Hematopathology Practice Questions

Q: A patient with Myeloproliferative Neoplasm presents with decreased white cell count and decreased platelets. What is the most likely diagnosis?

Myelofibrosis. **Explanation:** The correct answer is **Myelofibrosis (Primary Myelofibrosis)**. **Why it is correct:** While most Myeloproliferative Neoplasms (MPNs) initially present with an increase in cell counts, Primary Myelofibrosis (PMF) is unique due to its progression into a **"spent phase."** In PMF, neoplastic megakaryocytes release cytokines like TGF-β, which stimulate fibroblasts to deposit collagen in the bone marrow [1]. This progressive **fibrosis** replaces the hematopoietic space, leading to bone marrow failure. Consequently, patients develop **cytopenias** (decreased WBCs and platelets) and extramedullary hematopoiesis (massive splenomegaly) [1]. **Why other options are incorrect:** * **Chronic Myeloid Leukemia (CML):** Characterized by marked leukocytosis (predominantly neutrophils and myelocytes) and often thrombocytosis. Cytopenia is rare unless the patient enters a Blast Crisis. * **Polycythemia Vera (PV):** Defined by an absolute increase in red cell mass, often accompanied by leukocytosis and thrombocytosis (panmyelosis). * **Essential Thrombocytosis (ET):** Primarily presents with a significantly elevated platelet count; WBC and RBC counts are usually normal or slightly increased. **High-Yield Clinical Pearls for NEET-PG:** * **Peripheral Smear:** Look for **Dacrocytes (Tear-drop RBCs)** and a **Leukoerythroblastic picture** (immature WBCs and nucleated RBCs). * **Bone Marrow:** Characterized by a **"Dry Tap"** on aspiration due to extensive fibrosis (Silver stain/Reticulin stain is used for diagnosis). * **Genetics:** Associated with **JAK2 V617F** (approx. 50%), CALR, or MPL mutations. * **Splenomegaly:** PMF causes some of the largest spleens in clinical practice due to compensatory extramedullary hematopoiesis [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 615-616.

Q: Which of the following cytogenetic abnormalities is NOT seen in acute myelodysplastic syndrome?

Monosomy 9. ### Explanation The question asks for the cytogenetic abnormality **not** typically associated with Myelodysplastic Syndromes (MDS). **1. Why Monosomy 9 is the Correct Answer:** Monosomy 9 (loss of an entire chromosome 9) is an extremely rare finding in MDS. While deletions of the long arm of chromosome 9 (**del 9q**) can be seen in AML (often associated with t(8;21)), **Monosomy 9** is not a characteristic or recurrent feature of MDS. In contrast, MDS is defined by specific recurrent chromosomal losses (monosomies or interstitial deletions) and specific gains [1]. **2. Analysis of Incorrect Options:** * **Trisomy 8:** This is the most common **gain** of a chromosome in MDS. It is considered an intermediate-risk cytogenetic abnormality [1]. * **Deletion of 5q (del 5q):** This is one of the most common and characteristic deletions in MDS. The "5q- syndrome" is a distinct clinical entity characterized by macrocytic anemia, thrombocytosis, and erythroid hypoplasia with a favorable prognosis [1]. * **Deletion of 20q (del 20q):** This is a common recurrent deletion in MDS and other myeloproliferative neoplasms. It is generally associated with a good prognosis when it occurs as an isolated abnormality [1]. **3. NEET-PG High-Yield Pearls:** * **Most common cytogenetic abnormalities in MDS:** del(5q), Monosomy 7/del(7q), Trisomy 8, and del(20q) [1]. * **Good Prognosis:** Isolated del(5q), isolated del(20q), and normal karyotype [1]. * **Poor Prognosis:** Monosomy 7 or Complex karyotype (≥3 abnormalities) [1]. * **MDS Hallmark:** Ineffective hematopoiesis leading to peripheral cytopenias despite a hypercellular bone marrow [1]. **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. 622-624.

Q: Low TIBC is seen in which of the following conditions?

