Hematopathology Practice Questions

Q: Severe congenital neutropenia syndrome is also known as:

Kostmann syndrome.. **Explanation:** **Severe Congenital Neutropenia (SCN)**, specifically the autosomal recessive form, is known as **Kostmann syndrome**. It is characterized by a profound reduction in the absolute neutrophil count (ANC < 500/µL) from birth. The underlying pathophysiology involves a "maturation arrest" of neutrophil precursors at the promyelocyte/myelocyte stage in the bone marrow, often due to mutations in the *ELANE* or *HAX1* genes. This leads to recurrent, life-threatening bacterial infections in early infancy. **Analysis of Incorrect Options:** * **Chediak-Higashi syndrome:** An autosomal recessive disorder of vesicle trafficking (*LYST* gene mutation). It presents with partial albinism, giant lysosomal granules in leukocytes, and mild neutropenia, but is primarily a defect in phagocyte function rather than a primary maturation arrest. * **Aldrich syndrome (Wiskott-Aldrich):** An X-linked recessive disorder characterized by the triad of thrombocytopenia (small platelets), eczema, and immunodeficiency. It affects T-cell and B-cell function rather than causing severe isolated neutropenia. * **Fanconi syndrome:** This refers to a generalized dysfunction of the proximal renal tubule. (Note: *Fanconi Anemia* is a DNA repair defect leading to pancytopenia, but it is not synonymous with isolated severe congenital neutropenia). **High-Yield Clinical Pearls for NEET-PG:** * **Bone Marrow Finding:** Maturation arrest at the **promyelocyte stage**. * **Complication:** Patients have a high risk of developing **Acute Myeloid Leukemia (AML)** or Myelodysplastic Syndrome (MDS). * **Treatment:** Recombinant human Granulocyte Colony-Stimulating Factor (rhG-CSF) is the mainstay; Hematopoietic Stem Cell Transplant (HSCT) is the definitive cure.

Q: For how long can blood be stored at 4°C?

21 days. ### Explanation The storage life (shelf life) of whole blood is primarily determined by the **anticoagulant-preservative solution** used in the blood bag. **1. Why 21 Days is Correct:** The standard anticoagulant used in basic blood banking is **CPD (Citrate Phosphate Dextrose)** or **ACD (Acid Citrate Dextrose)**. These solutions maintain red cell viability by providing glucose for metabolism and buffering pH. At a storage temperature of **2–6°C**, blood collected in CPD or ACD has a shelf life of **21 days**. By the end of this period, at least 70% of the transfused red cells must remain in the recipient's circulation for 24 hours to meet regulatory standards. **2. Analysis of Incorrect Options:** * **7 and 14 days (Options A & B):** These durations are too short. While metabolic changes (the "storage lesion") begin immediately, the cells remain clinically viable and safe for transfusion well beyond two weeks. * **28 days (Option D):** This is incorrect for standard CPD. However, if **CPDA-1** (Citrate Phosphate Dextrose Adenine) is used, the shelf life extends to **35 days** because adenine helps resynthesize ATP. **3. High-Yield Clinical Pearls for NEET-PG:** * **Storage Temperature:** Always **2–6°C** (never allowed to freeze unless using glycerol). * **CPDA-1:** Extends life to **35 days** (Adenine is the key additive). * **SAGM (Saline Adenine Glucose Mannitol):** An additive solution that extends shelf life to **42 days**. * **Storage Lesion:** During storage, **Potassium increases** (due to leakage from RBCs), while **2,3-DPG and pH decrease**. This shift in 2,3-DPG causes a left shift in the oxygen dissociation curve (increased oxygen affinity).

Q: Anemia of chronic disease is due to which of the following mechanisms?

