Hematopathology Practice Questions

Q: Palatal edema is significant for which of the following conditions?

Gamma heavy chain disease. **Explanation:** Heavy chain diseases (HCDs) are rare B-cell proliferative disorders characterized by the production of monoclonal immunoglobulin heavy chains without associated light chains [1]. **Gamma Heavy Chain Disease (Franklin’s Disease):** This condition resembles a systemic lymphoma rather than multiple myeloma. A classic, high-yield clinical feature of Franklin’s disease is **palatal edema** (uvular edema). This occurs due to the infiltration of Waldeyer’s ring by malignant lymphoid cells, leading to lymphatic obstruction and swelling of the soft palate and uvula. This is a characteristic physical finding often tested in NEET-PG. **Analysis of Incorrect Options:** * **Alpha Heavy Chain Disease (Seligmann’s Disease):** This is the most common HCD. It primarily involves the gastrointestinal tract (immunoproliferative small intestinal disease - IPSID), presenting with malabsorption, chronic diarrhea, and abdominal pain. * **Mu Heavy Chain Disease:** This is the rarest form and is almost always associated with **Chronic Lymphocytic Leukemia (CLL)**. It typically presents with hepatosplenomegaly but lacks the characteristic palatal edema. * **Beta Heavy Chain Disease:** This does not exist as a clinical entity, as there is no "Beta" immunoglobulin heavy chain (the five types are Alpha, Gamma, Mu, Delta, and Epsilon). **High-Yield Clinical Pearls for NEET-PG:** * **Franklin’s Disease (Gamma):** Think "Palatal Edema" and systemic lymphadenopathy. * **Seligmann’s Disease (Alpha):** Think "Small Intestine" and "Malabsorption." * **Diagnosis:** HCDs are diagnosed via **immunofixation electrophoresis**, which shows a monoclonal band of heavy chains but a total absence of light chains (no Bence-Jones proteinuria). **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. 606-607.

Q: Which of the following tests is used to screen a woman with a family history of thalassemia?

NESTROFT. **Explanation:** The correct answer is **NESTROFT** (Naked Eye Single Tube Red Cell Osmotic Fragility Test). **1. Why NESTROFT is correct:** In resource-limited settings and large-scale population screening (especially for Thalassemia Trait), NESTROFT is the preferred **screening test**. It is based on the principle of osmotic fragility. Thalassemic red cells are microcytic and hypochromic with a high surface-area-to-volume ratio, making them more resistant to hemolysis in hypotonic saline (0.36%) compared to normal cells. If the solution remains turbid (cannot see a black line through the tube), the test is positive, indicating a high probability of Thalassemia Trait. It is cost-effective, rapid, and has high sensitivity. **2. Why other options are incorrect:** * **Hb A2 Levels:** This is the **Gold Standard for diagnosis** of Beta-Thalassemia Trait (values >3.5%). However, it is an expensive and specialized test, making it unsuitable for initial mass screening. * **High Performance Liquid Chromatography (HPLC):** This is the **confirmatory test** used to quantify HbA2, HbF, and detect abnormal hemoglobin variants. It is the "investigation of choice" for definitive diagnosis, not primary screening. * **Peripheral Smear and Reticulocyte Count:** While a smear shows microcytic hypochromic cells and target cells, it is non-specific and cannot differentiate between Iron Deficiency Anemia (IDA) and Thalassemia. **Clinical Pearls for NEET-PG:** * **Mentzer Index:** (MCV/RBC count) 13 suggests IDA. * **Best Screening Test:** NESTROFT. * **Investigation of Choice/Confirmatory Test:** HPLC. * **Target Cells:** Characteristically seen in Thalassemia, Liver disease, and Post-splenectomy.

Q: Which one of the following is the most common immunologic type of multiple myeloma?

