Langerhans cells histiocytosis in one family.
Histiocytosis of Langerhans cells (class 1 histiocytosis) includes a range of clinical manifestations that have been described as bone eosinophilic granuloma, Hand-Schüller-Christian syndrome, and Letterer-Siwe diseases. This syndrome represents a spectrum of severity and prognosis of some underlying disorder which is usually sporadic. This report describes three cases in one family, who developed the disease a few years after their brother was found to be suffering from histiocytosis. All 3 patients had the same clinical manifestations: hyperthermia, eczematic rash, and swelling in skull, hand, and foot. X rays showed lytic areas in the skull and metacarp of fourth finger. Serology for EBV infection was negative. Infiltration of abnormal Langerhans cells histiocytes were demonstrated in bone biopsies. These patients were given chemotherapy. Case 1 (brother) died 1 year after chemotherapy, case 2 (girl) was given chemotherapy without success. She was given T-cell suppressor (cyclosporine), which induced remission, and case 3 was given chemotherapy, which was successful.
Shahla
Ansari
A
Ali Asghar Children's Hospital, Department of Hematology-Oncology, Faculty of Medicine, Iran University of Medical Sciences, Tehran, Iran. shahladamavandi@yahoo.com
Parvaneh
Vossough
V
Hossein
Haddad Deylami
HD
D000970:Antineoplastic Agents / Q000627:therapeutic use; D002675:Child, Preschool; D016572:Cyclosporine / Q000627:therapeutic use; D004803:Eosinophilic Granuloma; D005192:Family Health; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D016896:Treatment Outcome
D000970:Antineoplastic Agents; D016572:Cyclosporine