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.
Louisiana State University Health Sciences Center, Department of Medicine/Pediatrics, Shreveport, LA, USA.
Chaitanya
C
Pant
Phillip
P
Madonia
Sami L
SL
Bahna
Pat F
PF
Bass
Majed
M
Jeroudi
D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D017338:Cladribine / Q000627:therapeutic use; D003561:Cytarabine / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D008297:Male; D011241:Prednisone / Q000627:therapeutic use; D012008:Recurrence; D016879:Salvage Therapy; D012867:Skin / Q000473:pathology; D014747:Vinblastine / Q000627:therapeutic use
D000970:Antineoplastic Agents; D003561:Cytarabine; D017338:Cladribine; D014747:Vinblastine; D011241:Prednisone