Hematology Practice Questions

Q: Which tumor is associated with polycythemia?

Cerebellar hemangioblastoma. ### Explanation The correct answer is **Cerebellar hemangioblastoma**. **1. Why Cerebellar Hemangioblastoma is Correct:** Polycythemia in the context of malignancy is usually a **paraneoplastic syndrome** caused by the ectopic production of **Erythropoietin (EPO)**. Cerebellar hemangioblastoma is a classic "EPO-secreting tumor" [1]. These tumors are highly vascular and are frequently associated with **Von Hippel-Lindau (VHL) syndrome**. The excess EPO stimulates the bone marrow to increase red blood cell production, leading to secondary polycythemia (erythrocytosis) [1]. **2. Why Other Options are Incorrect:** * **Sarcoma:** While some rare sarcomas (like uterine fibroids/leiomyomas) have been sporadically linked to erythrocytosis, "Sarcoma" as a general category is not a classic or high-yield association for polycythemia. * **Pituitary Adenoma:** These tumors typically present with hormonal excesses (like GH in acromegaly or ACTH in Cushing’s) or mass effect symptoms (visual field defects). They do not typically secrete EPO. **3. High-Yield Clinical Pearls for NEET-PG:** To master questions on ectopic EPO production, remember the mnemonic **"Potentially Really High Hematocrit"**: * **P:** **P**heochromocytoma * **R:** **R**enal Cell Carcinoma (The most common cause) [1] * **H:** **H**epatocellular Carcinoma (HCC) [1] * **H:** **H**emangioblastoma (Cerebellar) [1] * **Uterine Leiomyoma:** Occasionally associated (Myomatous Erythrocytosis Syndrome) [1]. **Key Distinction:** * **Primary Polycythemia (Polycythemia Vera):** Low EPO levels (due to JAK2 mutation). * **Secondary Polycythemia (Paraneoplastic):** High EPO levels (due to ectopic secretion by the tumors listed above) [1].

Q: A 16-year-old female presents with generalized weakness and palpitations. Her Hb is 7 g/dL and peripheral smear shows microcytic hypochromic anemia. Reticulocyte count is 0.8%. Serum bilirubin is 1 mg%. What is the most likely diagnosis?

Iron deficiency anemia. ### Explanation The correct diagnosis is **Iron Deficiency Anemia (IDA)**. This conclusion is based on the integration of the clinical presentation and laboratory findings: 1. **Microcytic Hypochromic Anemia:** The peripheral smear shows small, pale red blood cells [2]. This is the hallmark of impaired hemoglobin synthesis, most commonly caused by iron deficiency [1]. 2. **Low Reticulocyte Count (0.8%):** A reticulocyte count <2% indicates an **underproduction anemia** (bone marrow failure or nutrient deficiency) rather than a regenerative response to blood loss or hemolysis. 3. **Normal Bilirubin:** A serum bilirubin of 1 mg% (normal range) rules out significant active hemolysis. #### Why the other options are incorrect: * **Hemolytic Anemia:** While it can cause weakness, it typically presents with a **high reticulocyte count** (compensatory erythropoiesis) and **indirect hyperbilirubinemia** (jaundice) due to RBC breakdown. * **Aplastic Anemia:** This presents as **pancytopenia** (low RBCs, WBCs, and platelets). While the reticulocyte count is low, the smear is usually **normocytic normochromic**, not microcytic. * **Folic Acid Deficiency:** This is a megaloblastic anemia characterized by **macrocytic** (large) RBCs and hypersegmented neutrophils on the peripheral smear. #### NEET-PG High-Yield Pearls: * **Most common cause of microcytic hypochromic anemia:** Iron Deficiency Anemia (Global and India) [1]. * **Mentzer Index:** (MCV/RBC count) >13 suggests IDA; <13 suggests Thalassemia trait. * **Earliest sign of response to Iron therapy:** Increase in reticulocyte count (peaks at 7–10 days). * **Serum Ferritin:** The most sensitive and specific biochemical test to diagnose iron deficiency.

Q: Hepatitis-associated aplastic anemia is caused by which type of hepatitis virus?

