Pediatric Hematology and Oncology MCQs

Pediatrics · 47 free questions with answers & explanations.

1. A 3-year-old child presents with pallor, jaundice, and splenomegaly. Blood film shows target cells, microcytes, and nucleated RBCs. Hb is 5.8 g/dL. HbA2 is 5.5% on HPLC. The father has HbA2 of 4.2%. What is the DIAGNOSIS in the child?
2. A 5-year-old boy presents with pallor, bone pain, hepatosplenomegaly, and lymphadenopathy. CBC shows WBC 80,000/mm3 with 85% blasts. Blast morphology shows lymphoblasts with TdT positive, CD10+, CD19+ markers. What is the MOST common cytogenetic abnormality associated with the BEST prognosis in this condition?
3. A 4-year-old child is diagnosed with pre-B ALL (CD19+, CD10+, TdT+). Bone marrow shows 85% blasts. Cytogenetics reveals t(12;21)(p13;q22) translocation. This translocation, which creates ETV6-RUNX1 fusion, is associated with which PROGNOSTIC category?
4. A 5-year-old presents with progressive abdominal mass, hypertension, hematuria, and aniridia. Ultrasound confirms a large intrarenal mass. Genetic analysis is most likely to reveal mutation in which gene, and which syndrome is this child MOST at risk for?
5. A 3-year-old child presents with easy bruising, pallor, and hepatosplenomegaly. Complete blood count shows: Hb 7 g/dL, WBC 85,000/mm³ (predominantly blasts), platelets 40,000/mm³. Bone marrow biopsy confirms acute lymphoblastic leukemia. Flow cytometry shows CD10+, CD19+, CD34+, TdT+. Which chromosomal abnormality in pediatric ALL confers the BEST prognosis?
6. A 4-year-old child presents with intermittent abdominal pain and a large, smooth, non-tender right abdominal mass that does not cross the midline. Urinalysis shows microscopic hematuria. Ultrasound shows a solid intrarenal mass. Which of the following staging criteria in Wilms tumor (nephroblastoma) would indicate Stage III disease according to the COG/NWTSG staging system?
7. A 3-year-old child presents with pallor, splenomegaly, and recurrent vaso-occlusive crises. Haemoglobin electrophoresis shows predominantly HbS with a small amount of HbF and no HbA. Parents are told about hydroxyurea therapy. The primary mechanism of benefit of hydroxyurea in sickle cell disease is:
8. An 18-month-old child has a large abdominal mass that does not cross the midline. Ultrasound shows a solid intrarenal mass with cystic areas. CT reveals involvement of the renal vein but no distant metastases. The most likely diagnosis and initial treatment is:
9. A 5-year-old child is diagnosed with B-cell precursor ALL. Cytogenetics reveal t(9;22) (Philadelphia chromosome). This translocation specifically affects prognosis because:
10. A 4-year-old boy presents with a 2-week history of pallor, fatigue, and easy bruising. CBC shows WBC 85,000/µL with predominantly blast cells (90% blasts on smear), Hb 6.5 g/dL, and platelets 18,000/µL. Bone marrow biopsy confirms ALL. Immunophenotyping shows CD10+, CD19+, TdT+, CD34+. Which cytogenetic finding, if present, would indicate the BEST prognosis?
11. A 3-year-old boy presents with an abdominal mass that crosses the midline, associated with fever and weight loss. Ultrasound shows a large heterogeneous retroperitoneal mass. AFP is markedly elevated. LDH is elevated. What is the MOST likely diagnosis?
12. A 4-year-old boy presents with pallor, petechiae, and a 2-week history of bone pain. CBC shows WBC 65,000/μL with 80% blasts, hemoglobin 6.8 g/dL, and platelets 18,000/μL. Bone marrow confirms B-cell ALL. Cytogenetics shows ETV6-RUNX1 (t(12;21)) translocation. Which statement about this cytogenetic finding is MOST accurate regarding prognosis?