IgG, Kappa light chain. **Explanation:** Multiple myeloma is a neoplastic proliferation of plasma cells that secrete a monoclonal (M) protein [1]. The classification of myeloma is based on the type of heavy chain and light chain produced. **1. Why Option A is Correct:** The most common heavy chain produced in multiple myeloma is **IgG** (found in approximately 50–60% of cases), followed by IgA [1]. Regarding light chains, **Kappa (κ)** is more common than Lambda (λ), occurring in a ratio of approximately 2:1. Therefore, **IgG-Kappa** is the most frequent immunologic subtype encountered in clinical practice. **2. Analysis of Incorrect Options:** * **Option B (IgA, Kappa):** This is the second most common heavy chain type (approx. 20–25% of cases) [1]. While common, it is significantly less frequent than the IgG type. * **Option C (IgD, Lambda):** IgD myeloma is rare (less than 2%) [1]. Interestingly, IgD myeloma is a unique exception where **Lambda** light chains are more common than Kappa, but it remains an uncommon subtype overall. * **Option D (IgM type):** IgM production is characteristic of **Waldenström Macroglobulinemia**, not Multiple Myeloma [1]. True IgM myeloma is extremely rare. **High-Yield Clinical Pearls for NEET-PG:** * **Bence-Jones Proteins:** These represent free light chains excreted in the urine [1]. * **M-Spike:** On Serum Protein Electrophoresis (SPEP), the monoclonal protein typically appears as a sharp spike in the **gamma-globulin** region. * **CRAB Criteria:** Remember the classic presentation: **C**alcium elevation, **R**enal insufficiency, **A**nemia, and **B**one lesions (lytic "punched-out" lesions). * **Diagnosis:** Plasma cells >10% on bone marrow biopsy is a key diagnostic criterion. **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. 608-609.

Question 1

A four-year-old boy presented with a two-month history of abdominal pain and fever, a ten-day history of maculopapular rash, and a three-day history of dry cough, dyspnea, and wheezing. On examination, the liver and spleen were enlarged 4 cm and 3 cm below the costal margin, respectively. His hemoglobin was 10.0 g/dL, platelet count was 3.7 x 10^9/L, and total leukocyte count was 70 x 10^9/L, with 80% eosinophils. Bone marrow examination revealed a cellular marrow with 45% blasts and 34% eosinophils and eosinophil precursors. The blasts stained negative for myeloperoxidase and nonspecific esterase and were positive for CD19, CD10, CD22, and CD20. Which one of the following statements is not true about the disease?

Accepted Answer

Inv(16) is often detected in the blasts and the eosinophils.

Answer

Eosinophils are not part of the neoplastic clone.

Answer

t(5;14) rearrangement may be detected in blasts.

Answer

Peripheral blood eosinophilia may normalize with chemotherapy.

Question 2

Which one of the following is a pan T lymphocyte marker?

Accepted Answer

CD3

Answer

CD2

Answer

CD19

Answer

CD25

Question 3

Which of the following is considered a good prognostic feature in Hodgkin's disease?

Accepted Answer

Clinical stage

Answer

Histologic subtype

Answer

Age of the patient

Answer

Number of lymph nodes involved

Question 4

Palatal edema is significant for which of the following conditions?

Accepted Answer

Gamma heavy chain disease

Answer

Alpha heavy chain disease

Answer

Mu heavy chain disease

Answer

Beta heavy chain disease

Question 5

A patient presents with a mass at the duodenojejunal flexure that invades the renal papillae. Histopathology confirms the diagnosis as lymphoma. Which of the following staging classifications is most appropriate based on this information?

Accepted Answer

Ann Arbor Stage IV Extranodal

Answer

Ann Arbor Stage II Extranodal

Answer

Ann Arbor Stage I Extranodal

Answer

Staging cannot be done until bone marrow examination is performed

Question 6

Testing of recipient cells against donor serum is:

Accepted Answer

Minor Crossmatching

Answer

Major Cross Matching

Answer

Direct Coomb's Test

Answer

Rh Group Matching

Question 7

Which of the following tests is used to screen a woman with a family history of thalassemia?

Accepted Answer

NESTROFT

Answer

Hb A2 Levels

Answer

High performance liquid chromatography

Answer

Peripheral smear and reticulocyte count

Question 8

Which one of the following is the most common immunologic type of multiple myeloma?

Accepted Answer

IgG, Kappa light chain

Answer

IgA, Kappa light chain

Answer

IgD, Lambda light chain

Answer

IgM, type

Question 9

Which of the following is associated with an intrinsic defect in the RBC membrane?

Accepted Answer

Hereditary spherocytosis

Answer

Autoimmune hemolytic anemia

Answer

Microangiopathic hemolytic anemia

Answer

Thermal injury anemia

Question 10

Massive transfusions result in which of the following complications?

Accepted Answer

Disseminated intravascular coagulation (DIC), Hypothermia, Hypercalcemia

Answer

Disseminated intravascular coagulation (DIC), Hypothermia, Thrombocytopenia

Answer

Disseminated intravascular coagulation (DIC), Hypercalcemia, Thrombocytopenia

Answer

Hypothermia, Hypercalcemia, Thrombocytopenia

Hematopathology — MCQs

Hematopathology — MCQs

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