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 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.[1]References
- Langerhans cell histiocytosis, a case of Letterer Siwe disease. Pant, C., Madonia, P., Bahna, S.L., Bass, P.F., Jeroudi, M. J. La. State. Med. Soc (2009) [Pubmed]
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