13. A 2-year-old child has an abdominal mass discovered by the parents during bathing. It does not cross the midline, is firm and smooth. Urinalysis shows microscopic hematuria. CT abdomen shows a heterogeneous intrarenal mass with claw sign. Which tumor suppressor gene on chromosome 11 is most commonly mutated in sporadic cases of this tumor?
14. A 4-year-old presents with pallor, petechiae, and hepatosplenomegaly. CBC shows Hb 6 g/dL, WBC 65,000/µL with 80% blasts, platelets 18,000/µL. Blast immunophenotype shows CD10+, CD19+, TdT+, and CD34+. Cytogenetics reveals t(12;21)(p13;q22) translocation [ETV6-RUNX1]. What is the prognostic implication of this finding?
15. A 2-year-old boy presents with a rapidly enlarging right-sided abdominal mass that does not cross the midline. He has fever and haematuria. Ultrasound shows an intrarenal solid mass with calcifications. Urinary VMA and HVA levels are normal. The MOST likely diagnosis and its embryological origin are:
16. A 4-year-old girl presents with progressive pallor, low-grade fever, and petechiae. CBC shows Hb 6.2 g/dL, WBC 2.1 × 10^9/L, platelets 18 × 10^9/L. Bone marrow biopsy shows hypocellular marrow with replacement by fat cells and reticulin. Fanconi anemia workup including chromosomal breakage test with diepoxybutane (DEB) is positive, showing increased chromosomal breaks. She has bilateral absent thumbs. What is the MOST appropriate curative treatment?
17. A 2-year-old boy has an abdominal mass detected incidentally. CT scan shows a large left renal mass with both solid and cystic components, contained within the renal capsule. No metastases are identified. Biopsy confirms Wilms tumor (nephroblastoma). According to the Children's Oncology Group (COG) staging system, this is Stage I. What is the treatment protocol?
18. A 2-year-old child presents with pallor, recurrent infections, and petechiae. CBC: Hb 6 g/dL, WBC 120,000/μL with 85% blasts, platelets 18,000/μL. Flow cytometry shows CD10+, CD19+, TdT+, CD34+ blasts. The specific cytogenetic finding associated with the BEST prognosis in this type of ALL is:
19. A 6-month-old boy is found to have an adrenal mass on abdominal ultrasound during evaluation of a palpable abdominal mass. Urine catecholamine metabolites (VMA and HVA) are elevated. CT shows heterogeneous suprarenal mass with calcification, crossing the midline. The bone marrow aspirate shows small round blue cells arranged in pseudo-rosettes. This tumor's cell of origin and the MOST COMMON site for stage 4S (special) neuroblastoma metastasis is:
20. A 5-year-old child with sickle cell anemia (HbSS) develops a sudden onset of fever (38.9°C), pallor, and splenomegaly (spleen acutely increased from baseline). Hb drops from 9 g/dL to 4.5 g/dL within 24 hours. Reticulocyte count is 0.2%. The MOST likely diagnosis is:
21. A 4-year-old boy presents with acute onset pallor, jaundice, dark urine, and hemoglobinuria after eating fava beans. CBC shows Hb 6.2 g/dL, reticulocyte count 12%, peripheral smear shows 'bite cells' and Heinz bodies on crystal violet staining. What is the MOST LIKELY explanation for why females are rarely as severely affected as hemizygous males in this condition?
22. A 3-year-old presents with a 3-week history of pallor, fever, lymphadenopathy, and hepatosplenomegaly. Bone marrow biopsy shows >25% lymphoblasts. Immunophenotyping: TdT positive, CD10 positive, CD19 positive, CD34 positive. Cytogenetics: t(12;21). Which statement CORRECTLY characterizes the prognosis of this cytogenetic finding?
23. A 7-year-old child with beta-thalassemia major on chronic transfusion therapy (every 3–4 weeks for 5 years) develops increasing skin pigmentation, growth failure, and elevated serum ferritin of 5500 ng/mL. Liver biopsy shows severe hepatic iron deposition. Which chelation strategy is MOST appropriate at this point?
