Successful treatment of Langerhans cell histiocytosis with 2-chlorodeoxyadenosine.
Langerhans cell histiocytosis (LCH) is a rare disorder which frequently involves the lungs of affected adults. Recent evidence suggests it is a clonal neoplastic disorder. Prognosis in this disease is variable, but in its multisystem form or when associated with progressive respiratory dysfunction, prognosis is poor. Recent case reports and a phase II trial of the antimonocyte drug 2-chlorodeoxy-adenosine (2CDA) have described success in treating LCH. We used 2CDA to treat a young Australian man with LCH involving lungs and bone. A complete symptomatic remission was achieved with no evidence of recurrence some 5 years after completion of chemotherapy.
Goh
Nicole S L
NS
Royal Brompton Hospital, London, United Kingdom.
McDonald
Christine E
CE
MacGregor
Duncan P
DP
Pretto
Jeffrey J
JJ
Brodie
Graeme N
GN
D000328:Adult; D000970:Antineoplastic Agents / Q000008:administration & dosage*; D001707:Biopsy, Needle; D017338:Cladribine / Q000008:administration & dosage*; D004334:Drug Administration Schedule; D005500:Follow-Up Studies; D006646:Histiocytosis, Langerhans-Cell / Q000188:drug therapy* / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007262:Infusions, Intravenous; D008171:Lung Diseases / Q000188:drug therapy* / Q000473:pathology*; D008297:Male; D012129:Respiratory Function Tests; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D000970:Antineoplastic Agents; D017338:Cladribine