Hematopathology Practice Questions

Q: Which syndrome is associated with Acute Myeloid Leukemia (AML) and Acute Lymphoblastic Leukemia (ALL)?

Turner syndrome. **Explanation:** The correct answer is **Turner Syndrome (45, XO)**. While Turner syndrome is primarily known for physical findings like short stature and webbed neck, it is associated with an increased risk of both **Acute Lymphoblastic Leukemia (ALL)** and **Acute Myeloid Leukemia (AML)**. The underlying mechanism is thought to involve chromosomal instability and the loss of tumor suppressor genes on the X chromosome. **Analysis of Options:** * **Down Syndrome (Trisomy 21):** This is the most common chromosomal disorder associated with leukemia. However, it has a very specific pattern: a 10-20 fold increased risk of ALL and a unique association with **AML-M7 (Acute Megakaryoblastic Leukemia)**, often preceded by Transient Myeloproliferative Disorder (TMD). * **Noonan Syndrome:** This "male version of Turner" is caused by mutations in the RAS-MAPK pathway. It is specifically associated with **Juvenile Myelomonocytic Leukemia (JMML)**, not typically a balanced risk of ALL/AML. * **Klinefelter Syndrome (47, XXY):** This syndrome is primarily associated with an increased risk of **extragonadal germ cell tumors** (specifically mediastinal) and breast cancer, rather than a significant predisposition to childhood leukemias. **High-Yield Clinical Pearls for NEET-PG:** * **Down Syndrome:** Under age 3, AML (M7) is more common; over age 3, ALL is more common. * **Fanconi Anemia:** Highest risk for **AML** and squamous cell carcinomas. * **Bloom Syndrome & Ataxia-Telangiectasia:** High risk for both ALL and AML due to DNA repair defects. * **Li-Fraumeni Syndrome (p53 mutation):** Associated with ALL, soft tissue sarcomas, and adrenocortical carcinoma.

Q: All of the following are seen in multiple myeloma except?

Dystrophic calcification. **Explanation:** Multiple Myeloma (MM) is a plasma cell dyscrasia characterized by the neoplastic proliferation of a single clone of plasma cells producing monoclonal (M) proteins. **Why Dystrophic Calcification is the correct answer (Except):** In Multiple Myeloma, hypercalcemia occurs due to increased osteoclast activity (mediated by RANKL and IL-6), leading to bone resorption [1]. This result in **Metastatic Calcification** (calcium deposition in normal tissues due to high serum calcium levels). **Dystrophic calcification**, conversely, occurs in dead or degenerated tissues despite *normal* serum calcium levels, which is not the mechanism in MM. **Analysis of Incorrect Options:** * **Visual Disturbance:** This occurs due to **Hyperviscosity Syndrome**, caused by high levels of circulating monoclonal immunoglobulins (especially IgA or IgG). This leads to retinal hemorrhages and "sausage-link" segmentation of retinal veins. * **Proteinuria:** Patients exhibit **Bence-Jones proteinuria** (monoclonal light chains). Additionally, MM can lead to AL-amyloidosis or light chain deposition disease, both causing significant protein loss in the urine. * **Bleeding Tendency:** This is multifactorial in MM: (1) M-proteins interfere with clotting factors and platelet aggregation, (2) Thrombocytopenia due to marrow infiltration, and (3) Amyloid deposits weakening vessel walls. **Clinical Pearls for NEET-PG:** * **CRAB Criteria:** **C**alcium (elevated), **R**enal insufficiency, **A**nemia, **B**one lesions (punched-out lytic lesions). * **Diagnosis:** Bone marrow plasma cells ≥10% or biopsy-proven plasmacytoma PLUS one or more myeloma-defining events. * **Blood Film:** **Rouleaux formation** (due to decreased zeta potential of RBCs by M-proteins). * **Urine:** Bence-Jones proteins are **not** detected by standard dipsticks (which detect albumin); they require sulfosalicylic acid test or electrophoresis.

Q: Myelofibrosis leading to a dry tap on bone marrow aspiration is seen with which of the following conditions?

