Hematology — MCQs

A 30-year-old woman presents with fatigue, dark "tea color" urine, and yellowish eyes. Her past medical history includes myasthenia gravis treated with azathioprine and pyridostigmine. Laboratory findings are consistent with autoimmune hemolytic anemia. A chest X-ray shows an anterior mediastinal mass. What is the most likely diagnosis?

Q609Easy

Common findings in hemochromatosis include all of the following except?

Q610Medium

Which of the following is NOT a poor prognostic factor for Hodgkin's disease?

Hematology Indian Medical PG Practice Questions and MCQs

Question 601: A patient presents with increased serum ferritin, decreased TIBC, increased serum iron, and increased % saturation. What is the most probable diagnosis?

A. Anemia of chronic disease
B. Sideroblastic anemia (Correct Answer)
C. Iron deficiency anemia
D. Thalassemia minor

Explanation: ### Explanation The correct answer is **Sideroblastic Anemia**. This condition is characterized by a defect in heme synthesis, leading to the accumulation of iron within the mitochondria of erythroid precursors (forming ringed sideroblasts). Because iron cannot be incorporated into hemoglobin, it leaks out of the cells, leading to **iron overload**. #### Why Sideroblastic Anemia is Correct: In Sideroblastic anemia, the body has an abundance of iron that it cannot utilize. This results in: * **Increased Serum Iron & % Saturation:** Iron is not being used for heme synthesis. * **Increased Ferritin:** Reflects high systemic iron stores. * **Decreased TIBC:** A compensatory response to high iron levels (the liver produces less transferrin when iron is plentiful). #### Why Other Options are Incorrect: * **Iron Deficiency Anemia (IDA):** This is the polar opposite. You would see **decreased** ferritin, **decreased** serum iron, and **increased** TIBC (the body’s attempt to "catch" more iron) [2]. * **Anemia of Chronic Disease (ACD):** While ferritin is increased (as an acute phase reactant), the hallmark is **decreased serum iron** and **decreased TIBC** because iron is "trapped" inside macrophages and unavailable for circulation [1]. * **Thalassemia Minor:** Usually presents with a normal iron profile. It is a defect in globin chain synthesis, not iron metabolism. #### NEET-PG High-Yield Pearls: * **Gold Standard Diagnosis:** Bone marrow aspiration showing **Ringed Sideroblasts** (Prussian Blue stain). * **Common Causes:** Alcohol (most common), Lead poisoning, Isoniazid (B6 deficiency), and X-linked ALAS2 mutation. * **Treatment:** Pyridoxine (Vitamin B6) is the first-line treatment for sideroblastic anemia, as it is a cofactor for the ALAS enzyme.

Question 602: Which of the following is NOT a cause of microcytic hypochromic anemia?

A. Sickle cell disease (Correct Answer)
B. Aplastic anemia
C. Iron deficiency anemia
D. Hereditary spherocytosis

Explanation: Microcytic hypochromic anemia is characterized by a Mean Corpuscular Volume (MCV) < 80 fL and a Mean Corpuscular Hemoglobin Concentration (MCHC) < 32 g/dL. This occurs due to defects in hemoglobin synthesis (heme or globin). **Why Sickle Cell Disease is the correct answer:** Sickle cell disease (SCD) is a qualitative hemoglobinopathy [1] (substitution of valine for glutamic acid) [2]. It typically presents as a **Normocytic Normochromic Anemia** with a high reticulocyte count due to its hemolytic nature. While chronic hemolysis can lead to secondary iron deficiency (microcytosis), the primary disease process itself is normocytic. **Analysis of other options:** * **Iron Deficiency Anemia (IDA):** The most common cause of microcytic hypochromic anemia worldwide due to defective heme synthesis [2]. * **Aplastic Anemia:** This is a bone marrow failure syndrome characterized by pancytopenia. It typically presents as **Normocytic** or occasionally **Macrocytic** anemia (due to stress erythropoiesis), but never microcytic. * **Hereditary Spherocytosis:** This is a red cell membrane defect. It typically presents as **Normocytic** anemia, but it is unique for having an **elevated MCHC** (hyperchromic appearance) due to membrane loss and cellular dehydration. *(Note: The question as provided contains a technical discrepancy. Both A, B, and D are typically NOT microcytic. However, in the context of NEET-PG, if forced to choose the "most" correct among non-microcytic options, SCD is often grouped with hemolytic anemias which are normocytic.)* **NEET-PG High-Yield Pearls:** * **Mnemonic for Microcytic Anemia (TAILS):** **T**halassemia, **A**nemia of chronic disease (late), **I**ron deficiency, **L**ead poisoning, **S**ideroblastic anemia. * **Mentzer Index (MCV/RBC):** <13 suggests Thalassemia; >13 suggests Iron Deficiency Anemia. * **Hereditary Spherocytosis:** Look for increased MCHC and a positive Osmotic Fragility Test.

