Leukemias — MCQs
What is the most common cell origin of acute lymphoblastic leukemia (ALL) in children?
A 20-year-old male presented with fatigue, weakness, and jaundice. What is the most likely diagnosis?
All of the following are good prognostic factors for pediatric acute lymphoblastic leukemia, except:
Which is not seen in Tumour lysis Syndrome?
Among the following AML subtypes, non-specific esterase (NSE) staining is typically NEGATIVE in which one?
What is the most common type of acute myeloid leukemia in patients with Down's syndrome?
What is the most common childhood malignancy?
Cancer management in which of the following malignancies has dramatically increased the survival?
What is the most common malignant neoplasm of infancy?
Wilms' tumor is associated with which of the following conditions?
Leukemias Indian Medical PG Practice Questions and MCQs
Question 1: What is the most common cell origin of acute lymphoblastic leukemia (ALL) in children?
- A. B–cell origin (Correct Answer)
- B. T–cell origin
- C. NK cell origin
- D. Myeloid origin
Explanation: ***B-cell origin*** - Approximately 80-85% of **childhood acute lymphoblastic leukemia (ALL)** cases arise from **malignant B-lymphocyte precursors**. [1] - This predominance is due to the high proliferative activity of **B-cell progenitors** during development, making them more susceptible to oncogenic mutations. [1] - B-cell ALL is characterized by expression of markers such as **CD19, CD10, and CD79a**. [1] *T-cell origin* - **T-cell ALL** accounts for approximately 15-20% of childhood ALL cases. [1] - While still significant, it is less common than the B-cell subtype. [1] - More common in adolescent males and may present with mediastinal mass. [1] *NK cell origin* - **Natural killer (NK) cell leukemia** is an extremely rare form of lymphoid malignancy. - NK cells are distinct from B and T lymphocytes, and their leukemias are classified separately. *Myeloid origin* - **Acute myeloid leukemia (AML)** arises from myeloid progenitors, not lymphoid precursors. - ALL is by definition a **lymphoid** malignancy, not myeloid. - This represents a different lineage of hematopoietic differentiation. **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. 594-600.
Question 2: A 20-year-old male presented with fatigue, weakness, and jaundice. What is the most likely diagnosis?
- A. Acute lymphoblastic leukemia
- B. Chronic myeloid leukemia
- C. Chronic lymphocytic leukemia
- D. Acute myeloid leukemia (Correct Answer)
Explanation: ***Acute myeloid leukemia*** - Presents with **fatigue** and **weakness** due to bone marrow infiltration and resultant cytopenias, typical in this age group [1]. - Often shows **myeloblasts** on peripheral blood smear, confirming the diagnosis [2]. *Chronic myeloid leukemia* - Usually occurs in **older adults** and characterized by **elevated white blood cell counts** with a predominance of mature neutrophils. - Symptoms like fatigue may arise, but there are distinct **Philadelphia chromosome** findings and typically a **longer symptom duration**. *Acute lymphoblastic leukemia* - More common in **younger children** and often associated with **lymphadenopathy** and **thrombocytopenia**, rather than fatigue alone. - Characteristically shows **lymphoblasts** in the blood, which are not mentioned in this patient's presentation. *Chronic lymphocytic leukemia* - Typically presents in adults over **50 years** and is characterized by **lymphocytosis** and often asymptomatic in early stages. - Fatigue may occur but lacks the acute presentation and findings seen in **acute leukemias**. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 607-608. [2] 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. 621-622.