24. A 3-year-old boy presents with pallor, petechiae, and hepatosplenomegaly. CBC shows WBC 95,000/mm³ with 85% blasts, Hb 6.2 g/dL, platelets 18,000/mm³. Bone marrow shows lymphoblasts with PAS-positive cytoplasm. Immunophenotyping: CD10+, CD19+, CD20−, TdT+. Which cytogenetic finding carries the BEST prognosis in this type of leukemia?
25. A 2-year-old boy is found to have a right-sided abdominal mass. CT scan shows a large heterogeneous intrarenal mass with focal areas of calcification. No lymph node involvement or metastases. This is stage I. Which treatment approach is used in India per NWTS/COG protocol?
26. A 4-year-old child has pallor, easy bruising, lymphadenopathy, hepatosplenomegaly, and bone pain. Peripheral smear shows lymphoblasts. CSF examination shows 15 lymphoblasts/mm³. Immunophenotyping reveals CD10+, CD19+, TdT+ blasts. Which prognosis category does the CSF finding indicate?
27. A 2-year-old boy has a palpable non-tender abdominal mass not crossing the midline. Urinalysis shows haematuria. CT abdomen confirms an intrarenal mass with a pseudocapsule that distorts the collecting system. Which tumour marker/molecular test is MOST useful in prognosis?
28. A 4-year-old boy has a 3-week history of pallor, bone pain, fever, and hepatosplenomegaly. CBC: Hb 6.2 g/dL, WBC 85,000/mm3 with 80% lymphoblasts, platelets 28,000/mm3. Immunophenotyping shows CD19+, CD10+, TdT+, and cytoplasmic Ig negative. This immunophenotype is most consistent with:
29. A 2-year-old boy is found to have a large abdominal mass. Abdominal CT shows a heterogeneous solid intrarenal mass with displacement of adjacent structures. Urinalysis shows microscopic hematuria. The most likely diagnosis and the chromosomal region specifically associated with its familial/bilateral form is:
30. A 4-year-old boy presents with pallor, petechiae, hepatosplenomegaly, and lymphadenopathy. CBC shows WBC 48,000/μL with 85% lymphoblasts, Hb 7.2 g/dL, platelets 22,000. Bone marrow confirms ALL. The cytogenetic finding that confers the BEST prognosis in childhood ALL is:
31. A 2-year-old child has an abdominal mass that crosses the midline. Ultrasound and CT show a large retroperitoneal mass with calcifications. Urine VMA and HVA are markedly elevated. This is neuroblastoma. The international staging system feature that most strongly predicts prognosis is:
32. A 4-year-old child is found to have an abdominal mass. CT scan shows a large heterogeneous renal mass with areas of necrosis and calcification. The contralateral kidney is normal. Chest CT shows no metastases. This is stage I disease. The treatment regimen is:
33. A 2-year-old presents with proptosis, orbital ecchymosis ('raccoon eyes'), a palpable abdominal mass and bone pain. Urine vanillylmandelic acid (VMA) is markedly elevated. The diagnosis is:
34. A 4-year-old child presents with abdominal mass, haematuria, and hypertension. CT scan shows a large unilateral intrarenal mass with nephroblastomatosis. FISH analysis shows loss of heterozygosity at 11p13 (WT1 gene deletion). What is the most likely diagnosis and the standard initial treatment in India (SIOP protocol)?
35. A 5-year-old child presents with pallor, petechiae, fever, and bone pain. CBC shows WBC 85,000/mm³ with 90% blasts, Hb 6 g/dL, platelets 18,000/mm³. Immunophenotyping shows CD19+, CD10+, TdT+. Cytogenetics reveals t(12;21)(p13;q22) (ETV6-RUNX1 fusion). This translocation is associated with which prognosis?