Question 603: Which of the following is associated with Chronic Lymphocytic Leukemia (CLL)?

A. Individuals over 50 years of age
B. Mature small lymphocytes in peripheral smear
C. Hepatosplenomegaly and lymphadenopathy
D. All of the above (Correct Answer)

Explanation: Chronic Lymphocytic Leukemia (CLL) is the most common leukemia in the Western world, characterized by the monoclonal proliferation and accumulation of functionally incompetent, **mature B-lymphocytes**. 1. **Age (Option A):** CLL is primarily a disease of the elderly [1]. The median age at diagnosis is approximately 70 years, and it is rarely seen in individuals under 40. Therefore, being over 50 years of age is a significant demographic association. 2. **Peripheral Smear (Option B):** The hallmark of CLL is an absolute lymphocytosis (>5,000/µL). On a blood film, these cells appear as **mature, small lymphocytes** with a narrow rim of cytoplasm and condensed "block-type" chromatin. A classic finding is the presence of **Smudge cells** (Basket cells), which are fragile lymphocytes ruptured during slide preparation. 3. **Clinical Features (Option C):** While many patients are asymptomatic at diagnosis (detected on routine CBC), progressive disease leads to painless **generalized lymphadenopathy** and **hepatosplenomegaly** due to infiltration by leukemic cells [1]. **Conclusion:** Since all three statements accurately describe the demographic, morphological, and clinical profile of CLL, **Option D** is the correct answer. **High-Yield Clinical Pearls for NEET-PG:** * **Immunophenotype:** Characteristically positive for **CD5** (a T-cell marker), CD19, CD20 (weak), and **CD23**. * **Richter Transformation:** The sudden transformation of CLL into an aggressive High-Grade B-cell Lymphoma (usually DLBCL). * **Complications:** Hypogammaglobulinemia (leading to recurrent infections) and Autoimmune Hemolytic Anemia (AIHA) [1]. * **Staging:** Uses the **Rai System** (USA) or **Binet Classification** (Europe) [1].

Question 604: A young person presents with a history of severe menorrhagia, palpable spleen, prolonged bleeding time, and normal clotting time and platelet count. What is the most likely diagnosis?

A. Hemophilia
B. Henoch-Schonlein purpura
C. Thrombasthenia (Correct Answer)
D. Allergic purpura

Explanation: ### Explanation The clinical presentation points toward a **qualitative platelet disorder**. The key findings are a **prolonged bleeding time (BT)** with a **normal platelet count** and **normal clotting time (CT)**. **1. Why Thrombasthenia is Correct:** **Glanzmann Thrombasthenia (GT)** is an autosomal recessive disorder caused by a deficiency or dysfunction of the **GP IIb/IIIa receptor**. This receptor is essential for fibrinogen binding and platelet aggregation. * **Clinical Clues:** Severe mucosal bleeding (menorrhagia, epistaxis) [1] and a palpable spleen (splenomegaly can occur in some chronic platelet disorders, though less common than in ITP [1]). * **Lab Profile:** Normal platelet count (ruling out thrombocytopenia) and normal CT (ruling out coagulation factor deficiencies), but prolonged BT due to the inability of platelets to aggregate and form a primary hemostatic plug. **2. Why Other Options are Incorrect:** * **Hemophilia:** This is a disorder of secondary hemostasis (Factor VIII or IX deficiency) [2]. It typically presents with deep tissue bleeds/hemarthrosis, a **prolonged Activated Partial Thromboplastin Time (aPTT)**, and a **normal bleeding time**. * **Henoch-Schönlein Purpura (HSP) / Allergic Purpura:** These are forms of small-vessel vasculitis. While they present with purpura, the **bleeding time and platelet counts are typically normal** because the pathology lies in the vessel wall inflammation, not platelet function or number [1]. **3. NEET-PG High-Yield Pearls:** * **Glanzmann Thrombasthenia:** Defect in Aggregation; failure to respond to ADP, epinephrine, or collagen on platelet aggregometry; **normal response to Ristocetin**. * **Bernard-Soulier Syndrome:** Defect in Adhesion (GP Ib-IX-V) [1]; characterized by **giant platelets**, thrombocytopenia, and **failure to aggregate with Ristocetin**. * **Von Willebrand Disease (vWD):** Most common inherited bleeding disorder; presents with prolonged BT and often a prolonged aPTT (due to low Factor VIII levels).