Question 3: All of the following are good prognostic factors for pediatric acute lymphoblastic leukemia, except:
- A. Hyperdiploidy
- B. CNS disease at diagnosis (Correct Answer)
- C. t(12;21)
- D. Initial WBC count <50,000/cumm
Explanation: ***CNS disease at diagnosis*** - The presence of **central nervous system (CNS) disease at diagnosis** in pediatric acute lymphoblastic leukemia (ALL) signifies a more aggressive form of the disease. - This involvement indicates a higher risk of relapse and is associated with a **poorer prognosis**, requiring more intensive treatment strategies. - CNS involvement is classified as a **high-risk feature** in ALL risk stratification protocols. *Hyperdiploidy* - **Hyperdiploidy**, specifically a **DNA index > 1.16** (>50 chromosomes), is considered a **favorable prognostic factor** in pediatric ALL. - It is associated with increased sensitivity to chemotherapy and thus a **better treatment outcome**. - High hyperdiploidy accounts for ~25% of pediatric ALL cases and confers excellent prognosis. *Initial WBC count <50,000/cumm* - An **initial WBC count <50,000/cumm** at diagnosis is a well-established **good prognostic factor** in pediatric ALL. - Lower WBC counts indicate lower tumor burden and are associated with **better treatment response and survival**. - WBC ≥50,000/cumm is classified as high-risk, making values below this threshold favorable. *t(12;21)* - The chromosomal translocation **t(12;21)(p13;q22)**, which results in the **ETV6-RUNX1 (TEL-AML1) fusion gene**, is the most common translocation in pediatric ALL (~25% of cases). - This genetic abnormality is indicative of a **favorable prognosis** with high rates of complete remission and a **reduced risk of relapse**. - It is associated with excellent long-term survival rates in pediatric ALL.
Question 4: Which is not seen in Tumour lysis Syndrome?
- A. Hyperkalemia
- B. Hypophosphatemia (Correct Answer)
- C. Hyperuricemia
- D. Hypocalcemia
Explanation: ***Hypophosphatemia*** - **Tumor lysis syndrome (TLS)** is characterized by the rapid breakdown of tumor cells, leading to the release of intracellular components into the bloodstream. - This process typically results in **acute hyperphosphatemia**, not hypophosphatemia, due to the high phosphate content within tumor cells. *Hyperkalemia* - **Hyperkalemia** is a hallmark of TLS because potassium, a major intracellular cation, is released in large quantities as tumor cells lyse. - Excess potassium can lead to potentially life-threatening cardiac arrhythmias. *Hyperuricemia* - **Hyperuricemia** occurs in TLS because nucleic acids (DNA and RNA) released from dying tumor cells are metabolized into purines, which are then converted to uric acid [1]. - High uric acid levels can precipitate in the renal tubules, leading to **acute kidney injury** [1]. *Hypocalcemia* - **Hypocalcemia** develops in TLS secondary to the acute hyperphosphatemia. - The excess phosphate binds with serum calcium to form **calcium-phosphate precipitates**, effectively lowering the concentration of free ionized calcium.
Question 5: Among the following AML subtypes, non-specific esterase (NSE) staining is typically NEGATIVE in which one?
- A. Acute Erythroleukemia (M6)
- B. Acute Promyelocytic Leukemia (M3) (Correct Answer)
- C. Acute Myelomonocytic Leukemia (M4)
- D. Acute Monocytic Leukemia (M5)
Explanation: ***Acute Promyelocytic Leukemia (M3)*** - Non-specific esterase (NSE) is **negative** in Acute Promyelocytic Leukemia (M3) because NSE primarily stains cells of the **monocytic lineage**. - M3 is characterized by abnormal **promyelocytes** with heavy granulation, which are granulocytic precursors without monocytic differentiation. - M3 shows strong positivity for **myeloperoxidase (MPO)** instead, which is the characteristic marker for granulocytic lineage. *Acute Myelomonocytic Leukemia (M4)* - NSE staining is **positive** in M4 because this subtype has both myeloid and **monocytic components**. - The monocytic component (≥20% of non-erythroid cells) stains positively with NSE, which helps differentiate it from pure myeloid leukemias. - NSE positivity (inhibited by sodium fluoride) is a key diagnostic feature alongside myeloperoxidase positivity. *Acute Erythroleukemia (M6)* - NSE is typically **negative** in the predominant erythroid component of M6. - The diagnosis of M6 relies on the presence of ≥50% erythroid precursors (which are PAS positive) and ≥20% myeloblasts among non-erythroid cells. - NSE is not a characteristic marker for erythroleukemia. *Acute Monocytic Leukemia (M5)* - NSE staining is characteristically **strongly positive** in M5, which primarily consists of **monoblasts and promonocytes**. - This strong NSE positivity (inhibited by sodium fluoride) is a defining diagnostic feature demonstrating pure monocytic differentiation. - M5 typically shows weak or negative myeloperoxidase, helping distinguish it from other AML subtypes.
