Hematopathology Practice Questions

Q: All of the following are seen in sickle cell anemia EXCEPT:

High hematocrit. **Explanation:** Sickle Cell Anemia (SCA) is a chronic hemolytic anemia caused by a point mutation in the $\beta$-globin gene (Glu $\to$ Val at position 6) [1]. The fundamental pathophysiology involves the polymerization of deoxygenated Hemoglobin S (HbS), leading to red blood cell (RBC) distortion and premature destruction [1]. **Why "High hematocrit" is the correct answer:** In SCA, the continuous destruction of sickled RBCs (extravascular and intravascular hemolysis) leads to a **low hematocrit** and low hemoglobin levels (typically 6–9 g/dL) [2]. A high hematocrit is characteristic of polycythemia, not a hemolytic state. **Analysis of incorrect options:** * **Target cells (Codocytes):** These are commonly seen on peripheral smears in SCA. They occur due to a decrease in hemoglobin volume relative to the cell membrane surface area, often exacerbated by functional asplenia. * **Jaundice:** Chronic hemolysis leads to increased breakdown of heme, resulting in **unconjugated hyperbilirubinemia**. This clinically manifests as icterus (jaundice) and predisposes patients to pigment gallstones. * **Reticulocytosis:** To compensate for the shortened lifespan of RBCs (10–20 days instead of 120), the bone marrow increases erythropoiesis, resulting in an elevated reticulocyte count. **Clinical Pearls for NEET-PG:** * **Peripheral Smear:** Look for Sickle cells (drepanocytes), Target cells, and **Howell-Jolly bodies** (indicating functional asplenia due to repeated splenic infarctions) [2]. * **Aplastic Crisis:** Usually triggered by **Parvovirus B19** infection, characterized by a sudden drop in hemoglobin and a *low* reticulocyte count [2]. * **Diagnosis:** Gold standard is **Hemoglobin Electrophoresis** (HbS >80%, HbF variable, HbA absent) [1]. * **Acute Chest Syndrome:** The leading cause of death in adult SCA patients.

Q: Which of the following conditions is involved when there is excessive bleeding after labor, and laboratory studies demonstrate decreased platelets, prolonged prothrombin time and partial thromboplastin time, and increased fibrin split products?

Disseminated intravascular coagulation. **Explanation:** The clinical presentation of excessive postpartum bleeding combined with the specific laboratory profile—**thrombocytopenia** (decreased platelets), **prolonged PT and PTT**, and **elevated fibrin split products (FSPs/D-dimers)**—is pathognomonic for **Disseminated Intravascular Coagulation (DIC)**. **Why DIC is correct:** DIC is a consumptive coagulopathy. In obstetrics, it is often triggered by the release of tissue factor into the maternal circulation (e.g., in placental abruption, amniotic fluid embolism, or retained dead fetus). This leads to widespread activation of the coagulation cascade, forming microthrombi that consume platelets and clotting factors (prolonging PT/PTT). Simultaneously, the fibrinolytic system is activated to break down these clots, resulting in elevated FSPs and D-dimers. **Why other options are incorrect:** * **Hemophilia A:** An X-linked recessive deficiency of Factor VIII. It typically presents with an isolated prolonged PTT; platelet count, PT, and FSPs remain normal. * **Severe Liver Disease:** While it can cause decreased clotting factors and prolonged PT/PTT, it rarely causes the acute, massive elevation of FSPs seen in the context of labor-related hemorrhage. * **Vitamin K Deficiency:** Leads to deficiency of Factors II, VII, IX, and X. This prolongs PT (and PTT in severe cases) but does not cause thrombocytopenia or elevated FSPs. **High-Yield Clinical Pearls for NEET-PG:** * **Most common cause of DIC in pregnancy:** Placental Abruption. * **Earliest lab indicator of DIC:** Decreased Fibrinogen levels (often <150 mg/dL in pregnancy is critical). * **Management:** Treat the underlying cause (e.g., deliver the fetus/placenta) and replace blood products (FFP, Cryoprecipitate, and Platelets).

Question 1

Severe hereditary spherocytosis is seen due to a defect in which of the following proteins?

Accepted Answer

Spectrin

Answer

Ankyrin

Answer

Band 3

Answer

Band 4.2

Question 2

In hereditary spherocytosis, the defect lies in which component?

Accepted Answer

Membrane cytoskeleton

Answer

Hemoglobin

Answer

Enzyme

Answer

None of the above

Question 3

Hereditary spherocytosis is characterized by all the following features, except:

Accepted Answer

Increased surface area to volume ratio

Answer

Increased osmotic fragility

Answer

Autosomal dominant inheritance

Answer

Direct Coombs test is diagnostic

Question 4

A patient with a bleeding disorder presents with increased bleeding time and normal clotting time. Which of the following conditions is the most likely diagnosis?

Accepted Answer

Idiopathic thrombocytopenic purpura

Answer

Classic hemophilia

Answer

Christmas disease

Answer

Vitamin K deficiency

Question 5

All of the following are seen in sickle cell anemia EXCEPT:

Accepted Answer

High hematocrit

Answer

Target cells

Answer

Jaundice

Answer

Reticulocytosis

Question 6

A child died soon after birth. On examination, there was hepatosplenomegaly and edema all over the body. What is the most probable diagnosis?

Accepted Answer

alpha-thalassemia

Answer

beta-thalassemia

Answer

Hereditary spherocytosis

Answer

ABO incompatibility/sickle cell anemia

Question 7

Which of the following statements about paroxysmal cold hemoglobinuria is NOT true?

Accepted Answer

Chronic autoimmune form responds well to splenectomy

Answer

Results from formation of Donath-Landsteiner antibody

Answer

Attacks are associated with hemoglobinuria

Answer

Can occur secondary to syphilis

Question 8

Which of the following conditions is involved when there is excessive bleeding after labor, and laboratory studies demonstrate decreased platelets, prolonged prothrombin time and partial thromboplastin time, and increased fibrin split products?

Accepted Answer

Disseminated intravascular coagulation

Answer

Hemophilia A

Answer

Severe liver disease

Answer

Vitamin K deficiency

Question 9

All of the following are true about neutrophils in peripheral smear examination, EXCEPT:

Accepted Answer

If neutrophil granules are smaller and stain red, they are called toxic granules.

Answer

Vacuolated neutrophils may be a sign of bacterial sepsis.

Answer

The presence of neutrophils with more than five nuclear lobes suggests megaloblastic anemia.

Answer

Large, misshapen granules may reflect the inherited Chediak-Higashi syndrome.

Question 10

A 42-year-old man presents with a 2-week history of fever, weakness, and bleeding gums. Peripheral smear shows pancytopenia. Bone marrow examination revealed 26% blasts, frequently exhibiting Auer rods and mature myeloid cells. An occasional neutrophil with pseudo Pelger-Huet anomaly was also noted. Which of the following cytochemical stains is most likely to be positive?

Accepted Answer

Myeloperoxidase

Answer

Acid phosphatase

Answer

Nonspecific esterase

Answer

Toluidine blue

Hematopathology — MCQs

Hematopathology — MCQs

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