Question 605: Which of the following is characteristic of Chronic Myeloid Leukemia?

A. The ABL gene on chromosome 22 is translocated to the BCR gene on chromosome 9.
B. The fusion gene bcr-abl forms a protein with tyrosine kinase activity. (Correct Answer)
C. Splenomegaly is unusual.
D. Philadelphia chromosome positive patients respond poorly to Imatinib.

Explanation: Chronic Myeloid Leukemia (CML) is a myeloproliferative neoplasm characterized by the presence of the **Philadelphia (Ph) chromosome**, which results from a reciprocal translocation between chromosomes 9 and 22, denoted as **t(9;22)(q34;q11)** [1]. 1. **Why Option B is Correct:** The translocation fuses the *BCR* (Breakpoint Cluster Region) gene on chromosome 22 with the *ABL1* (Abelson murine leukemia viral oncogene homolog 1) gene from chromosome 9 [1], [2]. This **BCR-ABL1 fusion gene** encodes a chimeric protein (p210) with **constitutive tyrosine kinase activity** [1]. This protein bypasses normal cellular regulation, leading to uncontrolled proliferation of myeloid cells and inhibition of apoptosis. 2. **Why Other Options are Incorrect:** * **Option A:** This is a common distractor. In CML, the *ABL* gene on chromosome 9 is translocated to the *BCR* gene on **chromosome 22** (not the other way around) [1]. The resulting shortened chromosome 22 is the Philadelphia chromosome. * **Option C:** **Splenomegaly** is the most common physical finding in CML, present in over 50-70% of patients. It is often massive and correlates with the peripheral white cell count. * **Option D:** Imatinib mesylate is a selective **tyrosine kinase inhibitor (TKI)** that specifically targets the BCR-ABL protein [1], [2]. Ph+ patients generally show an excellent response to Imatinib, which has revolutionized CML treatment [2]. **High-Yield Clinical Pearls for NEET-PG:** * **Leukocyte Alkaline Phosphatase (LAP) Score:** Characteristically **decreased** in CML (helps differentiate it from a Leukemoid reaction where LAP is high). * **Peripheral Smear:** Shows a "whole spectrum" of myeloid cells (myeloblasts to mature neutrophils) with a characteristic **"myelocytic bulge"** (excess of myelocytes and metamyelocytes). * **Basophilia:** An increase in absolute basophil count is a classic hallmark of CML. * **Gold Standard Diagnosis:** Cytogenetics (Karyotyping) for t(9;22) or FISH/RT-PCR for the *BCR-ABL1* gene [2].

Question 606: What is the most common cause for complications of blood transfusion?

A. Human error (Correct Answer)
B. Anaphylaxis
C. Graft-versus-host disease (GVHD)
D. Presensitization

Explanation: Explanation: 1. Why Human Error is the Correct Answer: Despite advancements in screening and cross-matching technology, human error remains the leading cause of transfusion-related complications and fatalities. This most commonly manifests as ABO incompatibility [1] due to clerical mistakes, such as mislabeling a sample, improper patient identification at the bedside, or administering the wrong unit to the wrong patient. Studies consistently show that technical failures are rare compared to procedural lapses in the "chain of transfusion." [1] 2. Why the Other Options are Incorrect: * Anaphylaxis: While serious, IgA-deficient patients experiencing anaphylaxis represent a very small percentage of transfusion reactions. * Graft-versus-host disease (GVHD): This is a rare, albeit fatal, complication occurring primarily in immunocompromised patients or when receiving blood from HLA-homozygous donors (e.g., directed donations from relatives). It is prevented by irradiation, not a common occurrence. * Presensitization: This leads to delayed hemolytic transfusion reactions or febrile non-hemolytic reactions due to prior exposure (pregnancy/transfusion). While frequent, these are statistically less common as a primary cause of major morbidity compared to errors in the administration process. 3. High-Yield Clinical Pearls for NEET-PG: * Most common overall reaction: Febrile Non-Hemolytic Transfusion Reaction (FNHTR) [1]. * Most common cause of transfusion-related death: Historically ABO incompatibility (human error), but in modern well-monitored settings, TRALI (Transfusion-Related Acute Lung Injury) is often cited as the leading cause of mortality. * Bedside Check: The final "clerical check" at the patient's bedside is the most critical step in preventing fatal hemolytic reactions [2]. * Bacterial Contamination: Most common with Platelets (stored at room temperature). [1] Davidson's Principles and Practice of Medicine, 22E, pp. 1033-1037.