Anemia of chronic disease. Explanation: Total Iron Binding Capacity (TIBC) is a functional measurement of the amount of Transferrin available to bind iron. It is generally inversely proportional to body iron stores. 1. Why Anemia of Chronic Disease (ACD) is correct: In ACD, the body produces high levels of Hepcidin (an acute-phase reactant) in response to inflammation. [1] Hepcidin sequesters iron within macrophages and hepatocytes, leading to low serum iron. [2] Simultaneously, the liver decreases the production of Transferrin to "starve" potential pathogens of iron. Since TIBC measures Transferrin levels, a decrease in Transferrin results in a Low TIBC. 2. Why the other options are incorrect: * Iron Deficiency Anemia (IDA): This is the classic "opposite" of ACD. [1] When iron stores are depleted, the liver compensates by increasing Transferrin production to maximize iron transport. Thus, IDA is characterized by a High TIBC. * Fanconi Anemia & Aplastic Anemia: These are bone marrow failure syndromes. They involve a primary defect in stem cells (pancytopenia) rather than a defect in iron metabolism. In these conditions, TIBC is usually normal or decreased (due to iron overload from multiple transfusions), but they are not the classic textbook examples for TIBC differentiation like ACD. [3] High-Yield Clinical Pearls for NEET-PG: * Ferritin: The most sensitive indicator for IDA (Low Ferritin). In ACD, Ferritin is Normal or High (as it is an acute-phase reactant). * Transferrin Saturation: Low in both IDA and ACD. * Soluble Transferrin Receptor (sTfR): Elevated in IDA but Normal in ACD. This is the best test to differentiate the two when they coexist. * Summary Table: * IDA: ↓ Iron, ↑ TIBC, ↓ Ferritin. * ACD: ↓ Iron, ↓ TIBC, ↑ Ferritin. [3]

Q: Which of the following is NOT a B cell marker?

CD 135. The identification of lymphocytes via **Cluster of Differentiation (CD)** markers is a high-yield topic in histology and pathology. B cells express specific surface antigens at various stages of their development [1], which are used clinically for immunophenotyping. **Why CD 135 is the correct answer:** **CD 135** (also known as Flt3) is a cytokine receptor expressed on **multipotent hematopoietic stem cells** and common lymphoid/myeloid progenitors. While it plays a role in the early commitment of cells to the lymphoid lineage, it is not a specific marker for B cells. It is primarily associated with early hematopoiesis and is often overexpressed in **Acute Myeloid Leukemia (AML)**. **Analysis of incorrect options (B cell markers):** * **CD 19:** This is the most specific and reliable pan-B cell marker. It is expressed from the earliest pro-B cell stage until just before terminal differentiation into plasma cells. * **CD 20:** A classic pan-B cell marker expressed on mature B cells. It is the clinical target for the monoclonal antibody **Rituximab**. Like CD 19, it is lost during the transition to plasma cells. * **CD 10:** Also known as **CALLA** (Common Acute Lymphoblastic Leukemia Antigen). It is expressed on pre-B cells and germinal center B cells. It is a crucial marker for diagnosing Childhood Acute Lymphoblastic Leukemia (ALL). **High-Yield Clinical Pearls for NEET-PG:** * **Pan-B cell markers:** CD 19, CD 20, CD 21, CD 22, and CD 79a. * **Plasma Cell markers:** CD 138 (Syndecan-1) and CD 38. (Note: Plasma cells usually lose CD 19 and CD 20). * **T-cell markers:** CD 3 (universal), CD 4 (Helper), CD 8 (Cytotoxic). * **NK cell markers:** CD 16 and CD 56.

Q: A 4-year-old female is being evaluated for the sudden onset of multiple petechiae and bruises. She is found to have a peripheral leukocyte count of 55,000, 86% of which are small, homogeneous cells that have nuclei with immature chromatin. Indistinct nucleoli are also present. Initial tests on these immature cells are as follows: TdT, positive; PAS, positive; acid phosphatase, positive; and myeloperoxidase, negative. Based on these findings, the immature cells most likely originated from?

