Hematopathology Practice Questions

Q: Which of the following findings is associated with the worst prognosis in Acute Myeloid Leukemia (AML)?

Monosomy 7. In Acute Myeloid Leukemia (AML), cytogenetics is the single most important predictor of treatment response and overall survival [1]. **Explanation of the Correct Answer:** **Monosomy 7 (-7)** or a deletion of the long arm of chromosome 7 (7q-) is categorized under **Adverse (Poor) Risk** cytogenetics [1]. It is frequently associated with complex karyotypes, therapy-related AML (t-AML), and AML arising from myelodysplastic syndrome (MDS) [1]. Patients with Monosomy 7 typically show poor response to standard induction chemotherapy and have a very high risk of relapse, necessitating aggressive management like Allogeneic Stem Cell Transplant. **Analysis of Incorrect Options:** * **A. t(8;21):** This translocation involves the *RUNX1-RUNX1T1* genes. It is a hallmark of AML-M2 and is classified as **Favorable Risk**, associated with high complete remission rates [1]. * **B. Inversion 16 [inv(16)]:** This involves the *CBFB-MYH11* fusion gene. Along with t(8;21), it belongs to the "Core Binding Factor" (CBF) leukemias, which carry a **Favorable prognosis** [1]. * **C. Normal Cytogenetics:** This is classified as **Intermediate Risk**. Prognosis in these patients is further refined by molecular markers (e.g., *NPM1* mutation is favorable, while *FLT3-ITD* is unfavorable) [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Best Prognosis:** t(15;17) [APML], followed by t(8;21) and inv(16). * **Worst Prognosis:** Monosomy 7, Monosomy 5, 17p deletions (p53 mutation), and complex karyotypes (≥3 abnormalities). * **Molecular Markers:** *NPM1* and *CEBPA* mutations improve prognosis in normal cytogenetics, whereas *FLT3-ITD* worsens it [1]. * **Chloroma (Granulocytic Sarcoma):** Most commonly associated with t(8;21). **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. 620-621.

Q: Acute promyelocytic leukaemia (AML - M3) includes which of the following subtypes?

Hyper granular and micro granular type. **Explanation:** Acute Promyelocytic Leukaemia (APL), classified as **AML-M3** in the FAB classification, is characterized by the malignant proliferation of promyelocytes [1]. Morphologically, APL is divided into two distinct subtypes based on the appearance of the cytoplasm: 1. **Hypergranular Type (Typical/Classic):** This is the most common form. The cells contain numerous coarse, azurophilic granules and characteristic **Auer rods**, often found in bundles called **"Faggot cells"** [1]. 2. **Microgranular Type (Hypogranular/M3v):** In this variant, the granules are so small and fine that they are often invisible under a light microscope, giving the cytoplasm a "dust-like" or clear appearance [1]. A key diagnostic feature is the **bilobed or reniform (kidney-shaped) nucleus**, which can sometimes be mistaken for monocytes (AML-M5) [1]. **Analysis of Options:** * **Option A & D:** While "hypogranular" is often used interchangeably with "microgranular" in clinical practice, the formal morphological classification specifically uses the term **Microgranular** to describe the M3 variant. * **Option B:** "Hypersegmented" refers to neutrophils in megaloblastic anemia, not the morphology of promyelocytes in APL. **High-Yield Clinical Pearls for NEET-PG:** * **Cytogenetics:** Strongly associated with **t(15;17)**, involving the *PML-RARA* fusion gene [1]. * **Complication:** High risk of **Disseminated Intravascular Coagulation (DIC)** due to the release of procoagulants from granules [1]. * **Treatment:** Highly responsive to **All-Trans Retinoic Acid (ATRA)** and Arsenic Trioxide, which induce differentiation of the blast cells. * **Immunophenotype:** Typically **CD34 negative** and **HLA-DR negative**, but strongly positive for MPO. **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. 620-622.

Q: A child presents with hypochromic microcytic anemia, with normal levels of free Erythrocyte Protoporphyrin. What is the most likely diagnosis?

