Lenalidomid–basiertes Regime bewährt sich bei einer multisystemischen Langerhans-Zellhistiozytose: Fallbericht
| Title in English | Lenalidomide–based regimen proved effective in multisystem Langerhans cell histiocytosis: case report |
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| Authors | |
| Year of publication | 2011 |
| Type | Conference abstract |
| MU Faculty or unit | |
| Citation | |
| Description | Introduction: Langerhans cell histiocytosis is a rare idiopathic disease with diverse clinical manifestations ranging from a single osteolytic lesion to generalized disease. Various treatment regimens have been proposed, however, with inconsistent outcomes. Herein we are the first to report on a therapy effect of lenalidomide (Revlimid)–based regimen. Materials and Methods: A male, born 1973, was diagnosed with multisystem Langerhans cell histiocytosis affecting the lymph nodes, skin and lungs at the age of 35. The symptoms reminded a lymphoma with expressed B-symptoms and generalized lymphadenopathy. Initially, the patient was treated with 6 cycles of cladribine-based regimen combined with radiotherapy of the perianal area, this led to complete remission. However, in two months the disease relapsed, newly also with bone involvement. The second line treatment consisted of 4 cycles of CHOEP regimen (cyclophosphamide, doxorubicin, vincristine, etoposide, prednisone) completed in March 2010 with high-dose BEAM (carmustine, etoposide, cytarabine, melphalan) chemotherapy followed by autologous peripheral blood stem cell transplantation, which put the disease into complete remission again. Nevertheless, after 5 months the 2nd relapse was diagnosed and the patient was started on lenalidomide (25 mg orally days 1-21 of a 28-day cycle) with dexamethasone (40 mg orally once a week) treatment in October 2010 (8 planned cycles). Results: Within several doses of lenalidomide, fatigue and other B-symptoms receded gradually, which was followed by a decrease of blood inflammatory markers. A restaging PET/CT examination during the 4th cycle showed reduction in the size of affected lymph nodes and their glucose uptake as well as generally reduced extent of the disease. Moreover, through a series of several ultrasound examinations, we documented gradual regression of enlarged lymph nodes during the therapy. To date, 6 cycles have been carried out. Conclusions: Lenalidomide–based regimen proved effective in a patient with the repeatedly relapsed aggressive form of multisystem Langerhans cell histiocytosis as demonstrated in laboratory and radiological data enclosed. |