[Abt-Letterer-Siwe disease. Diagnosis and therapy (author's transl)]

An infant with Abt-Letterer-Siwe disease is reported. The diagnosis was established by histopathology and electron microscopy.--2. Complete remission was achieved with vinblastine and prednisone. Treatment, however, in these cases has to be continued for a longer period of time.--3. Early diagnosis and therapy are important because Abt-Letterer-Siwe disease is lethal in most untreated cases. With adequate chemotherapy, long remissions or cures can be expected in about 70% of the cases.--4. In Abt-Letterer-Siwe disease, the racket-like Langerhans cell granules are found by electron microscopy within the specific infiltrating cells. The demonstration of these organells allows the unequivocal diagnosis in cases with uncharacteristic clinical or histopathological appearance. The same structures are characteristic of Hand-Schüller-Christian disease and of eosinophilic granuloma. The electron microscopic findings confirm the grouping of these three diseases together as "histiocytosis X".