Langerhans cell histiocytosis, a case of Letterer Siwe disease

An 8-month-old male infant presented with a progressively worsening generalized rash of 5-6 months duration, fever, poor feeding, and abdominal distension. An initial laboratory workup revealed anemia, thrombocytopenia, and hepatosplenomegaly. The patient was started on i.v. antibiotics, and a worki...

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Bibliographic Details
Published inThe Journal of the Louisiana State Medical Society Vol. 161; no. 4; p. 211
Main Authors Pant, Chaitanya, Madonia, Phillip, Bahna, Sami L, Bass, Pat F, Jeroudi, Majed
Format Journal Article
LanguageEnglish
Published United States 01.07.2009
Subjects
Antineoplastic Agents - therapeutic use
Child
Cladribine - therapeutic use
Cytarabine - therapeutic use
Drug Therapy, Combination
Histiocytosis, Langerhans-Cell - diagnosis
Histiocytosis, Langerhans-Cell - drug therapy
Humans
Male
Prednisone - therapeutic use
Recurrence
Salvage Therapy
Skin - pathology
Vinblastine - therapeutic use
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Summary:An 8-month-old male infant presented with a progressively worsening generalized rash of 5-6 months duration, fever, poor feeding, and abdominal distension. An initial laboratory workup revealed anemia, thrombocytopenia, and hepatosplenomegaly. The patient was started on i.v. antibiotics, and a working diagnosis of Langerhans cell histiocytosis was reached that was later confirmed with a skin biopsy. Subsequently, the patient received first-round chemotherapy with vinblastine and prednisone, on which he appeared to improve clinically; however, he soon relapsed. He then received combination salvage therapy with cladribine (2CdA) and cytarabine (Ara-C) for three cycles. The patient responded well to this regimen with resolution of his condition. The patient was then referred for a bone marrow transplant.
ISSN:0024-6921