Non-A, Non-B, Non-C hepatitis viruses. Explanation: Hepatitis-associated aplastic anemia (HAAA) is a distinct clinical syndrome where pancytopenia follows an episode of acute hepatitis. Why the correct answer is right: In the majority of cases (up to 90%), the causative agent is not one of the known hepatotropic viruses (A, B, C, D, or E). Instead, it is attributed to Non-A, Non-B, Non-C, Non-E hepatitis viruses [1]. The pathophysiology involves an immune-mediated destruction of hematopoietic stem cells. It is believed that an unknown virus triggers a T-cell-mediated (CD8+ cytotoxic T-lymphocytes) inflammatory response that cross-reacts with bone marrow antigens, leading to marrow aplasia. Why other options are incorrect: * Hepatitis A, B, and C: While there are rare case reports of aplastic anemia following infection with these specific viruses, they are statistically insignificant causes of HAAA. In standard clinical practice and for examination purposes, HAAA is specifically linked to seronegative (Non-A-E) hepatitis. High-Yield Clinical Pearls for NEET-PG: * Demographics: Most commonly affects young males. * Timeline: Aplastic anemia typically develops 2 to 3 months after the peak of the acute hepatitis episode. * Severity: The hepatitis is usually mild or resolving when the anemia begins, but the subsequent bone marrow failure is often severe or even fatal if untreated. * Treatment: The treatment of choice is Allogeneic Bone Marrow Transplantation (if a donor is available) or Immunosuppressive Therapy (IST) with Antithymocyte Globulin (ATG) and Cyclosporine. * Prognosis: Without treatment, the mortality rate is very high.

Q: A 25-year-old female presented in December with chronic fatigue and cyanosis with bluish lips and arthralgia. Peripheral blood film is shown below. What is the likely cause?

Cold autoimmune hemolytic anemia (AIHA). ***Cold autoimmune hemolytic anemia (AIHA)*** - Presentation in **December** (cold weather) with **cyanosis** and **bluish lips** indicates **cold agglutinins** causing vascular occlusion and hemolysis at low temperatures. - **RBC agglutination** (clumping) on peripheral blood smear is the hallmark feature, along with **fatigue** from hemolysis and **arthralgia** from immune complex deposition. *Warm autoimmune hemolytic anemia (AIHA)* - Occurs at **body temperature** (37°C) and would not be triggered by cold weather or cause **acrocyanosis**. - Peripheral blood smear shows **spherocytes** rather than **RBC agglutination**, and **direct Coombs test** is positive for **IgG**. *Hemoglobinopathy* - Structural or quantitative **hemoglobin disorders** like sickle cell disease or thalassemia would show characteristic **RBC morphology** (sickle cells, target cells). - Symptoms are typically **chronic** from childhood and not specifically triggered by **cold weather** exposure. *G6PD Deficiency* - **X-linked disorder** causing hemolysis triggered by **oxidative stress** from medications, foods (fava beans), or infections. - Peripheral blood smear shows **bite cells** and **Heinz bodies**, not **RBC agglutination**, and symptoms are episodic rather than cold-induced.

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

Sideroblastic anemia. ### 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.

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

Human error. 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.

Q: A 25-year-old patient presents with fatigue and abdominal pain. Examination reveals jaundice and splenomegaly. Ultrasound shows gallstones. What is the most likely diagnosis?

