Hematopathology Practice Questions

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

Human error. **Explanation:** The most common cause of transfusion-related complications and fatalities is **Human Error**. Despite advanced screening for pathogens and immunological testing, clerical mistakes remain the leading risk factor. These errors typically occur during sample labeling, patient identification at the bedside, or issuing the wrong blood unit from the blood bank [1]. This leads to **ABO incompatibility**, which can trigger life-threatening acute hemolytic transfusion reactions (AHTR). **Analysis of Options:** * **Anaphylaxis (Option B):** While common among allergic reactions, it is far less frequent than clerical errors. It is usually caused by IgA deficiency in the recipient who has anti-IgA antibodies [1]. * **Graft-versus-host disease (Option C):** GVHD is a rare but highly fatal complication occurring in immunocompromised patients. It is prevented by gamma irradiation of blood products, not a "most common" occurrence. * **Presensitization (Option D):** This refers to the presence of pre-formed antibodies (from prior transfusions or pregnancies). While it causes delayed hemolytic reactions, modern cross-matching techniques have significantly reduced its incidence compared to human-led bedside errors. **High-Yield Clinical Pearls for NEET-PG:** * **Most common overall reaction:** Febrile Non-Hemolytic Transfusion Reaction (FNHTR), caused by cytokines or HLA antibodies. * **Most common cause of transfusion-related death:** Historically ABO incompatibility (human error) [1], but in many modern registries, **TRALI** (Transfusion-Related Acute Lung Injury) is cited as the leading cause of mortality. * **Golden Rule:** Always perform a double-check of patient identity and blood bag labels at the bedside to prevent the most common cause of morbidity. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 673-674.

Q: A 27-year-old black man develops sudden intravascular hemolysis 2 days after receiving the antimalarial drug primaquine, resulting in a decreased hematocrit, hemoglobinemia, and hemoglobinuria. Examination of the peripheral blood film is given. What is the most likely diagnosis?

Glucose-6-phosphate dehydrogenase deficiency. ***Glucose-6-phosphate dehydrogenase deficiency*** - Classic presentation of **G6PD deficiency** triggered by an **oxidant drug** (primaquine) in a high-risk population (African male). - Peripheral blood smear shows characteristic **bite cells** (degmacytes), **blister cells**, and **Heinz bodies** indicating oxidative damage to red blood cells. *Hereditary spherocytosis* - Presents with **spherocytes** on blood smear and is associated with **osmotic fragility**, not drug-induced hemolysis. - Usually has a **positive family history** and **splenomegaly**, lacking the acute oxidant trigger seen here. *Paroxysmal nocturnal hemoglobinuria* - Characterized by **complement-mediated hemolysis** that typically occurs at night or during sleep. - Blood smear shows **normocytic anemia** without the specific oxidative damage morphology seen in G6PD deficiency. *Microangiopathic hemolytic anemia* - Blood smear shows **schistocytes** (fragmented red blood cells) due to mechanical damage in small vessels. - Associated with conditions like **thrombotic thrombocytopenic purpura** or **hemolytic uremic syndrome**, not drug-induced oxidative stress.

Q: A 56-year-old male presents with low back pain, weight loss, fatigue, and recurrent sinus infections. He has no history of trauma. Examination reveals focal tenderness at the T10 level. Laboratory investigations show Hb of 9 g/dl and deranged renal function tests. A skull X-ray was performed. Bone marrow biopsy and serum protein electrophoresis are planned. What is the characteristic abnormality expected on the peripheral blood film?

