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Iron-refractory microcytic anemia as the presenting feature of unicentric Castleman disease in children

Shanmuganathan Chandrakasan1, Nihal Bakeer1, Jun Qin Mo2

  • 1Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.

The Journal of Pediatrics
|December 26, 2013
PubMed

Insights

Unexplained iron-refractory anemia in children can signal occult Castleman disease. Surgical removal of the intra-abdominal tumor resolved both the cancer and the anemia, highlighting interleukin-6

Area of Science:

  • Pediatric Hematology
  • Oncology
  • Immunology

Background:

  • Chronic, iron-refractory, microcytic anemia presents diagnostic and therapeutic challenges.
  • Castleman disease is a rare lymphoproliferative disorder that can manifest with anemia.

Observation:

  • Two pediatric cases presented with unexplained, chronic, iron-refractory, microcytic anemia as the primary symptom.
  • Diagnosis was delayed due to occult, intra-abdominal lymphoproliferation and absence of palpable lymphadenopathy.

Findings:

  • Surgical resection of unicentric Castleman disease successfully cured both the anemia and the underlying disease.
  • Elevated interleukin-6 (IL-6) levels were identified as a key diagnostic clue, correlating with anemia induction via hepcidin.

Implications:

  • Highlights the importance of considering Castleman disease in pediatric patients with refractory anemia.
  • Suggests IL-6 as a potential biomarker for Castleman disease-associated anemia.
  • Demonstrates the curative potential of surgical intervention for this specific presentation of Castleman disease.

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