair pigmentation. His family reports increased irritability over the past year. Laboratory findings include Hb: 9 gm%, MCV: 110 fl, MCH: 36 Pg, MCHC: 34 gm/dl, Reticulocyte count: 0.1 x 10^9/L, LDH: 600 U/L, Indirect bilirubin: 1 mg/dL, S. iron: Normal, S. Ferritin: Normal, WBC: 2 x 10^9/L, and Platelet count: 90 x 10^9/L. Peripheral blood smear and bone marrow aspiration findings are also noted. Which of the following drugs is the patient most likely taking?

A. Metformin (Correct Answer)
B. Pioglitazone
C. Vildagliptin
D. Exenatide

Explanation: The patient presents with classic features of **Megaloblastic Anemia** due to **Vitamin B12 (Cobalamin) deficiency**. **1. Why Metformin is the Correct Answer:** Metformin is a first-line oral hypoglycemic agent known to cause Vitamin B12 deficiency in up to 30% of patients, especially with long-term use (as seen in this 10-year history). It interferes with the **calcium-dependent absorption** of the B12-intrinsic factor complex in the terminal ileum. * **Clinical Clues:** Glossitis, hyperpigmentation (a high-yield dermatological finding in B12 deficiency), and neuropsychiatric symptoms (irritability). * **Laboratory Clues:** Macrocytic anemia (MCV 110 fl), pancytopenia (low WBC and Platelets), and markers of **ineffective erythropoiesis** (elevated LDH and indirect bilirubin due to intramedullary hemolysis) [1]. **2. Why Other Options are Incorrect:** * **Pioglitazone (Thiazolidinedione):** Primarily associated with fluid retention, weight gain, and heart failure exacerbation; it does not cause macrocytic anemia. * **Vildagliptin (DPP-4 Inhibitor):** Generally weight-neutral and does not affect B12 absorption or hematological parameters. * **Exenatide (GLP-1 Agonist):** Associated with gastrointestinal side effects and weight loss, but not specifically linked to B12 deficiency or pancytopenia. **3. NEET-PG High-Yield Pearls:** * **Hyperpigmentation:** While B12 deficiency is usually associated with pallor, skin hyperpigmentation (knuckles/dorsum of hands) is a characteristic board-exam finding. * **Ineffective Erythropoiesis:** High LDH + Low Reticulocyte Count + Macrocytosis = Think B12/Folate deficiency [1]. * **Metformin Monitoring:** ADA guidelines suggest periodic monitoring of B12 levels in metformin-treated patients, especially those with anemia or peripheral neuropathy.

Question 539: Splenomegaly and reticulocytosis are typically absent in which type of anaemia?

A. G-6 PD deficiency anaemia
B. Myeloblastic anaemia
C. Iron deficiency anaemia
D. Aplastic anaemia (Correct Answer)

Explanation: **Explanation:** The correct answer is **Aplastic Anaemia**. To understand why, we must look at the pathophysiology of the bone marrow. **1. Why Aplastic Anaemia is correct:** Aplastic anaemia is characterized by **pancytopenia** resulting from **bone marrow failure** (hypocellular marrow). * **Reticulocytosis:** Reticulocytes are immature red cells released by a functioning marrow in response to anemia. In aplastic anaemia, the "factory" (marrow) is damaged; therefore, the reticulocyte count is characteristically low (reticulocytopenia). * **Splenomegaly:** The absence of splenomegaly is a hallmark of aplastic anaemia. If a patient has pancytopenia *with* an enlarged spleen, clinicians must look for alternative diagnoses like leukemia, myelofibrosis, or hypersplenism. **2. Why other options are incorrect:** * **G6PD Deficiency:** This is a hemolytic anemia. During an oxidative crisis, the marrow is healthy and responds by increasing RBC production, leading to **reticulocytosis**. Mild splenomegaly may occur during acute hemolytic episodes. * **Myeloblastic Anaemia (Leukemia):** Acute Myeloid Leukemia (AML) frequently presents with **splenomegaly** due to extramedullary hematopoiesis or leukemic infiltration. * **Iron Deficiency Anaemia (IDA):** While splenomegaly is rare in IDA, it can occur in severe, chronic cases (especially in pediatric populations or as part of Plummer-Vinson syndrome). More importantly, IDA can show a modest reticulocyte response once iron therapy is initiated. **Clinical Pearls for NEET-PG:** * **Definition of Aplastic Anaemia:** Corrected Reticulocyte Count <1% and Bone Marrow cellularity <25%. * **Fanconi Anaemia:** The most common inherited cause of aplastic anaemia (look for short stature, thumb deformities, and café-au-lait spots). * **Rule of Thumb:** In any case of pancytopenia, the **presence of splenomegaly** strongly points *away* from aplastic anaemia and *towards* malignancy or storage disorders.

Question 540: A patient presents with microcytic hypochromic anemia, Hb of 9 g/dL, serum iron of 20 µg/dL, ferritin level of 800 ng/mL, and transferrin saturation of 64%. What is the most likely diagnosis?

A. Atransferrinemia (Correct Answer)
B. Iron deficiency anemia
C. DMT1 mutation
D. Sideroblastic anemia

Explanation: **Explanation:** The clinical presentation of microcytic hypochromic anemia with paradoxical iron overload (high ferritin and high transferrin saturation) is the hallmark of **Atransferrinemia** (Congenital Hypotransferrinemia). **1. Why Atransferrinemia is correct:** In this rare autosomal recessive disorder, there is a deficiency of transferrin, the primary protein responsible for transporting iron to the bone marrow for erythropoiesis [1]. * **Anemia:** Without transferrin, iron cannot be delivered to developing RBCs, leading to iron-deficient erythropoiesis and **microcytic hypochromic anemia** [1]. * **Iron Overload:** Because iron is not being utilized by the marrow, it deposits in peripheral tissues (liver, heart, pancreas). This results in **very high serum ferritin** and **high transferrin saturation** (since the total iron-binding capacity is extremely low, even a small amount of iron saturates the available protein). **2. Why other options are incorrect:** * **Iron Deficiency Anemia:** Characterized by **low** ferritin and **low** transferrin saturation [2]. * **DMT1 Mutation:** While it causes microcytic anemia and systemic iron overload, the serum iron and transferrin saturation are typically normal or low because the defect lies in iron transport *into* the cell, not transport *in the plasma*. * **Sideroblastic Anemia:** While it shows high ferritin and saturation, the primary defect is in heme synthesis within the mitochondria (often showing ring sideroblasts). Atransferrinemia is a more specific fit for the combination of profound transferrin deficiency and systemic overload. **Clinical Pearls for NEET-PG:** * **Atransferrinemia** is a "Paradoxical Iron State": Tissue iron overload + Erythroid iron deficiency. * **Treatment:** Periodic infusions of plasma or purified transferrin. * **Differential for High Ferritin + Microcytic Anemia:** Sideroblastic anemia, Thalassemia, Lead poisoning, and Atransferrinemia [2].

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Multiple Myeloma and Plasma Cell Disorders

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Hematology — MCQs

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