Hematopathology Practice Questions

Q: Stored blood, which has been preserved in a blood bank, is deficient in which of the following coagulation factors?

V and VIII. **Explanation:** The correct answer is **V and VIII** because these are known as **labile coagulation factors**. In blood bank storage (typically at 1–6°C in CPDA-1), the activity of most coagulation factors remains relatively stable. However, Factors V and VIII are highly sensitive to temperature and storage time. Their activity begins to decline significantly within 24–48 hours of collection. By the time a unit of whole blood reaches its expiration (35–42 days), these factors are virtually absent. This is why patients requiring these specific factors (e.g., in Hemophilia A or massive transfusion) require **Fresh Frozen Plasma (FFP)** or **Cryoprecipitate**, where these factors are preserved by rapid freezing. **Analysis of Incorrect Options:** * **Option A & B (II and VII):** Factor II (Prothrombin) and Factor VII are **stable factors** [1]. They maintain adequate activity levels throughout the shelf-life of stored blood. * **Option D (IX and X):** These are also stable factors. Along with Factors II and VII, they are Vitamin K-dependent and do not degrade significantly during standard refrigerated storage [2]. **NEET-PG High-Yield Pearls:** 1. **Massive Transfusion Complication:** Transfusing large volumes of stored blood (which lacks V and VIII) can lead to **dilutional coagulopathy**. 2. **Storage Lesion:** This term refers to the biochemical and morphological changes in stored blood, including decreased 2,3-DPG (shifting the oxygen dissociation curve to the left), decreased pH, and increased potassium. 3. **Factor VIII Source:** Cryoprecipitate is the richest source of Factor VIII, Fibrinogen, and von Willebrand Factor (vWF). **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 582-583. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 624-625.

Q: Macrocytic anemia is not seen in which of the following conditions?

Anemia of chronic disease. The classification of anemia is primarily based on the **Mean Corpuscular Volume (MCV)**. Macrocytic anemia is defined by an MCV >100 fL. **Why Anemia of Chronic Disease (ACD) is the correct answer:** ACD is typically a **normocytic, normochromic anemia**. In long-standing cases, it may progress to a **microcytic** pattern, but it is **never macrocytic** [2]. The pathophysiology involves high levels of **Hepcidin** (induced by IL-6), which sequesters iron in macrophages and decreases intestinal iron absorption, mimicking a functional iron deficiency [1]. **Why the other options are incorrect:** * **Vitamin B12 deficiency:** This is a classic cause of **megaloblastic macrocytic anemia** [1]. Lack of B12 impairs DNA synthesis, leading to nuclear-cytoplasmic asynchrony where the nucleus matures slower than the cytoplasm, resulting in large cells. * **Hemolytic Anemia & Post-hemorrhagic Anemia:** Both conditions trigger a robust bone marrow response, leading to **reticulocytosis** (increased young RBCs). Since reticulocytes are larger than mature erythrocytes, a high reticulocyte count falsely elevates the MCV, causing a **non-megaloblastic macrocytic** picture. **High-Yield Clinical Pearls for NEET-PG:** * **Megaloblastic Macrocytosis:** Characterized by hypersegmented neutrophils (≥5 lobes) on peripheral smear. Causes: B12/Folate deficiency, Drugs (Methotrexate, Phenytoin, Zidovudine). * **Non-megaloblastic Macrocytosis:** No hypersegmented neutrophils. Causes: Alcoholism (most common), Liver disease, Hypothyroidism, and Reticulocytosis. * **ACD Hallmark:** Low Serum Iron, **Low TIBC**, and **High/Normal Ferritin** (distinguishes it from Iron Deficiency Anemia where Ferritin is low).

Q: Hemolytic disease of the newborn is commonly due to incompatibility of which antigen?

