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Difficulty: MediumPediatric Hematology and Oncology

Match each pediatric patient's clinical and laboratory presentation to the most likely hematologic or oncologic diagnosis.

  • A 3-year-old boy presents with pallor, bone pain, and hepatosplenomegaly. Bone marrow aspirate reveals >20% blasts positive for TdT, CD10, and CD19, with a t(12;21) translocation.B-cell Acute Lymphoblastic Leukemia (B-ALL)
  • A 7-year-old boy from central Africa presents with a rapidly growing jaw mass. Biopsy reveals a 'starry sky' appearance and a t(8;14) translocation.Endemic Burkitt Lymphoma
  • A 10-month-old girl of Mediterranean descent presents with severe anemia and hepatosplenomegaly. Hemoglobin electrophoresis shows absent HbA and elevated HbF.Beta-Thalassemia Major
  • A 5-year-old girl presents with petechiae, bruising, and mucosal bleeding following a recent viral illness. Laboratory testing shows isolated thrombocytopenia with normal coagulation studies.Immune Thrombocytopenia (ITP)

Answer

Properly match the clinical vignettes to B-ALL, Endemic Burkitt Lymphoma, Beta-Thalassemia Major, and Immune Thrombocytopenia (ITP) respectively.
Each vignette provides pathognomonic clues for a specific pediatric hematologic or oncologic diagnosis. TdT and t(12;21) are specific for B-ALL; 'starry sky' histology and t(8;14) are specific for Burkitt Lymphoma; absent HbA identifies Beta-Thalassemia Major; and isolated post-viral thrombocytopenia indicates ITP.

Step-by-Step Solution

1
Analyze the first presentation: 3-year-old with bone pain, hepatosplenomegaly, and TdT+/CD10+ blasts with t(12;21).
Matches B-cell Acute Lymphoblastic Leukemia.
These are classic markers and a favorable prognostic translocation for pediatric B-ALL.
2
Analyze the second presentation: 7-year-old with a jaw mass, 'starry sky' biopsy, and t(8;14).
Matches Endemic Burkitt Lymphoma.
The African origin, jaw involvement, and c-myc translocation strongly point to the endemic form of this high-grade B-cell lymphoma.
3
Analyze the third presentation: 10-month-old with severe anemia and absent HbA on electrophoresis.
Matches Beta-Thalassemia Major.
The timing corresponds with the physiologic decline of fetal hemoglobin, and the absence of HbA confirms a severe beta-globin gene defect.
4
Analyze the fourth presentation: 5-year-old with post-viral petechiae and isolated thrombocytopenia.
Matches Immune Thrombocytopenia (ITP).
The lack of other cytopenias or coagulation abnormalities in a post-viral setting is highly specific for ITP.

Key Concept

Identifying hallmark clinical, genetic, and laboratory features of common pediatric hematologic and oncologic disorders.
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Topics

Pediatric Hematology and Oncology

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