Hematopathology Practice Questions

Q: Which of the following conditions is associated with decreased total iron-binding capacity?

Anemia of chronic inflammation. **Explanation:** The **Total Iron-Binding Capacity (TIBC)** is a functional measurement of the amount of **Transferrin** available in the blood. In the body, Transferrin and Iron levels usually have an inverse relationship to maintain homeostasis. **Why Anemia of Chronic Inflammation (ACD) is correct:** In chronic inflammation, the liver produces high levels of **Hepcidin** (an acute-phase reactant). Hepcidin degrades ferroportin, trapping iron inside macrophages and hepatocytes. Simultaneously, the body downregulates the production of Transferrin to "starve" potential pathogens of iron. Since TIBC is a surrogate marker for Transferrin, a decrease in Transferrin leads to a **decreased TIBC**. **Analysis of Incorrect Options:** * **Iron Deficiency Anemia (IDA):** This is the classic condition where **TIBC is increased**. The liver compensates for low serum iron by synthesizing more Transferrin to maximize iron transport efficiency. * **Thalassemia:** This is a quantitative defect in globin chain synthesis, not a primary disorder of iron metabolism. Iron studies are typically normal, though TIBC may decrease only if the patient develops secondary iron overload from multiple transfusions. * **Sideroblastic Anemia:** This involves a defect in protoporphyrin synthesis leading to iron overload. Here, Transferrin becomes saturated, leading to a **decreased or normal TIBC**, but it is less characteristic than in ACD. **NEET-PG High-Yield Pearls:** * **Hepcidin** is the "Master Regulator" of iron metabolism; it is increased in ACD and decreased in IDA. * **Ferritin** is an acute-phase reactant; it is **increased** in ACD (iron is trapped) but **decreased** in IDA (iron stores are exhausted). * **Soluble Transferrin Receptor (sTfR) test:** This is the best test to differentiate IDA (increased sTfR) from ACD (normal sTfR).

Q: What is the commonest mode of inheritance of Von Willebrand's disease?

Autosomal dominant. **Explanation:** **1. Why Autosomal Dominant is Correct:** Von Willebrand Disease (vWD) is the most common inherited bleeding disorder worldwide. It is primarily caused by quantitative or qualitative defects in Von Willebrand Factor (vWF) [3]. The gene for vWF is located on **Chromosome 12**. The vast majority of clinical cases (approximately 70-80%) fall under **Type 1** (quantitative deficiency) and **Type 2** (qualitative defect), both of which are inherited in an **Autosomal Dominant** fashion. Because it is autosomal, it affects males and females equally, distinguishing it from Hemophilia. **2. Why the Other Options are Incorrect:** * **Codominant:** While some blood group systems (like ABO) exhibit codominance, vWD does not follow this pattern. * **Autosomal Recessive:** This is the mode of inheritance for **Type 3 vWD** (the most severe form with near-total absence of vWF) and certain rare subtypes of Type 2 (like 2N). However, these represent a very small percentage of total cases. * **X-linked Recessive:** This is the classic inheritance pattern for **Hemophilia A (Factor VIII deficiency) and Hemophilia B (Factor IX deficiency)**. vWD is specifically known for being the "autosomal" alternative to Hemophilia [2]. **3. NEET-PG High-Yield Pearls:** * **Most Common Type:** Type 1 (Autosomal Dominant). * **Clinical Presentation:** Mucocutaneous bleeding (epistaxis, menorrhagia, gingival bleeding) [4]. * **Lab Findings:** Prolonged Bleeding Time (BT) and often a prolonged aPTT (since vWF stabilizes Factor VIII). Platelet count is usually normal (except in Type 2B). * **Screening Test:** Ristocetin Cofactor Activity (decreased) [3]. * **Treatment of Choice:** Desmopressin (DDAVP) for Type 1 [1]; Factor VIII concentrates containing vWF for severe cases.

Q: Blood platelets in stored blood do not remain functional after what duration?

24 hours. **Explanation:** The functionality of platelets in stored blood is highly sensitive to temperature and storage conditions. When whole blood is collected and stored under standard refrigeration (1°C to 6°C), platelets undergo a rapid loss of viability and hemostatic activity. **Why 24 hours is correct:** Platelets are metabolically active cells that require specific conditions to maintain their discoid shape and aggregative function. In refrigerated stored blood, platelets lose their ability to form a primary hemostatic plug within **24 hours**. After this period, while the cells may still be physically present, they are functionally inert. For effective platelet transfusion, "Platelet Concentrates" must be stored at room temperature (20°C–24°C) with continuous agitation to remain viable for up to 5 days. **Analysis of Incorrect Options:** * **A (12 hours):** While some degradation begins immediately, a significant portion of platelets remains functional up to the 24-hour mark. * **C & D (48 and 72 hours):** By this time, refrigerated platelets have undergone structural changes (becoming spherical) and irreversible metabolic exhaustion, making them clinically useless for stopping a bleed. **High-Yield Clinical Pearls for NEET-PG:** * **Storage Lesion:** This refers to the loss of viability and function of blood components during storage. For platelets, the "refrigeration lesion" is the primary cause of dysfunction. * **Massive Transfusion:** Patients receiving large volumes of stored blood (older than 24 hours) often develop **dilutional thrombocytopenia**, necessitating the administration of fresh frozen plasma (FFP) and separate platelet concentrates. * **Factor Stability:** While platelets fail at 24 hours, Labile Coagulation Factors (Factor V and VIII) also decrease significantly in stored blood, though at a slightly slower rate than platelet dysfunction.