Rouleaux formation. The clinical presentation of bone pain (low back pain), constitutional symptoms (weight loss, fatigue), recurrent infections, and renal impairment in an elderly male is highly suggestive of **Multiple Myeloma (MM)** [1]. The "CRAB" features (Calcium elevation, Renal insufficiency, Anemia, and Bone lesions) are classic indicators [1]. **1. Why Rouleaux formation is correct:** In Multiple Myeloma, there is a monoclonal proliferation of plasma cells leading to the overproduction of monoclonal (M) proteins (immunoglobulins) [1]. These high levels of paraproteins reduce the zeta potential (negative charge) on the surface of Red Blood Cells (RBCs). This allows RBCs to stack together like a "pile of coins," a phenomenon known as **Rouleaux formation** [2]. This is the characteristic peripheral smear finding in MM and is also responsible for the characteristically high ESR seen in these patients [1]. **2. Analysis of Incorrect Options:** * **Anemia (A):** While the patient has anemia (Hb 9 g/dl), it is a *finding*, not a *characteristic morphological abnormality* on the film. * **Pelger-Huet anomaly (B):** This refers to hyposegmented neutrophils, typically seen in Myelodysplastic Syndromes (MDS) or congenital conditions, not MM. * **Neutrophilia (D):** Patients with MM are often neutropenic due to marrow infiltration or have normal counts; neutrophilia is not a characteristic feature. **Clinical Pearls for NEET-PG:** * **Skull X-ray:** Look for "punched-out" lytic lesions [1]. * **Diagnosis:** Gold standard is Bone Marrow Biopsy (>10% clonal plasma cells) [1]. * **Bence-Jones Proteins:** These are free light chains found in urine; they do not show up on standard dipsticks [1]. * **M-Spike:** Seen on Serum Protein Electrophoresis (SPEP), usually in the Gamma globulin region [1].

Q: In polycythemia vera, which of the following is not elevated?

Erythropoietin. Polycythemia Vera (PV) is a chronic myeloproliferative neoplasm characterized by the autonomous, clonal overproduction of red blood cells. The primary pathology is a mutation in the JAK2 gene (most commonly V617F), which makes hematopoietic stem cells hypersensitive to growth factors, independent of physiological regulation. Why Erythropoietin (EPO) is the correct answer: In PV, the massive increase in red cell mass is "primary," meaning it is not driven by Erythropoietin. Due to the high number of circulating RBCs, the body’s negative feedback mechanism suppresses the production of EPO in the kidneys. Therefore, serum Erythropoietin levels are characteristically low or undetectable in PV [1]. This is a crucial diagnostic criterion to differentiate it from secondary polycythemia (e.g., due to high altitude or smoking), where EPO levels are elevated. Analysis of Incorrect Options: * RBC Count & Hematocrit: These are hallmark findings. PV involves an absolute increase in red cell mass, leading to an elevated RBC count and a high hematocrit (often >52% in men, >48% in women). * Platelet Count: PV is a "panmyelosis," meaning all three myeloid lineages are typically increased. Most patients exhibit thrombocytosis (elevated platelets) and leukocytosis (elevated WBCs). High-Yield Clinical Pearls for NEET-PG: * JAK2 V617F Mutation: Present in >95% of PV cases. * Clinical Feature: Aquagenic pruritus (itching after a warm bath) is a classic symptom. * Complication: Increased risk of both arterial and venous thrombosis due to hyperviscosity. * Spent Phase: PV can progress to myelofibrosis or acute myeloid leukemia (AML).

Q: All are true about splenic marginal zone lymphoma, EXCEPT:

The clinical course is virulent.. **Splenic Marginal Zone Lymphoma (SMZL)** is a rare, low-grade B-cell neoplasm. Understanding its indolent nature is key to answering this question. ### **Explanation of the Correct Answer** **Option D is the correct answer (the false statement)** because SMZL typically follows an **indolent (slow-growing) clinical course**, not a virulent one. Most patients have a median survival exceeding 10 years. Treatment is often conservative ("watch and wait") or involves splenectomy/Rituximab, rather than aggressive chemotherapy. ### **Analysis of Incorrect Options** * **Option A:** Autoimmune manifestations are common in SMZL. Approximately 10–15% of patients present with **autoimmune hemolytic anemia (AIHA)** or **immune thrombocytopenia (ITP)**. * **Option B:** As the name implies, the tumor originates from the **marginal zone B-cells** of the splenic white pulp. These cells eventually infiltrate the red pulp and the peripheral blood. * **Option C:** While peripheral lymphadenopathy is rare in SMZL (a key diagnostic feature), **splenic hilar lymph nodes** are frequently involved as they are the primary drainage site for the spleen. ### **High-Yield NEET-PG Pearls** * **Clinical Presentation:** Massive splenomegaly is the hallmark. Peripheral lymphadenopathy is characteristically absent. * **Morphology:** Look for **"Villous lymphocytes"** (short, polar cytoplasmic projections) in the peripheral blood smear. * **Immunophenotype:** Typically **CD20+, CD5–, CD10–, CD23–, and CD103–**. This helps differentiate it from CLL (CD5+) and Hairy Cell Leukemia (CD103+). * **Association:** There is a strong clinical association with **Hepatitis C Virus (HCV)** infection; treating the virus can sometimes lead to lymphoma regression. * **Genetics:** Often associated with **7q31 deletion**.