Question 607: Pancytopenia with hypercellular marrow is due to all EXCEPT:

A. Myelodysplasia
B. Paroxysmal nocturnal hemoglobinuria
C. Sarcoidosis
D. Dyskeratosis congenita (Correct Answer)

Explanation: **Explanation:** The core concept of this question lies in differentiating between **"Ineffective Hematopoiesis"** (where the marrow is full of cells that fail to mature or enter circulation) and **"Aplastic/Hypoplastic Anemia"** (where the marrow is empty). **1. Why Dyskeratosis Congenita is the Correct Answer:** Dyskeratosis congenita is a congenital form of **Aplastic Anemia** caused by telomere biology disorders. It is characterized by a **hypocellular (empty) bone marrow**. Because the hematopoietic stem cells themselves are depleted, the marrow cannot be hypercellular. It classically presents with the triad of abnormal skin pigmentation, nail dystrophy, and oral leukoplakia. **2. Analysis of Incorrect Options (Causes of Hypercellular Marrow with Pancytopenia):** * **Myelodysplasia (MDS):** This is the classic example of "ineffective hematopoiesis." The marrow is hypercellular or normocellular, but the cells are dysplastic and undergo apoptosis before reaching the peripheral blood. * **Paroxysmal Nocturnal Hemoglobinuria (PNH):** While PNH can be associated with aplastic anemia, it frequently presents with a hypercellular marrow during hemolytic phases or when evolving from/into other myelodysplastic processes. * **Sarcoidosis:** This represents "marrow infiltration" (Myelophthisic anemia). Granulomas infiltrate the bone marrow, leading to a reactive hypercellularity of the non-involved marrow spaces or a "packed" appearance, despite low peripheral counts. **NEET-PG High-Yield Pearls:** * **Pancytopenia + Hypercellular Marrow:** Think Vitamin B12/Folate deficiency (Megaloblastic anemia), MDS, Aleukemic Leukemia, and Subleukemic Leukemia. * **Pancytopenia + Hypocellular Marrow:** Think Aplastic Anemia, Dyskeratosis Congenita, and Fanconi Anemia. * **MDS** is often called "Pre-leukemia" and is the most common cause of macrocytic pancytopenia in the elderly with a hypercellular marrow.

Question 608: A 30-year-old woman presents with fatigue, dark "tea color" urine, and yellowish eyes. Her past medical history includes myasthenia gravis treated with azathioprine and pyridostigmine. Laboratory findings are consistent with autoimmune hemolytic anemia. A chest X-ray shows an anterior mediastinal mass. What is the most likely diagnosis?

A. thymoma (Correct Answer)
B. nodular sclerosing Hodgkin disease
C. small cleaved cell non-Hodgkin lymphoma
D. teratoma

Explanation: **Explanation:** The clinical presentation describes a classic triad: **Myasthenia Gravis (MG)**, **Autoimmune Hemolytic Anemia (AIHA)**, and an **Anterior Mediastinal Mass**. This constellation is highly characteristic of a **Thymoma**. **1. Why Thymoma is correct:** Thymomas are epithelial tumors of the thymus gland. They are unique for their strong association with paraneoplastic autoimmune syndromes due to the failure of T-cell self-tolerance within the tumor. * **Myasthenia Gravis:** The most common association (seen in ~30-45% of thymoma patients). All patients with MG should have a thoracic CT to exclude thymoma [1]. * **Hematologic disorders:** While Pure Red Cell Aplasia (PRCA) is the most classic hematologic association, **AIHA** (causing the "tea-colored" urine/jaundice) and Hypogammaglobulinemia (Good Syndrome) are also well-documented. **2. Why incorrect options are wrong:** * **Nodular Sclerosing Hodgkin Disease:** While it is the most common mediastinal lymphoma in young women, it typically presents with B-symptoms (fever, weight loss) and lymphadenopathy, not paraneoplastic MG or AIHA. * **Small Cleaved Cell NHL:** Usually presents with disseminated lymphadenopathy in older adults; it is not specifically associated with MG. * **Teratoma:** A common germ cell tumor in the anterior mediastinum, but it is usually asymptomatic or causes local pressure symptoms. It does not trigger the T-cell dysregulation required to cause MG or AIHA. **Clinical Pearls for NEET-PG:** * **Anterior Mediastinal Mass (The 4 Ts):** **T**hymoma, **T**eratoma (Germ cell tumors), **T**errible Lymphoma, and **T**hyroid (Retrosternal goiter). * **Thymoma Associations:** MG (most common), PRCA (most specific hematologic), and Good Syndrome (hypogammaglobulinemia). * **Management:** Surgical resection (thymectomy) is the treatment of choice and may improve the associated autoimmune conditions [1].

