Langerhans Cell Histiocytosis Responding to 2-Cda and Imatinib Mesylate (IM)

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare condition in which granulomas form in tissues through the accumulation of abnormal histiocytes (Langerhans cells), granulocytes and lymphocytes. Prognosis in patients with disseminated disease has been associated with a chronic course, and a high morbidity and mortality rate. In terms of treatment, etoposide and vinblastine are considered to be the most effective agents in monochemotherapy. Here we report the case of an adult patient with LCH who has improved considerably after treatment with 2-CDA followed by IM. CLINICAL CASE: 37 year-old Hispanic female with history of burning epigastric pain, nausea and vomiting starting in 1998. In 1999 diarrhea and steatorrhea appeared. Whipple disease was diagnosed in another hospital but treatment was unsuccessful. She came to our hospital in 2003, complaining of the aforementioned symptoms plus severe malnutrition. Upper GI endoscopy and colonoscopy were performed and the biopsies showed infiltration with LCH. Bone marrow (BM) biopsy showed LCH infiltration too. CD117 (KIT) expression was investigated both in gastric and BM biopsies, turning out to be highly positive. Therapies and outcomes are shown in table 1. | DATE | DRUGS | TREATMENT DURATION | WEIGHT (Kg) | ALBUMIN (g/dL) | GI ENDOSCOPY | BIOPSY | |:------ | ------------- | ------------------ | ----------- | -------------- | -------------------------- | ----------------------------- | | Dec/03 | VBL/VP-16/PDN | 5 cycles | 33.5 | 0.99 | Polyps and pseudopolyps | Infiltrated by LCH. | | Sep/05 | MTX/PDN | 18 mos. | 39.5 | 3.18 | Polyps and pseudopolyps | Infiltrated. | | Jul/07 | 2-CDA | 9 cycles | 55.3 | 4.0 | Polyps | Infiltrated. | | May/08 | IM | 6 mos. | 57 | 4.0 | Nodular pattern. No polyps | Decreased infiltration by LCH | CONCLUSIONS: This case illustrates that multisystemic LCH can be successfully treated with, at least two more drug choices: 2-CDA and, in those patients with expression of KIT and/or PDGF, IM.