Decreased utilization of stored iron. **Explanation:** **Anemia of Chronic Disease (ACD)**, also known as Anemia of Inflammation, is primarily driven by the inflammatory cytokine **Interleukin-6 (IL-6)**. IL-6 stimulates the liver to produce **Hepcidin**, the master regulator of iron homeostasis [1]. Hepcidin acts by binding to and degrading **ferroportin** (the iron export channel) on macrophages and enterocytes. This leads to: 1. **Sequestration of iron** within macrophages (increased storage iron/Ferritin) [1]. 2. **Reduced intestinal iron absorption.** 3. **Decreased delivery of iron** to the bone marrow for erythropoiesis [1]. Thus, the correct answer is **decreased utilization of stored iron**, as iron is "trapped" and unavailable for hemoglobin synthesis despite adequate body stores. **Analysis of Incorrect Options:** * **Options A & B (Vitamin B12 and Folate deficiency):** These cause **Megaloblastic Anemia** due to impaired DNA synthesis, characterized by macrocytosis and hypersegmented neutrophils. * **Option D (Chronic blood loss):** This leads to **Iron Deficiency Anemia (IDA)**. Unlike ACD, IDA is characterized by depleted iron stores (low Ferritin) and an increase in Total Iron Binding Capacity (TIBC). **High-Yield NEET-PG Pearls:** * **Hallmark Lab Findings in ACD:** Low Serum Iron, **High/Normal Ferritin**, Low TIBC, and Low Transferrin Saturation. * **Morphology:** Initially Normocytic Normochromic; can become Microcytic Hypochromic in long-standing cases. * **Key Mediator:** Hepcidin (Acute phase reactant) [1]. * **Common Causes:** Chronic infections (TB, Osteomyelitis), Autoimmune diseases (RA, SLE), and Malignancies. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 658-662.

Question 1

Severe congenital neutropenia syndrome is also known as:

Accepted Answer

Kostmann syndrome.

Answer

Chediak-Higashi syndrome.

Answer

Aldrich syndrome.

Answer

Fanconi syndrome.

Question 2

Birbeck granules are characteristically seen in which of the following cell types?

Accepted Answer

Langerhans cells

Answer

Eosinophils

Answer

Reed-Sternberg cells

Answer

Anitschkow cells

Question 3

In pernicious anemia, against which component is an antibody formed?

Accepted Answer

Parietal cell

Answer

G cell

Answer

Stem cell

Answer

All

Question 4

CD59 defect is a marker of which disease?

Accepted Answer

Paroxysmal Nocturnal Hemoglobinuria

Answer

PTEN

Answer

Hereditary spherocytosis

Answer

Cowden syndrome

Question 5

A normal platelet count is typically seen in which of the following conditions?

Accepted Answer

Von Willebrand's disease

Answer

Disseminated intravascular coagulation (DIC)

Answer

Microangiopathic hemolytic anemia

Answer

Splenomegaly

Question 6

A triad of leukoerythroblastosis, tear drop erythrocytes, and large platelets is seen in which of the following conditions?

Accepted Answer

Primary myelofibrosis

Answer

Essential thrombocytosis

Answer

Myelodysplastic syndrome

Answer

Langerhans cell histiocytosis

Question 7

For how long can blood be stored at 4°C?

Accepted Answer

21 days

Answer

7 days

Answer

14 days

Answer

28 days

Question 8

All of the following are causes of fragmented RBCs in peripheral blood EXCEPT:

Accepted Answer

Hemophilia A

Answer

Microangiopathic hemolytic anemia

Answer

Disseminated intravascular coagulation

Answer

Malignant hypertension

Question 9

Anemia of chronic disease is due to which of the following mechanisms?

Accepted Answer

Decreased utilization of stored iron

Answer

Vitamin B12 deficiency

Answer

Folate deficiency

Answer

Chronic blood loss

Question 10

A deficiency of iron can result in anemia. Which type of anemia would occur if iron were deficient?

Accepted Answer

Hypochromic, microcytic anemia

Answer

Megaloblastic anemia

Answer

Hemolytic anemia

Answer

Sickle cell anemia

Hematopathology — MCQs

Hematopathology — MCQs

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