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Difficulty: EasyPediatric Hematology and Oncology

Correlate the following pediatric hematology and oncology conditions with their characteristic microscopic, histologic, or laboratory hallmarks.

  • Sickle cell disease (in a school-aged child)Nuclear remnants in red blood cells (Howell-Jolly bodies)
  • Hereditary spherocytosisErythrocytes lacking central pallor with elevated mean corpuscular hemoglobin concentration (MCHC)
  • Acute lymphoblastic leukemiaLymphoblasts expressing terminal deoxynucleotidyl transferase (TdT)
  • NeuroblastomaSmall round blue cells forming Homer-Wright rosettes

Answer

Sickle cell disease correlates with Howell-Jolly bodies; hereditary spherocytosis correlates with erythrocytes lacking central pallor; acute lymphoblastic leukemia correlates with TdT-positive lymphoblasts; and neuroblastoma correlates with Homer-Wright rosettes.
Each condition has a pathognomonic diagnostic feature: Sickle cell disease in older children uniformly leads to autosplenectomy, producing Howell-Jolly bodies. Hereditary spherocytosis involves RBC membrane defects resulting in spherocytes and high MCHC. Acute lymphoblastic leukemia originates from immature lymphoid precursors which are universally TdT-positive. Neuroblastoma is an embryonal tumor of the sympathetic nervous system characterized by Homer-Wright rosettes.

Step-by-Step Solution

1
Evaluate the clinical consequence of sickle cell disease in older children.
Identify that autosplenectomy occurs, leading to the appearance of Howell-Jolly bodies on peripheral smear.
The spleen normally removes these nuclear remnants, but microvascular occlusion destroys splenic function early in childhood.
2
Recall the classic cellular morphology of hereditary spherocytosis.
Match it with erythrocytes lacking central pallor and an elevated MCHC.
A defect in RBC membrane tethering proteins reduces surface area-to-volume ratio, making the cells round rather than biconcave.
3
Identify the primary flow cytometry marker for the most common pediatric leukemia (ALL).
Match ALL with TdT-positive lymphoblasts.
TdT is a classic marker of early lymphoid differentiation, distinguishing lymphoblasts from myeloblasts.
4
Recall the classic histologic finding for neuroblastoma.
Match it with Homer-Wright rosettes.
These rosettes are characteristic of several pediatric neural crest and embryonal tumors, most notably neuroblastoma.

Key Concept

Classic pathologic and laboratory findings in pediatric hematology and oncology
Estimated Time:45s
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Topics

Pediatric Hematology and Oncology

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