Hematology Practice Questions

Q: What is a common cause of acute chest syndrome?

Sickle cell disease. **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.

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

Increased Transferrin Saturation. **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).

Q: What is the most common plasma cell tumor?

Multiple myeloma. **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.

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

Hodgkin's disease. **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.

Q: Which condition is associated with ADAMS13 mutation?

Thrombotic thrombocytopenic purpura (TTP). **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.

Q: 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?

Metformin. 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.

Q: 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?

Atransferrinemia. **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].

Question 1

What is a common cause of acute chest syndrome?

Accepted Answer

Sickle cell disease

Answer

Pneumonia

Answer

Acute myocardial infarction

Answer

Penetrating chest trauma

Question 2

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

Accepted Answer

Increased Transferrin Saturation

Answer

Increased Serum Iron

Answer

Increased Serum Ferritin

Answer

Increased Liver Enzymes

Question 3

What is the most common plasma cell tumor?

Accepted Answer

Multiple myeloma

Answer

Plasmacytoma

Answer

Waldenstrom's macroglobulinemia

Answer

Primary amyloidosis

Question 4

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

Accepted Answer

Hodgkin's disease

Answer

Chronic lymphocytic leukemia

Answer

Acute lymphoblastic leukemia

Answer

Non-Hodgkin's lymphoma

Question 5

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

Accepted Answer

Immune thrombocytopenic purpura (ITP)

Answer

Henoch-Schonlein purpura (HSP)

Answer

Scurvy

Answer

Mixed cryoglobulinemia

Question 6

Which condition is associated with ADAMS13 mutation?

Accepted Answer

Thrombotic thrombocytopenic purpura (TTP)

Answer

Hemolytic uremic syndrome (HUS)

Answer

Disseminated intravascular coagulation (DIC)

Answer

Immune thrombocytopenic purpura (ITP)

Question 7

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:

Accepted Answer

The most common mode of inheritance is autosomal recessive.

Answer

Coombs test should be done to exclude an immunological cause.

Answer

Cholecystectomy may be required in the future.

Answer

Splenectomy may be required.

Question 8

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?

Accepted Answer

Metformin

Answer

Pioglitazone

Answer

Vildagliptin

Answer

Exenatide

Question 9

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

Accepted Answer

Aplastic anaemia

Answer

G-6 PD deficiency anaemia

Answer

Myeloblastic anaemia

Answer

Iron deficiency anaemia

Question 10

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?

Accepted Answer

Atransferrinemia

Answer

Iron deficiency anemia

Answer

DMT1 mutation

Answer

Sideroblastic anemia

Hematology — MCQs

Hematology — MCQs

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