Leukemias — MCQs
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?
Cancer management in which of the following malignancies has dramatically increased the survival?
What is the most common malignant neoplasm of infancy?
A 4-year-old child presented with a palpable, painless, and slowly increasing abdominal mass in the right flank region, accompanied by episodes of fever and hematuria. Examination revealed hypertension. Following a CT scan of the abdomen, the patient underwent surgical resection of the mass. The gross specimen and histopathological examination are provided. Which of the following drugs is NOT approved for the management of this condition?
WAGR syndrome includes all except?
What is the most common malignancy of the liver in children?
Leukemias Indian Medical PG Practice Questions and MCQs
Question 1: 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 2: 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 3: 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 4: 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 5: 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 6: 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 7: 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 8: A 4-year-old child presented with a palpable, painless, and slowly increasing abdominal mass in the right flank region, accompanied by episodes of fever and hematuria. Examination revealed hypertension. Following a CT scan of the abdomen, the patient underwent surgical resection of the mass. The gross specimen and histopathological examination are provided. Which of the following drugs is NOT approved for the management of this condition?
- A. Doxorubicin
- B. Dactinomycin
- C. Vincristine
- D. Bleomycin (Correct Answer)
Explanation: ***Bleomycin*** - **Bleomycin** is NOT part of standard **Wilms' tumor** chemotherapy protocols (NWTS/COG regimens). - It is primarily used for **germ cell tumors** and **lymphomas**, not for **nephroblastoma**. *Doxorubicin* - **Doxorubicin** is a key component of **intermediate** and **high-risk** Wilms' tumor treatment protocols. - It is used in combination with other agents for **stage III-V** disease or **unfavorable histology**. *Dactinomycin* - **Dactinomycin** is a cornerstone drug in **all risk groups** of Wilms' tumor treatment. - It is part of the **standard two-drug regimen** along with vincristine for **favorable histology** cases. *Vincristine* - **Vincristine** is universally used in **all Wilms' tumor protocols** regardless of stage or histology. - It forms the **backbone** of treatment, often combined with dactinomycin in **low-risk** cases.
Question 9: WAGR syndrome includes all except?
- A. Wilms tumour
- B. Mental Retardation
- C. Anorectal malformation (Correct Answer)
- D. Genital anomalies
Explanation: **Explanation:** WAGR syndrome is a rare genetic contiguous gene deletion syndrome caused by the microdeletion of chromosome **11p13**. This specific region contains the **WT1** (Wilms Tumor 1) gene and the **PAX6** gene, which are responsible for the clinical manifestations of the syndrome. The acronym **WAGR** stands for: * **W – Wilms tumor:** Approximately 45-50% of affected children develop this nephroblastoma. * **A – Aniridia:** The absence of the iris (due to *PAX6* deletion), often the first sign noted at birth. * **G – Genitourinary anomalies:** Including cryptorchidism, hypospadias, or ambiguous genitalia. * **R – Range of developmental delays:** Formerly referred to as Mental Retardation. **Why Option C is correct:** **Anorectal malformations** are not a component of WAGR syndrome. They are more commonly associated with the **VACTERL** association (Vertebral, Anal atresia, Cardiac, Tracheo-Esophageal, Renal, Limb defects) or Currarino syndrome. **Why other options are incorrect:** * **Wilms tumor (A):** A core component; these patients require frequent renal ultrasounds for early screening. * **Mental Retardation (B):** Now termed intellectual disability, it is a classic feature of the syndrome. * **Genital anomalies (D):** These occur due to the involvement of the *WT1* gene, which is essential for normal urogenital development. **High-Yield Clinical Pearls for NEET-PG:** * **Genetics:** Microdeletion of **11p13**. * **WAGRO Syndrome:** If the deletion extends to include the *BDNF* gene, patients also present with **O**besity. * **Denys-Drash Syndrome:** Another *WT1* related disorder characterized by Wilms tumor, pseudohermaphroditism, and early-onset nephrotic syndrome (diffuse mesangial sclerosis). * **Beckwith-Wiedemann Syndrome:** Associated with Wilms tumor but involves chromosome **11p15** (WT2).
Question 10: What is the most common malignancy of the liver in children?
- A. Hepatoblastoma (Correct Answer)
- B. Hepatocellular carcinoma
- C. Lymphoma
- D. None of the above
Explanation: **Explanation:** **Hepatoblastoma** is the most common primary liver malignancy in children, accounting for approximately 50% of all pediatric liver tumors. It typically presents in infants and toddlers, with the majority of cases diagnosed before the age of 3 years. The tumor originates from primitive epithelial cells (blasts) and is strongly associated with prematurity, low birth weight, and genetic syndromes like **Beckwith-Wiedemann Syndrome** and **Familial Adenomatous Polyposis (FAP)**. **Analysis of Options:** * **Hepatocellular Carcinoma (HCC):** While it is the most common primary liver cancer in adults, it is the second most common in children. It usually occurs in older children (10–15 years) and is often associated with underlying chronic liver diseases like Hepatitis B or Tyrosinemia. * **Lymphoma:** While the liver can be involved in systemic lymphoma (secondary involvement), primary hepatic lymphoma is extremely rare in the pediatric population. * **None of the above:** Incorrect, as Hepatoblastoma is the established leading primary malignancy. **High-Yield Clinical Pearls for NEET-PG:** * **Tumor Marker:** Serum **Alpha-fetoprotein (AFP)** is elevated in >90% of cases and is used for both diagnosis and monitoring treatment response. * **Imaging:** Ultrasound is the initial screening tool, but CT/MRI is required for Pre-treatment Extent of Disease (PRETEXT) staging. * **Pathology:** Look for "fetal" or "embryonal" epithelial cells on histology. * **Treatment:** A combination of neoadjuvant chemotherapy and surgical resection. It is a highly vascular tumor; hence, complete resection is the goal.
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