D antigen in the Rh group. **Explanation:** **Hemolytic Disease of the Newborn (HDN)**, also known as erythroblastosis fetalis, occurs when maternal antibodies cross the placenta and destroy fetal red blood cells. This happens when the mother is sensitized to a fetal blood group antigen that she lacks. **Why Option B is Correct:** The **D antigen** of the Rh blood group system is the most potent immunogen among all human blood group antigens. Rh incompatibility typically occurs when an **Rh-negative mother (d/d)** carries an **Rh-positive fetus (D/d)**. If fetal D-positive cells enter the maternal circulation (usually during delivery), the mother produces anti-D IgG antibodies. In subsequent pregnancies, these IgG antibodies cross the placenta, leading to hemolysis. Despite the use of Rh immunoglobulin (Anti-D prophylaxis), it remains the most common cause of severe HDN. **Why Other Options are Incorrect:** * **Options A & C (C and E antigens):** While other Rh antigens like C, c, E, and e can cause HDN, they are significantly less immunogenic than the D antigen and are rare causes of clinical disease. * **Option D (Lewis antigen):** Lewis antigens (Le^a, Le^b) are not implicated in HDN because they are primarily IgM (which does not cross the placenta) and are not well-developed on fetal red cells at birth. **NEET-PG High-Yield Pearls:** * **Most Common Cause of HDN overall:** ABO incompatibility (usually Mother 'O' and Baby 'A' or 'B'). It is often milder and can occur in the first pregnancy. * **Most Common Cause of SEVERE HDN:** Rh (D) incompatibility. * **Standard Prophylaxis:** 300 mcg of Anti-D is given to Rh-negative mothers at 28 weeks gestation and within 72 hours of delivery of an Rh-positive infant. * **Kleihauer-Betke Test:** Used to quantify the amount of fetal-maternal hemorrhage to determine the required dose of Anti-D.

Q: A 4-year-old boy presents with chronic microcytic anemia and splenomegaly, but no other symptoms. His condition is due to decreased alpha-chain production, leading to the formation of four beta-chain tetramers (HbH). For this patient with a hemoglobin abnormality, what is the most likely diagnosis?

HbH disease. ### Explanation **Correct Answer: B. HbH disease** The clinical presentation and pathophysiology described are classic for **HbH disease**, a subtype of **Alpha-thalassemia**. 1. **Pathophysiology:** Alpha-thalassemia occurs due to the deletion of alpha-globin genes (normally four genes exist). HbH disease specifically results from the **deletion of 3 out of 4 alpha genes (--/-α)**. 2. **Mechanism:** When alpha-chain production is severely decreased, there is a relative excess of beta-chains. These excess beta-chains aggregate to form **tetramers ($\beta_4$)**, known as **Hemoglobin H (HbH)**. 3. **Clinical Features:** HbH has a very high affinity for oxygen, making it ineffective at delivering oxygen to tissues. It is also unstable and precipitates as **Heinz bodies**, leading to chronic hemolytic anemia and splenomegaly. --- ### Why the other options are incorrect: * **A. Beta-thalassemia major:** This is caused by a deficiency in beta-chain production, leading to an excess of alpha-chains (not beta-chains). It typically presents with severe anemia and "chipmunk facies" due to extramedullary hematopoiesis. * **C. Sickle cell disease:** This is a qualitative defect caused by a point mutation (glutamic acid to valine) in the beta-globin chain, forming HbS. It does not involve beta-tetramers. * **D. HbC disease:** This is caused by a mutation where glutamic acid is replaced by lysine. It is characterized by "HbC crystals" on peripheral smear, not HbH tetramers. --- ### NEET-PG High-Yield Pearls: * **Hb Bart’s:** A tetramer of four gamma chains ($\gamma_4$) seen in **Hydrops Fetalis** (deletion of all 4 alpha genes). * **Peripheral Smear:** HbH disease shows a "golf ball appearance" of RBCs when stained with **Supravital stains** (like Brilliant Cresyl Blue) due to precipitated HbH. * **Genetics:** Alpha-thalassemia is usually due to **gene deletions**, whereas Beta-thalassemia is usually due to **point mutations**.

Q: A 72-year-old female presents with complaints of generalized lymphadenopathy and a total WBC count of 80,000. Flow cytometry revealed CD19+, CD20+, CD5+, CD23-. Cyclin D1 is positive. Which of the following genetic abnormalities is expected in this condition?