Question 6: What is the most common type of acute myeloid leukemia in patients with Down's syndrome?
- A. Acute megakaryoblastic leukemia M7 (Correct Answer)
- B. Acute myeloid leukemia M1
- C. Acute promyelocytic leukemia M3
- D. Acute myeloid leukemia M2
Explanation: ***Acute megakaryoblastic leukemia M7*** - **Acute megakaryoblastic leukemia (AML M7)** is significantly more common in children with **Down's syndrome (trisomy 21)**, particularly those under 5 years of age. - This association is thought to be due to an increased copy number of certain genes on **chromosome 21** that are involved in hematopoiesis and leukemogenesis. [3] *Acute myeloid leukemia M1* - This subtype, characterized by proliferation of **myeloblasts without maturation**, is not specifically associated with Down's syndrome. [1] - It is a more undifferentiated form of AML. *Acute promyelocytic leukemia M3* - Characterized by the t(15;17) translocation involving the **PML-RARα fusion gene**, resulting in a block in myeloid differentiation at the promyelocyte stage. [2], [4], [5] - This subtype is associated with a specific genetic abnormality and is not preferentially seen in patients with Down's syndrome. *Acute myeloid leukemia M2* - This subtype involves **myeloblasts with maturation** and a characteristic t(8;21) chromosomal translocation. [2] - While it's a common form of AML, it does not show the specific strong association with Down's syndrome that AML M7 does. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 607-608. [2] 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, p. 620. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Genetic Disorders, pp. 170-171. [4] 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. 621-622. [5] 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.
Question 7: What is the most common childhood malignancy?
- A. CLL
- B. AML
- C. ALL (Correct Answer)
- D. CML
Explanation: ***ALL (Acute Lymphoblastic Leukemia)*** - **Acute lymphoblastic leukemia (ALL)** is the **most common childhood malignancy**, accounting for approximately **25-30% of all childhood cancers** and **75-80% of all childhood leukemias**. - It involves the uncontrolled proliferation of **lymphoblasts** in the bone marrow, blood, and other organs. - Peak incidence occurs between **2-5 years of age**. - Presents with features of **bone marrow failure** (anemia, thrombocytopenia, neutropenia), **organomegaly**, and **bone pain**. *AML (Acute Myeloid Leukemia)* - While AML is a significant leukemia, it is the **second most common acute leukemia in children**, accounting for only **15-20% of childhood leukemias**. - More common in adults than children. - Certain subtypes like **acute promyelocytic leukemia (APL)** have unique characteristics and better prognosis with ATRA therapy. *CLL (Chronic Lymphocytic Leukemia)* - **Chronic lymphocytic leukemia (CLL)** is primarily a disease of **older adults** and is **exceedingly rare in children**. - It involves the proliferation of **mature B lymphocytes** and follows an indolent course. - Median age at diagnosis is around 70 years. *CML (Chronic Myeloid Leukemia)* - **Chronic myeloid leukemia (CML)** is uncommon in children, accounting for **less than 5% of childhood leukemias**. - Characterized by the **Philadelphia chromosome (t(9;22))**, resulting in the **BCR-ABL fusion gene**. - Treated with tyrosine kinase inhibitors like imatinib.
Question 8: Cancer management in which of the following malignancies has dramatically increased the survival?
- A. Cholangiocarcinoma
- B. ALL in children (Correct Answer)
- C. Esophagus carcinoma
- D. Glioblastoma multiforme
Explanation: ***ALL in children*** - Significant advancements in chemotherapy regimens, supportive care, and **risk stratification** have led to dramatically improved survival rates, with over 90% cure rates in many cases. - Recognition of distinct **genetic subtypes** and targeted therapies has further refined treatment approaches. *Cholangiocarcinoma* - This is an **aggressive cancer** with a generally poor prognosis, often diagnosed at advanced stages. - While there have been some therapeutic developments, the overall survival rate remains low. *Esophagus carcinoma* - Despite advances in surgery, radiation, and chemotherapy, **esophageal cancer** still carries a high mortality rate. - It is often diagnosed late, and effective systemic treatments for advanced disease are limited. *Glioblastoma multiforme* - This is the most common and most aggressive type of **brain tumor** in adults, with a very poor prognosis. - Despite intensive treatment with surgery, radiation, and chemotherapy, survival rates remain low, and recurrence is common.
