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Langerhans cell histiocytosis, a case of Letterer Siwe disease

Chaitanya Pant1, Phillip Madonia, Sami L Bahna

  • 1Louisiana State University Health Sciences Center, Department of Medicine/Pediatrics, Shreveport, LA, USA.

The Journal of the Louisiana State Medical Society : Official Organ of the Louisiana State Medical Society
|September 30, 2009
PubMed

Insights

This case study details an infant with Langerhans cell histiocytosis, a rare condition. Salvage therapy with cladribine and cytarabine effectively treated the infant after initial chemotherapy failed.

Area of Science:

  • Pediatric Oncology
  • Dermatology
  • Hematology

Background:

  • Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder of Langerhans cell precursors.
  • Infantile LCH often presents with multisystem involvement, including skin, bone, and visceral organs.

Observation:

  • An 8-month-old infant presented with a chronic rash, fever, poor feeding, and abdominal distension.
  • Initial workup revealed anemia, thrombocytopenia, and hepatosplenomegaly, leading to a suspicion of LCH.
  • Diagnosis was confirmed via skin biopsy.

Findings:

  • The patient initially improved with vinblastine and prednisone chemotherapy but relapsed.
  • Combination salvage therapy with cladribine (2CdA) and cytarabine (Ara-C) resulted in complete resolution of LCH.
  • The patient was subsequently referred for a bone marrow transplant.

Implications:

  • This case highlights the potential efficacy of cladribine and cytarabine combination salvage therapy for refractory or relapsed LCH in infants.
  • Early diagnosis and appropriate treatment strategies are crucial for improving outcomes in pediatric LCH.
  • Further research into novel therapeutic approaches for LCH is warranted.

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