Thalassemia. **Explanation:** The key to solving this question lies in understanding the heme synthesis pathway. **Free Erythrocyte Protoporphyrin (FEP)** is the precursor that combines with iron to form heme. If iron is unavailable or its utilization is blocked, FEP accumulates in the red blood cell. **1. Why Thalassemia is correct:** Thalassemia is a quantitative defect in **globin chain synthesis**, not heme synthesis. Since the machinery for producing protoporphyrin and the availability of iron are both normal, FEP levels remain **normal**. This is a classic biochemical marker used to differentiate Thalassemia from Iron Deficiency Anemia (IDA). **2. Why the other options are incorrect:** * **Iron Deficiency Anemia (IDA):** There is insufficient iron to bind with protoporphyrin. Consequently, "unbound" or Free Erythrocyte Protoporphyrin **increases**. * **Lead Toxicity:** Lead inhibits the enzyme *Ferrochelatase*, which prevents the insertion of iron into the protoporphyrin ring. This leads to a significant **increase** in FEP (specifically Zinc Protoporphyrin). * **Anemia of Chronic Disease (ACD):** Iron is trapped within macrophages (high ferritin) and is unavailable for erythropoiesis. Similar to IDA, the lack of available iron leads to **increased** FEP. **High-Yield Clinical Pearls for NEET-PG:** * **Mentzer Index:** (MCV/RBC count) 13 suggests IDA. * **RDW:** Usually normal in Thalassemia trait but increased in IDA. * **Confirmatory Test:** Hb Electrophoresis (showing increased HbA2 > 3.5%) is the gold standard for Beta-Thalassemia trait. * **FEP** is also increased in Sideroblastic anemia (except the X-linked ALAS2 deficiency subtype).

Question 1

Which of the following findings is associated with the worst prognosis in Acute Myeloid Leukemia (AML)?

Accepted Answer

Monosomy 7

Answer

t(8;21) translocation

Answer

Inversion 16

Answer

Normal cytogenetics

Question 2

Which of the following carcinomas is not associated with disseminated intravascular coagulation (DIC)?

Accepted Answer

Breast carcinoma

Answer

Stomach carcinoma

Answer

Pancreatic carcinoma

Answer

Prostate carcinoma

Question 3

A 28-year-old, previously healthy man has noted increasing fatigue for the past 6 months and the formation of bruises after minimal trauma. Over the past 2 days, he has developed a cough. On physical examination, his temperature is 38.9deg C, and he has diffuse rales in both lungs. He has no hepatosplenomegaly and no lymphadenopathy. Laboratory findings include a sputum culture positive for Streptococcus pneumoniae, hemoglobin of 7.2 g/dL, hematocrit of 21.7%, platelet count of 23,400/ mm3, WBC count of 1310/ mm3, prothrombin time of 13 seconds, partial thromboplastin time of 28 seconds, and total bilirubin of 1 mg/dL. The ANA test result is negative. What is the most likely explanation of these findings?

Accepted Answer

Hematopoietic stem cell defect

Answer

Hemolysis of antibody-coated cells

Answer

Increased susceptibility to lysis by complement

Answer

Metastatic adenocarcinoma to bone marrow

Question 4

Mucosa associated lymphoid tumour (MALToma) is most commonly associated with which of the following microorganisms?

Accepted Answer

Helicobacter pylori

Answer

Candida albicans

Answer

Escherichia coli

Answer

Cytomegalovirus

Question 5

Hodgkin's lymphoma is caused by which virus?

Accepted Answer

Epstein-Barr virus (EBV)

Answer

Cytomegalovirus (CMV)

Answer

Human herpesvirus 6 (HHV6)

Answer

Human herpesvirus 8 (HHV8)

Question 6

Acute promyelocytic leukaemia (AML - M3) includes which of the following subtypes?

Accepted Answer

Hyper granular and micro granular type

Answer

Hyper granular and hypo granular type

Answer

Hyper granular and hyper segmented

Answer

Hypo granular and micro granular type

Question 7

Which of the following laboratory tests is affected by platelet count?

Accepted Answer

Bleeding time

Answer

Prothrombin time

Answer

Partial thromboplastin time

Answer

Thrombin time

Question 8

Which of the following is NOT a method of hemoglobin estimation?

Accepted Answer

Wintrobe's method

Answer

Drabkin's method

Answer

Sahli's method

Answer

Spectrophotometric method

Question 9

A child presents with hypochromic microcytic anemia, with normal levels of free Erythrocyte Protoporphyrin. What is the most likely diagnosis?

Accepted Answer

Thalassemia

Answer

Iron deficiency anemia

Answer

Lead toxicity

Answer

Anemia of chronic disease

Question 10

In sickle cell crisis, bone pain is primarily due to which of the following mechanisms?

Accepted Answer

Bone infarction

Answer

Osteoporosis

Answer

Osteomalacia

Answer

Periosteal reaction

Hematopathology — MCQs

Hematopathology — MCQs

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