Question 609: Common findings in hemochromatosis include all of the following except?

A. Diabetes mellitus
B. Diabetes insipidus (Correct Answer)
C. Impotence
D. Hepatocellular carcinoma

Explanation: **Explanation:** Hereditary Hemochromatosis is an autosomal recessive disorder characterized by excessive iron absorption and deposition in various organs, leading to tissue damage (siderosis) [1]. **1. Why Diabetes Insipidus is the Correct Answer:** Iron deposition in the pituitary gland primarily affects the **anterior pituitary** (adenohypophysis), specifically the gonadotroph cells. This leads to hypogonadotropic hypogonadism. **Diabetes Insipidus**, which results from a deficiency of ADH (produced in the hypothalamus and stored in the posterior pituitary), is **not** a feature of hemochromatosis. Iron rarely involves the posterior pituitary or the vasopressin-secreting neurons. **2. Analysis of Incorrect Options:** * **Diabetes Mellitus (Option A):** Known as "Bronze Diabetes," this occurs due to direct iron deposition in the pancreatic beta cells (leading to insulin deficiency) and associated liver cirrhosis (leading to insulin resistance) [1]. * **Impotence (Option B):** This is a very common early symptom in males, caused by iron deposition in the anterior pituitary leading to decreased LH/FSH secretion (secondary hypogonadism), rather than direct testicular failure [1]. * **Hepatocellular Carcinoma (Option D):** Cirrhosis is a major complication of hemochromatosis. Patients with cirrhosis due to iron overload have a significantly higher risk (up to 20-200 fold) of developing HCC compared to the general population [1]. **NEET-PG High-Yield Pearls:** * **Classic Triad:** Cirrhosis, Diabetes Mellitus, and Skin Pigmentation (Bronze skin) [1]. * **Most Common Early Symptom:** Lethargy/Fatigue; however, **Arthropathy** (specifically involving the 2nd and 3rd MCP joints with "hook-like" osteophytes) is a specific diagnostic clue. * **Cardiac Involvement:** Can present as restrictive or dilated cardiomyopathy. * **Screening:** Transferrin saturation is the best initial test (>45% is suggestive). * **Gold Standard:** Liver biopsy (Perls' Prussian blue stain) or MRI (T2* weighted) to quantify iron [2].

Question 610: Which of the following is NOT a poor prognostic factor for Hodgkin's disease?

A. Younger age (Correct Answer)
B. Systemic manifestations
C. Lymphocyte depletion
D. Stomach involvement

Explanation: In Hodgkin’s Lymphoma (HL), prognosis is determined by clinical stage, histological subtype, and specific laboratory parameters. **Explanation of the Correct Answer:** **A. Younger age:** This is a **favorable** prognostic factor. Epidemiologically, HL shows a bimodal age distribution. Younger patients (typically <45 years) generally have a better performance status, fewer comorbidities, and a higher tolerance for intensive chemotherapy/radiotherapy compared to older patients (>45–50 years), who often present with more aggressive disease and poorer outcomes. **Explanation of Incorrect Options:** * **B. Systemic manifestations:** Also known as **"B-symptoms"** (fever >38°C, drenching night sweats, and weight loss >10% in 6 months) [1]. Their presence indicates a higher tumor burden and is a well-established poor prognostic indicator. * **C. Lymphocyte depletion:** This is the **least common** and **worst prognosis** histological subtype of HL. It is characterized by a high number of Reed-Sternberg cells and a lack of reactive lymphocytes, often seen in HIV-positive or elderly patients. * **D. Stomach involvement:** Extranodal involvement (Stage IV) significantly worsens the prognosis [1]. Involvement of the gastrointestinal tract, bone marrow, or liver indicates advanced disseminated disease. **NEET-PG High-Yield Pearls:** * **Best Prognosis Subtype:** Lymphocyte Predominant (often presents in early stages). * **Most Common Subtype:** Nodular Sclerosis (common in young females; associated with mediastinal mass). * **Hasford/International Prognostic Index (IPI) for HL (Advanced Stage):** Poor factors include Age ≥45, Male sex, Stage IV, Albumin <4 g/dL, Hemoglobin <10.5 g/dL, WBC ≥15,000/µL, and Lymphocytes <600/µL (or <8%). * **Staging System:** Ann Arbor Staging (modified by Cotswolds) [1].

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Hemoglobinopathies

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Thalassemias

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Platelet Disorders

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Coagulation Disorders

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Thrombotic Disorders

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Leukemias

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Lymphomas

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Multiple Myeloma and Plasma Cell Disorders

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Myeloproliferative Neoplasms

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Hematology — MCQs

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