Lymphoblasts. **Explanation:** The clinical presentation of a 4-year-old with petechiae, bruising, and a high leukocyte count (55,000/µL) with immature cells strongly suggests **Acute Lymphoblastic Leukemia (ALL)**, the most common pediatric malignancy. **Why Lymphoblasts are correct:** The diagnosis is confirmed by the specific cytochemical and immunophenotypic profile: * **TdT (Terminal Deoxynucleotidyl Transferase):** A hallmark marker for immature lymphocytes (both B and T cells). It is absent in myeloid cells. * **PAS (Periodic Acid-Schiff):** Characteristically shows "block-like" positivity in lymphoblasts. * **Acid Phosphatase:** Strong focal positivity in the perinuclear area is highly suggestive of **T-cell ALL**. * **MPO (Myeloperoxidase):** Negative results rule out myeloid lineage. * **Morphology:** Small, homogeneous cells with immature chromatin and indistinct nucleoli are characteristic of the **L1 subtype** (FAB classification), common in childhood ALL. **Why other options are incorrect:** * **Myeloblasts:** Would be **MPO positive** and TdT negative. They typically show Auer rods and more prominent nucleoli. * **Monoblasts:** Characteristically show **Non-specific Esterase (NSE)** positivity and are MPO negative or weakly positive. * **Megakaryoblasts:** Associated with AML-M7; they are usually negative for PAS and TdT but positive for platelet markers like CD41/CD61. **High-Yield Pearls for NEET-PG:** 1. **Most common childhood cancer:** ALL (Peak age: 2–5 years). 2. **Best Prognostic Factor:** Age 1–10 years and WBC count <50,000. 3. **Cytogenetics:** t(12;21) has a good prognosis; t(9;22) or Philadelphia chromosome has a poor prognosis. 4. **Common sites of relapse:** Testes and CNS (due to the blood-brain/blood-testis barrier).

Question 1

A patient with Myeloproliferative Neoplasm presents with decreased white cell count and decreased platelets. What is the most likely diagnosis?

Accepted Answer

Myelofibrosis

Answer

Chronic myeloid leukemia

Answer

Polycythemia vera

Answer

Essential thrombocytosis

Question 2

Richter transformation is the conversion of?

Accepted Answer

CLL into DLBCL

Answer

DLBCL into CLL

Answer

SLL into CLL

Answer

CLL into SLL

Question 3

A thrombotic event is seen in all of the following conditions except?

Accepted Answer

Immune thrombocytopenic purpura (ITP)

Answer

Paroxysmal nocturnal hemoglobinuria (PNH)

Answer

Disseminated intravascular coagulation (DIC)

Answer

Heparin-induced thrombocytopenia (HIT)

Question 4

Which of the following cytogenetic abnormalities is NOT seen in acute myelodysplastic syndrome?

Accepted Answer

Monosomy 9

Answer

Trisomy 8

Answer

Deletion of 20q

Answer

Deletion of 5q

Question 5

Low TIBC is seen in which of the following conditions?

Accepted Answer

Anemia of chronic disease

Answer

Iron deficiency anemia

Answer

Fanconi anemia

Answer

Aplastic anemia

Question 6

Features seen in hemolytic anemia are all, EXCEPT:

Accepted Answer

Tear drop and Burr cells

Answer

Reduced Haptoglobin

Answer

Reticulocytosis

Answer

Hemoglobinuria

Question 7

A patient presents with progressive pallor, hyper-segmented neutrophils, and an MCV > 100 fL. What is the most likely diagnosis?

Accepted Answer

Megaloblastic anemia

Answer

Hereditary spherocytosis

Answer

Dimorphic anemia

Answer

Thalassemia

Question 8

Which of the following is NOT a B cell marker?

Accepted Answer

CD 135

Answer

CD 19

Answer

CD 20

Answer

CD 10

Question 9

A 4-year-old female is being evaluated for the sudden onset of multiple petechiae and bruises. She is found to have a peripheral leukocyte count of 55,000, 86% of which are small, homogeneous cells that have nuclei with immature chromatin. Indistinct nucleoli are also present. Initial tests on these immature cells are as follows: TdT, positive; PAS, positive; acid phosphatase, positive; and myeloperoxidase, negative. Based on these findings, the immature cells most likely originated from?

Accepted Answer

Lymphoblasts

Answer

Monoblasts

Answer

Megakaryoblasts

Answer

Myeloblasts

Question 10

Which of the following statements regarding umbilical cord banking is FALSE?

Accepted Answer

Blood is collected from the umbilical artery.

Answer

It is used for allogeneic stem cell transplant.

Answer

Transplantation can be done in people with different HLA matching.

Answer

Blood is preserved by cryopreservation.

Hematopathology — MCQs

Hematopathology — MCQs

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