Hereditary spherocytosis. ### Explanation **Correct Answer: C. Hereditary spherocytosis** The clinical triad of **anemia (fatigue), jaundice, and splenomegaly** in a young patient, coupled with the presence of **gallstones**, is a classic presentation of chronic extravascular hemolysis. In **Hereditary Spherocytosis (HS)**, a defect in red blood cell (RBC) membrane proteins (most commonly **Ankyrin**, followed by Spectrin) leads to the formation of spherical, fragile RBCs. These spherocytes are trapped and destroyed in the splenic sinusoids, leading to splenomegaly. The chronic breakdown of RBCs results in unconjugated hyperbilirubinemia, which predisposes the patient to **pigment (calcium bilirubinate) gallstones**. **Why other options are incorrect:** * **A. Sickle cell anemia:** While it causes hemolysis and gallstones, the spleen in an adult sickle cell patient is usually small and fibrotic due to repeated infarctions (**autosplenectomy**), making splenomegaly unlikely at age 25. * **B. Acute pancreatitis:** Presents with severe epigastric pain radiating to the back and elevated amylase/lipase; it does not typically cause chronic jaundice or splenomegaly. * **D. Cholangitis:** Presents with Charcot’s Triad (fever, jaundice, RUQ pain). It is an acute bacterial infection of the biliary tree, not a chronic hemolytic process. **High-Yield NEET-PG Pearls:** * **Gold Standard Test:** Eosin-5-maleimide (EMA) binding test via flow cytometry. * **Screening Test:** Osmotic Fragility Test (increased fragility). * **Peripheral Smear:** Spherocytes (small, dark RBCs lacking central pallor) and reticulocytosis. * **MCHC:** Characteristically **increased** (the only anemia with high MCHC). * **Treatment of Choice:** Splenectomy (indicated in moderate to severe cases, usually after age 5 to reduce sepsis risk).

Question 1

Which tumor is associated with polycythemia?

Accepted Answer

Cerebellar hemangioblastoma

Answer

Sarcoma

Answer

Pituitary adenoma

Answer

None of the above.

Question 2

A 16-year-old female presents with generalized weakness and palpitations. Her Hb is 7 g/dL and peripheral smear shows microcytic hypochromic anemia. Reticulocyte count is 0.8%. Serum bilirubin is 1 mg%. What is the most likely diagnosis?

Accepted Answer

Iron deficiency anemia

Answer

Hemolytic anemia

Answer

Aplastic anemia

Answer

Folic acid deficiency

Question 3

A 69-year-old woman presents with a hip fracture sustained from a fall. She has a history of recurrent pneumonia over the past year. Laboratory findings include a normal white blood cell count, thrombocytopenia, and an elevated erythrocyte sedimentation rate (ESR). X-rays show multiple lytic bone lesions. Serum electrophoresis reveals an M-protein spike. What is the most likely diagnosis?

Accepted Answer

Multiple myeloma (plasma cell myeloma)

Answer

Chronic lymphocytic leukemia

Answer

Monoclonal gammopathy of uncertain significance

Answer

Plasmacytoma

Question 4

Hepatitis-associated aplastic anemia is caused by which type of hepatitis virus?

Accepted Answer

Non-A, Non-B, Non-C hepatitis viruses

Answer

Hepatitis A

Answer

Hepatitis B

Answer

Hepatitis C

Question 5

A 25-year-old female presented in December with chronic fatigue and cyanosis with bluish lips and arthralgia. Peripheral blood film is shown below. What is the likely cause?

Accepted Answer

Cold autoimmune hemolytic anemia (AIHA)

Answer

Warm autoimmune hemolytic anemia (AIHA)

Answer

Hemoglobinopathy

Answer

G6PD Deficiency

Question 6

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

Accepted Answer

Sideroblastic anemia

Answer

Anemia of chronic disease

Answer

Iron deficiency anemia

Answer

Thalassemia minor

Question 7

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?

Accepted Answer

Thrombasthenia

Answer

Hemophilia

Answer

Henoch-Schonlein purpura

Answer

Allergic purpura

Question 8

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

Accepted Answer

Human error

Answer

Anaphylaxis

Answer

Graft-versus-host disease (GVHD)

Answer

Presensitization

Question 9

A 67-year-old elderly male presents with headache, recurrent infections, and multiple punched-out lytic lesions on X-ray skull and lumbago for the past 1 month. Which investigation will be most helpful in establishing a diagnosis?

Accepted Answer

Protein electrophoresis

Answer

Serum calcium

Answer

Alkaline phosphatase levels

Answer

PSA levels

Question 10

A 25-year-old patient presents with fatigue and abdominal pain. Examination reveals jaundice and splenomegaly. Ultrasound shows gallstones. What is the most likely diagnosis?

Accepted Answer

Hereditary spherocytosis

Answer

Sickle cell anemia

Answer

Acute pancreatitis

Answer

Cholangitis

Hematology — MCQs

Hematology — MCQs

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