Q: Which subtype of Acute Myeloid Leukemia (AML) is characterized by gum infiltration and hepatosplenomegaly?

M4 subtype. **Explanation:** The correct answer is **M4 subtype (Acute Myelomonocytic Leukemia)**. In the FAB (French-American-British) classification of AML, subtypes with a **monocytic component** (M4 and M5) are uniquely associated with **extramedullary involvement**. This occurs because monoblasts and monocytes have a high propensity to migrate out of the bone marrow and infiltrate tissues [1]. Clinical manifestations of this tissue infiltration include **gum hypertrophy (gingival hyperplasia)**, skin involvement (leukemia cutis), and organomegaly (hepatosplenomegaly). **Analysis of Options:** * **A. Acute Lymphoblastic Leukemia (ALL):** While ALL commonly presents with hepatosplenomegaly and lymphadenopathy (especially in children), it is not typically associated with gum infiltration. * **B. M3 Subtype (Acute Promyelocytic Leukemia):** Characterized by the t(15;17) translocation and a high risk of **DIC (Disseminated Intravascular Coagulation)** due to the release of procoagulants from Auer rods [1]. It does not typically cause gum infiltration. * **C. M2 Subtype (AML with Maturation):** This is the most common subtype of AML. It is often associated with the t(8;21) translocation and the presence of **chloromas** (granulocytic sarcomas), but not specifically gum hypertrophy [1]. **High-Yield Clinical Pearls for NEET-PG:** * **M4 (Myelomonocytic):** Positive for both Myeloperoxidase (MPO) and Non-Specific Esterase (NSE). * **M5 (Monocytic):** Strongest association with gum hypertrophy; predominantly NSE positive. * **M3 (APL):** Associated with **Auer rods in faggot cells** and treated with ATRA (All-Trans Retinoic Acid). * **M0/M1/M2:** Primarily MPO positive. **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, p. 620.

Question 1

Which of the following statements is FALSE regarding the cytochemistry of Acute Myeloid Leukemia (AML)?

Accepted Answer

Periodic Acid-Schiff (PAS) stain is negative in erythroleukemia.

Answer

Myeloperoxidase and Sudan Black stains are positive.

Answer

Non-specific esterase is positive in M4 and M5 subtypes.

Answer

All the above statements are false.

Question 2

Which of the following human malignancies is highly malignant, rapidly progressive, and where a delay in treatment of even 1-2 days can lead to death?

Accepted Answer

Burkitt's lymphoma

Answer

Hodgkin's lymphoma

Answer

Neuroblastoma

Answer

Mycosis fungoides

Question 3

Which of the following conditions is associated with decreased total iron-binding capacity?

Accepted Answer

Anemia of chronic inflammation

Answer

Iron deficiency

Answer

Thalassemia

Answer

Sideroblastic anemia

Question 4

Richter's transformation is associated with which of the following conditions?

Accepted Answer

Chronic Lymphocytic Leukemia (CLL)

Answer

Chronic Myeloid Leukemia (CML)

Answer

Acute Myeloid Leukemia (AML)

Answer

Acute Lymphoblastic Leukemia (ALL)

Question 5

What is the commonest mode of inheritance of Von Willebrand's disease?

Accepted Answer

Autosomal dominant

Answer

Codominant

Answer

Autosomal recessive

Answer

X-linked recessive

Question 6

Incompatible blood transfusion leads to all except?

Accepted Answer

Increased plasma prothrombin

Answer

Increased plasma bilirubin

Answer

Jaundice

Answer

Renal failure

Question 7

Which of the following statements about Sickle cell anemia is INCORRECT?

Accepted Answer

It is caused by the deletion of a gene.

Answer

It is commonly seen in people of African descent.

Answer

Red blood cell size is typically altered.

Answer

There is a substitution of valine for glutamic acid in the beta-globin chain.

Question 8

Blood platelets in stored blood do not remain functional after what duration?

Accepted Answer

24 hours

Answer

12 hours

Answer

48 hours

Answer

72 hours

Question 9

Heterozygous sickle cell anemia provides protection against which of the following?

Accepted Answer

Malaria

Answer

G6PD deficiency

Answer

Thalassemia

Answer

Dengue fever

Question 10

Which subtype of Acute Myeloid Leukemia (AML) is characterized by gum infiltration and hepatosplenomegaly?

Accepted Answer

M4 subtype

Answer

Acute lymphoblastic leukemia (ALL)

Answer

M3 subtype

Answer

M2 subtype

Hematopathology — MCQs

Hematopathology — MCQs

On this page

Practice by Chapter

Want unlimited practice?