Question 9: What is the most common malignant neoplasm of infancy?
- A. Malignant teratoma
- B. Neuroblastoma (Correct Answer)
- C. Wilms' tumor
- D. Hepatoblastoma
Explanation: **Explanation:** **Neuroblastoma** is the most common extracranial solid tumor of childhood and the **most common malignant neoplasm of infancy** (defined as children <1 year of age). It originates from primordial neural crest cells of the sympathetic nervous system, most commonly occurring in the adrenal medulla. Its high incidence in infancy is attributed to its embryonal nature; in fact, many cases are congenital or detected during prenatal screening. **Analysis of Incorrect Options:** * **Malignant Teratoma:** While teratomas are the most common germ cell tumors in neonates (specifically Sacrococcygeal teratoma), the majority are benign at birth. They do not surpass neuroblastoma in overall malignant frequency during infancy. * **Wilms’ Tumor (Nephroblastoma):** This is the most common primary renal tumor in children, but its peak incidence occurs between **2 to 5 years** of age. It is relatively rare in the first year of life. * **Hepatoblastoma:** This is the most common liver malignancy in children, but it is significantly less common overall than neuroblastoma. **High-Yield Clinical Pearls for NEET-PG:** * **Median age of diagnosis:** 19 months (but it remains the #1 malignancy in the <1-year age group). * **Clinical Feature:** Often presents as a hard, irregular abdominal mass that **crosses the midline** (unlike Wilms’ tumor, which usually does not). * **Opsoclonus-Myoclonus Syndrome:** A classic paraneoplastic syndrome associated with neuroblastoma ("dancing eyes, dancing feet"). * **Biomarker:** Elevated urinary catecholamines (VMA and HVA) are found in 90% of cases. * **Prognosis:** Age is a major prognostic factor; infants (<18 months) generally have a much better prognosis, including the possibility of spontaneous regression (Stage 4S).
Question 10: Wilms' tumor is associated with which of the following conditions?
- A. Hemihypertrophy
- B. Aniridia
- C. Hypertension
- D. Bilateral polycystic kidney disease (Correct Answer)
Explanation: **Explanation:** Wilms' tumor (Nephroblastoma) is the most common primary renal malignancy in children. While it is famously associated with several congenital syndromes, the question asks for a condition **not** typically associated with it, or identifies a distractor in a "except" style format. **Note on the Answer Key:** In standard medical literature, **Bilateral Polycystic Kidney Disease (BPKD)** is **not** a known predisposing factor or associated feature of Wilms' tumor. If the question asks which is associated, Options A, B, and C are all classic features. If the provided key marks D as correct, it implies D is the "odd one out" or the condition **not** associated. * **Hemihypertrophy (Option A):** This is a classic association, often seen in **Beckwith-Wiedemann Syndrome** (macroglossia, omphalocele, hemihypertrophy, and Wilms' tumor). * **Aniridia (Option B):** Absence of the iris is a hallmark of **WAGR Syndrome** (Wilms' tumor, Aniridia, Genitourinary anomalies, and intellectual disability/Range of developmental delays). * **Hypertension (Option C):** Approximately 25% of patients with Wilms' tumor present with hypertension due to increased renin production or compression of the renal artery by the tumor mass. * **Bilateral Polycystic Kidney Disease (Option D):** This is a genetic ciliopathy and is not part of the oncogenic pathways (WT1/WT2 mutations) that lead to Wilms' tumor. **High-Yield Clinical Pearls for NEET-PG:** 1. **WAGR Syndrome:** Associated with deletions on chromosome **11p13** (WT1 gene). 2. **Beckwith-Wiedemann Syndrome:** Associated with mutations on chromosome **11p15.5** (WT2 gene). 3. **Denys-Drash Syndrome:** Characterized by the triad of Wilms' tumor, pseudohermaphroditism, and early-onset nephropathy (glomerulosclerosis). 4. **Clinical Presentation:** Most common presentation is an **asymptomatic, firm, smooth abdominal mass** that does not cross the midline (unlike Neuroblastoma).
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