Hematopathology Practice Questions

Q: Stored plasma is deficient in which coagulation factors?

Factors 5 and 8. ### Explanation The correct answer is **Factors 5 and 8**. **1. Why Factors 5 and 8 are correct:** Coagulation factors are categorized based on their stability during storage. **Factor V (Proaccelerin)** and **Factor VIII (Anti-hemophilic factor)** are known as **labile factors**. They are highly sensitive to temperature and rapidly lose their procoagulant activity when blood or plasma is stored at 4°C. In stored bank blood, Factor VIII activity drops by about 50% within 24 hours. Therefore, to provide these factors, **Fresh Frozen Plasma (FFP)** or **Cryoprecipitate** (specifically for Factor VIII) must be used instead of stored whole blood or stored plasma. **2. Why the other options are incorrect:** * **Factors 2, 7, 9, and 10:** These are the Vitamin K-dependent factors. They are considered **stable factors** and maintain their activity levels for several weeks in stored plasma. * **Factor 1 (Fibrinogen):** This is also a stable factor and remains functional in stored blood. **3. Clinical Pearls & High-Yield Facts for NEET-PG:** * **Labile Factors:** V and VIII (Think: "5 and 8 keep the blood great, but they don't like to wait"). * **Fresh Frozen Plasma (FFP):** Contains all coagulation factors. It is the treatment of choice for multiple factor deficiencies (e.g., Liver disease, DIC, Warfarin overdose). * **Cryoprecipitate:** Contains Factor VIII, Fibrinogen, von Willebrand Factor (vWF), and Factor XIII. It does *not* contain Factor V. * **Storage Lesion:** This refers to the biochemical and morphological changes in stored blood, which include a decrease in 2,3-DPG (shifting the oxygen dissociation curve to the left), a decrease in pH, and an increase in plasma potassium.

Q: Heavy chain disease with kappa light chains in the urine is known as which of the following?

Mu chain disease. **Explanation:** **Heavy Chain Diseases (HCDs)** are rare B-cell neoplasms characterized by the production of truncated monoclonal heavy chains without associated light chains. However, **Mu chain disease** is the unique exception to this rule. 1. **Why Mu chain disease is correct:** In Mu chain disease (associated with Chronic Lymphocytic Leukemia/CLL), the malignant cells produce incomplete IgM heavy chains. Unlike other HCDs, approximately **50-60% of patients with Mu chain disease also secrete monoclonal kappa light chains**, which appear in the urine as Bence-Jones proteins. This co-secretion is a diagnostic hallmark that distinguishes it from Alpha and Gamma chain diseases. 2. **Analysis of Incorrect Options:** * **Seligman disease (Alpha chain disease):** The most common HCD, typically involving the gastrointestinal tract (IPSID). It produces alpha heavy chains but **never** secretes light chains. * **Franklin disease (Gamma chain disease):** Presents like a systemic lymphoma with lymphadenopathy and palatal edema. It involves gamma heavy chains and **does not** involve light chain secretion. * **Waldenstrom macroglobulinemia:** This is a lymphoplasmacytic lymphoma that produces **complete** IgM antibodies (both heavy and light chains), not just isolated heavy chains. [1] **Clinical Pearls for NEET-PG:** * **Alpha chain disease:** Most common HCD; associated with *Campylobacter jejuni*; presents as malabsorption. * **Gamma chain disease:** Characterized by "uvular edema" due to Waldeyer’s ring involvement. * **Mu chain disease:** Least common HCD; almost always associated with underlying CLL and hepatosplenomegaly; look for **vacuolated plasma cells** in the bone marrow. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 616-617.

Question 1

Bence Jones proteins are:

Accepted Answer

Light chains of IgG

Answer

Heavy chains of IgG

Answer

Present in bone marrow

Answer

Seen in lymphoma

Question 2

A 33-year-old alcoholic patient on anti-tuberculosis treatment (ATT) presents with increased serum iron and increased transferrin saturation. What is the most likely diagnosis?

Accepted Answer

Sideroblastic anemia

Answer

Iron deficiency anemia

Answer

Megaloblastic anemia

Answer

Anemia of chronic disease

Question 3

In Disseminated Intravascular Coagulation (DIC), which of the following is true?

Accepted Answer

All of the above

Answer

Increased clotting time

Answer

Increase in the level of fibrin degradation products

Answer

Bleeding from all mucous membranes

Question 4

A 16-year-old girl presents with bowel obstruction. Laparotomy reveals markedly enlarged para-aortic lymph nodes. Biopsy of the lymph nodes exhibits a diffuse neoplastic infiltrate of small, round lymphocytes with a 'starry sky' appearance on low power. The cytoplasm of some lymphocytes is vacuolated and fat stains are positive. What would you expect the neoplastic cells to demonstrate?

Accepted Answer

t(8;14) translocation

Answer

Positive non-specific esterase stain of the cells

Answer

Positive specific esterase stain of the cells

Answer

Low leukocyte alkaline phosphatase score

Question 5

Which of the following protein defects does NOT cause hereditary spherocytosis?

Accepted Answer

Glycophorin C

Answer

Ankyrin

Answer

Palladin

Answer

Anion transport protein

Question 6

Stored plasma is deficient in which coagulation factors?

Accepted Answer

Factors 5 and 8

Answer

Factors 7 and 8

Answer

Factors 2 and 5

Answer

Factors 7 and 9

Question 7

Sickle cell anemia is inherited as which pattern?

Accepted Answer

Autosomal recessive

Answer

Autosomal dominant

Answer

X-linked recessive

Answer

X-linked dominant

Question 8

In the Philadelphia chromosome, what genetic defect is present?

Accepted Answer

Long arm of chromosome 22

Answer

Short arm of chromosome 22

Answer

Short arm of chromosome 9

Answer

Chromosome 21

Question 9

Heavy chain disease with kappa light chains in the urine is known as which of the following?

Accepted Answer

Mu chain disease

Answer

Seligman disease

Answer

Franklin disease

Answer

Waldenstrom macroglobulinemia

Question 10

Autosplenectomy is seen in which of the following conditions?

Accepted Answer

Sickle cell disease

Answer

Hereditary spherocytosis

Answer

Thalassaemia

Answer

Autoimmune anemia

Hematopathology — MCQs

Hematopathology — MCQs

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