Acute megakaryocytic leukemia. **Explanation:** The correct answer is **Acute megakaryocytic leukemia (AML-M7)**. **Underlying Medical Concept:** A "dry tap" occurs when bone marrow cannot be aspirated despite multiple attempts, usually due to extensive **myelofibrosis** (replacement of marrow space by collagen/fibrous tissue). In Acute Megakaryocytic Leukemia (AML-M7), the malignant megakaryoblasts release potent fibrogenic cytokines, specifically **Transforming George Factor-beta (TGF-β)** and **Platelet-Derived George Factor (PDGF)**. These cytokines stimulate marrow fibroblasts to deposit excessive reticulin and collagen, leading to rapid and severe fibrosis. **Analysis of Options:** * **Acute Megakaryocytic Leukemia (Correct):** It is the classic cause of acute myelofibrosis among the leukemias. It is also notably associated with Down Syndrome in children under the age of 5. * **Burkitt’s Lymphoma:** This is a high-grade B-cell lymphoma characterized by a "starry sky" appearance on histology. While it can involve the marrow, it typically presents with hypercellularity rather than primary fibrosis. * **Acute Erythroblastic Leukemia (AML-M6):** This involves the proliferation of erythroid precursors. While the marrow is hypercellular, it does not typically induce the cytokine-mediated fibrotic response seen in M7. * **Acute Undifferentiated Leukemia:** These are rare leukemias lacking lineage-specific markers. They do not have a specific association with early-onset myelofibrosis. **High-Yield Clinical Pearls for NEET-PG:** * **Common causes of Dry Tap:** Myelofibrosis (Primary or Secondary), Hairy Cell Leukemia, Aplastic Anemia, and Metastatic carcinoma. * **AML-M7 Association:** Strongly associated with **Trisomy 21 (Down Syndrome)**. * **Diagnosis:** When a dry tap occurs, a **Trephine Biopsy** is mandatory to visualize the architecture and confirm fibrosis using Silver (Reticulin) stain.

Question 1

Which syndrome is associated with Acute Myeloid Leukemia (AML) and Acute Lymphoblastic Leukemia (ALL)?

Accepted Answer

Turner syndrome

Answer

Down syndrome

Answer

Noonan syndrome

Answer

Klinefelter syndrome

Question 2

Proliferation and survival of myeloma cells are dependent on which of the following cytokines?

Accepted Answer

IL-6

Answer

IL-1

Answer

IL-2

Answer

IL-5

Question 3

Diagnosis of Hodgkin's disease is based on all of the following, except:

Accepted Answer

Atypical cells in background

Answer

Reed-Sternberg cells

Answer

Reactive cellular background

Answer

CD30 positivity

Question 4

A patient with previously normal hemoglobin suffered sudden massive acute hemorrhage. Which of the following changes is LEAST likely to occur?

Accepted Answer

Low mean corpuscular volume

Answer

High reticulocyte count

Answer

High neutrophil count

Answer

High packed cell volume

Question 5

All of the following are seen in multiple myeloma except?

Accepted Answer

Dystrophic calcification

Answer

Visual disturbance

Answer

Proteinuria

Answer

Bleeding tendency

Question 6

Which X-linked recessive disease in males presents with a clotting defect?

Accepted Answer

Hemophilia A

Answer

Idiopathic Thrombocytopenic Purpura (ITP)

Answer

Von-Willebrand disease

Answer

None of the above

Question 7

Myelofibrosis leading to a dry tap on bone marrow aspiration is seen with which of the following conditions?

Accepted Answer

Acute megakaryocytic leukemia

Answer

Burkitt's lymphoma

Answer

Acute erythroblastic leukemia

Answer

Acute undifferentiated leukemia

Question 8

A 36-year-old patient is undergoing mediastinoscopy with biopsy. During the procedure, several lymph node biopsies are taken. Pathologic examination reveals normal lymphoid tissue replaced by a mixed population of cells including histiocytes, lymphocytes, monocytes, plasma cells, and eosinophils. Interspersed among these cells are infrequent large binucleate cells with prominent eosinophilic nucleoli. Immunophenotyping shows these cells are positive for CD15 and CD30. What is the most likely diagnosis for these CD15 and CD30 positive cells?

Accepted Answer

Reed-Sternberg cells

Answer

Aschoff cells

Answer

Langhans giant cells

Answer

Megakaryocytes

Question 9

Which of the following is NOT a B cell marker?

Accepted Answer

CD-15

Answer

CD-19

Answer

CD-21

Answer

CD-24

Question 10

Target cells are seen in peripheral blood in which of the following conditions?

Accepted Answer

Thalassemia

Answer

Pernicious anemia

Answer

Aplastic anemia

Answer

Sickle cell anemia

Hematopathology — MCQs

Hematopathology — MCQs

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