36. A 4-year-old with ALL (B-cell precursor) in complete remission is on maintenance chemotherapy. He develops fever 39°C, and absolute neutrophil count (ANC) is 300/µL. Blood culture grows Pseudomonas aeruginosa 24 hours later. What is the MOST important empirical antibiotic decision in febrile neutropenia in this high-risk child?
37. A 6-year-old has been diagnosed with Wilms tumor (nephroblastoma) involving the right kidney with no metastases (Stage I). Histopathology shows favorable histology. According to COG/SIOP staging, which is the preferred initial management approach in North America (COG protocol) vs. Europe (SIOP protocol)?
38. A 4-year-old girl presents with a 3-week history of pallor, progressive fatigue, and multiple ecchymoses. CBC shows: Hb 5.2 g/dL, WBC 85,000/mm³ (90% blasts), platelets 18,000/mm³. Bone marrow examination reveals >80% lymphoblasts; immunophenotyping shows CD10+, CD19+, CD22+, terminal deoxynucleotidyl transferase (TdT)+, CD34+. Cytogenetics shows t(12;21)(p13;q22). What is the prognostic implication of this cytogenetic finding?
39. A 2-year-old boy is incidentally found to have an abdominal mass on physical examination during a routine check. Ultrasound shows a large, well-circumscribed, intrarenal mass. CT confirms an 8 cm heterogeneous intrarenal mass with enhancing components. The contralateral kidney is normal. Which histological finding in this tumor is associated with worst prognosis?
40. A 4-year-old girl presents with pallor, hepatosplenomegaly, and lymphadenopathy. CBC shows WBC 48,000/mm³ with 85% blasts, Hb 7.2 g/dL, platelets 38,000/mm³. Bone marrow biopsy confirms ALL. Which immunophenotype of ALL carries the BEST prognosis?
41. A 3-year-old child is found to have an abdominal mass crossing the midline on examination. Ultrasound shows a large renal mass with calcifications. Urine VMA/HVA ratio is 3:1 (HVA predominant). The MOST likely diagnosis is:
42. A 5-year-old boy with known beta-thalassemia major on regular blood transfusions develops increasing serum ferritin (>2500 ng/mL). The first-line iron chelation agent for this child is:
43. A 3-year-old child from a low socioeconomic background is brought with pallor, irritability, and pica (eating mud). He is exclusively fed cow's milk with minimal dietary diversification. Hemoglobin is 7.2 g/dL. Peripheral smear shows microcytic hypochromic red cells with anisocytosis and poikilocytosis. Serum ferritin is 4 ng/mL. What is the first-line treatment?
44. An 8-year-old boy presents with a 2-week history of fever, fatigue, bone pain, and easy bruising. Examination reveals pallor, petechiae, and non-tender lymphadenopathy with splenomegaly. CBC shows Hb 6.5 g/dL, WBC 60,000/mm3, platelets 18,000/mm3. Peripheral smear shows numerous blasts. What is the most likely diagnosis, and which immunophenotype carries the best prognosis?
45. A 2-year-old child presents with a large abdominal mass that does not cross the midline. CT scan shows a well-encapsulated intrarenal mass. Biopsy reveals blastemal, stromal, and epithelial cell types. Which genetic alteration is characteristically associated with the bilateral form of this tumor?
46. A 5-year-old boy presents with recurrent painful swelling of hands and feet (dactylitis), severe anemia (Hb 6 g/dL), and jaundice. Peripheral smear shows sickle-shaped red cells and target cells. Hemoglobin electrophoresis confirms Hb SS disease. Which of the following organisms most commonly causes life-threatening sepsis in this child due to functional asplenia?
47. A 4-year-old boy has easy bruising since learning to walk, hemarthroses, and prolonged bleeding after dental extraction. His maternal uncle has a similar condition. Laboratory investigations: PT normal, aPTT markedly prolonged, bleeding time normal, platelet count normal. Mixing study corrects the aPTT. Factor VIII assay: 2% (severely reduced). What is the mode of inheritance and treatment of choice for acute bleeds?