Question 1

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

Accepted Answer

Human error

Answer

Anaphylaxis

Answer

Graft-versus-host disease (GVHD)

Answer

Presensitization

Question 2

A 20-year-old carpenter presents with an enlarged and tender lymph node in the axilla due to a wound infection on his left thumb. A lymph node biopsy shows follicular enlargement and hyperemia, with sinuses filled with neutrophils. What is the most likely diagnosis?

Accepted Answer

Suppurative lymphadenitis

Answer

Castleman disease

Answer

Interfollicular hyperplasia

Answer

Sinus histiocytosis

Question 3

A 27-year-old black man develops sudden intravascular hemolysis 2 days after receiving the antimalarial drug primaquine, resulting in a decreased hematocrit, hemoglobinemia, and hemoglobinuria. Examination of the peripheral blood film is given. What is the most likely diagnosis?

Accepted Answer

Glucose-6-phosphate dehydrogenase deficiency

Answer

Hereditary spherocytosis

Answer

Paroxysmal nocturnal hemoglobinuria

Answer

Microangiopathic hemolytic anemia

Question 4

All of the following are true regarding Von Willebrand's disease EXCEPT:

Accepted Answer

Normal Ristocetin test

Answer

Factor VIII C deficiency

Answer

Bleeding time is prolonged

Answer

Defective platelet aggregation

Question 5

A 56-year-old male presents with low back pain, weight loss, fatigue, and recurrent sinus infections. He has no history of trauma. Examination reveals focal tenderness at the T10 level. Laboratory investigations show Hb of 9 g/dl and deranged renal function tests. A skull X-ray was performed. Bone marrow biopsy and serum protein electrophoresis are planned. What is the characteristic abnormality expected on the peripheral blood film?

Accepted Answer

Rouleaux formation

Answer

Anemia

Answer

Pelger-Huet anomaly

Answer

Neutrophilia

Question 6

Which of the following is NOT a cytogenetic abnormality found in myelodysplastic syndromes?

Accepted Answer

Chromosome 20q deletion

Answer

Chromosome 5q deletion

Answer

Monosomy 7

Answer

Trisomy 8

Question 7

Direct Coombs test detects which of the following?

Accepted Answer

Antibodies attached to the RBC surface

Answer

Antibodies in the serum

Answer

Antigens attached to the RBC surface

Answer

Antigens in the serum

Question 8

In polycythemia vera, which of the following is not elevated?

Accepted Answer

Erythropoietin

Answer

Hematocrit

Answer

Platelet count

Answer

RBC count

Question 9

All are true regarding Myelofibrosis EXCEPT?

Accepted Answer

Absence of splenic enlargement

Answer

Tear drop poikilocytes

Answer

Giant abnormal platelets

Answer

Leucoerythroblastic blood picture

Question 10

All are true about splenic marginal zone lymphoma, EXCEPT:

Accepted Answer

The clinical course is virulent.

Answer

Autoimmune anemia or thrombocytopenia is present.

Answer

This tumor of small lymphocytes originates in the marginal zone of the spleen white pulp.

Answer

Splenic hilar nodes are involved.

Hematopathology — MCQs

Hematopathology — MCQs

On this page

Practice by Chapter

Want unlimited practice?