t(11;14). ### Explanation The clinical presentation and immunophenotype point toward a diagnosis of **Mantle Cell Lymphoma (MCL)**. **1. Why t(11;14) is correct:** The key diagnostic markers in this case are **CD5+**, **CD23-**, and **Cyclin D1 positivity** [1]. While both Chronic Lymphocytic Leukemia (CLL) and MCL are CD5+ B-cell neoplasms, MCL is characteristically CD23 negative and expresses Cyclin D1 [1]. The genetic hallmark of MCL is **t(11;14)(q13;q32)**. This translocation involves the *CCND1* gene (Cyclin D1) on chromosome 11 and the *IGH* gene (Immunoglobulin Heavy chain) on chromosome 14. This leads to the overexpression of Cyclin D1, which promotes cell cycle progression from G1 to S phase. **2. Why the other options are incorrect:** * **t(8;21):** Associated with **Acute Myeloid Leukemia (AML-M2)**; it involves the *RUNX1-RUNX1T1* fusion. * **13q deletion:** The most common cytogenetic abnormality in **CLL/SLL**. CLL is typically CD5+ but, unlike this case, it is **CD23+** and Cyclin D1 negative [1]. * **t(11;18):** Associated with **MALT lymphoma** (Marginal Zone Lymphoma); it involves the *BIRC3-MALT1* fusion. **3. High-Yield Clinical Pearls for NEET-PG:** * **Mantle Cell Lymphoma:** Typically presents in elderly males with lymphadenopathy and often involves the GI tract (Lymphomatous polyposis). * **Immunophenotype Triad:** CD5+, CD23 negative, and FMC7+. * **Cyclin D1:** Overexpression is the "gold standard" for diagnosis; if Cyclin D1 is negative, **SOX11** is used as a highly specific alternative marker [1]. * **CLL vs. MCL:** Always check CD23. **CLL = CD23+**; **MCL = CD23-** [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 610-612.

Question 1

Estimation of the degradation products of the cross-linked fibrin fibers in DIC can best, specifically, and accurately be done by which of the following tests?

Accepted Answer

D-dimer assay

Answer

Fibrinopeptide assay

Answer

Euglobin lysis test

Answer

Coombs test

Question 2

Which of the following statements regarding megaloblastic anemia is true?

Accepted Answer

Serum lactate dehydrogenase (LDH) is increased.

Answer

Megaloblastic precursors are present in the bone marrow.

Answer

Mean corpuscular volume is increased.

Answer

Thrombocytosis occurs.

Question 3

Stored blood, which has been preserved in a blood bank, is deficient in which of the following coagulation factors?

Accepted Answer

V and VIII

Answer

II only

Answer

II and VII

Answer

IX and X

Question 4

Macrocytic anemia is not seen in which of the following conditions?

Accepted Answer

Anemia of chronic disease

Answer

Vitamin B12 deficiency

Answer

Hemolytic anemia

Answer

Post-hemorrhagic anemia

Question 5

Hemolytic disease of the newborn is commonly due to incompatibility of which antigen?

Accepted Answer

D antigen in the Rh group

Answer

C antigen in the Rh group

Answer

E antigen in the Rh group

Answer

Lewis antigen

Question 6

A 62-year-old man develops itchy, scaling, and non-scaling patches and plaques over his chest and back. The rest of the examination is normal, except for lymphadenopathy. Blood film and skin biopsy histology both reveal unusually large monocytoid cells. Which of the following is the most likely diagnosis?

Accepted Answer

Primary cutaneous T-cell lymphoma

Answer

Leukemia

Answer

Visceral B-cell lymphoma

Answer

Viral infection (usually Epstein-Barr)

Question 7

Which of the following conditions is LEAST likely to be accompanied by lymphocytosis in the peripheral blood?

Accepted Answer

Bacterial infection

Answer

Viral infection

Answer

Fungal infection

Answer

Protozoal infection

Question 8

What is the most common cause of congestive splenomegaly?

Accepted Answer

Cirrhosis

Answer

Chronic congestive cardiac failure

Answer

Hepatic vein occlusion

Answer

Stenosis of the splenic vein

Question 9

A 4-year-old boy presents with chronic microcytic anemia and splenomegaly, but no other symptoms. His condition is due to decreased alpha-chain production, leading to the formation of four beta-chain tetramers (HbH). For this patient with a hemoglobin abnormality, what is the most likely diagnosis?

Accepted Answer

HbH disease

Answer

Beta-thalassemia major

Answer

Sickle cell disease

Answer

HbC disease

Question 10

A 72-year-old female presents with complaints of generalized lymphadenopathy and a total WBC count of 80,000. Flow cytometry revealed CD19+, CD20+, CD5+, CD23-. Cyclin D1 is positive. Which of the following genetic abnormalities is expected in this condition?

Accepted Answer

t(11;14)

Answer

t(8;21)

Answer

13q deletion

Answer

t(11;18)

Hematopathology — MCQs

Hematopathology — MCQs

On this page

Practice by Chapter

Want unlimited practice?