[Abt-Letterer-Siwe disease. Diagnosis and therapy (author's transl)]
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[Abt-Letterer-Siwe disease. Diagnosis and therapy (author's transl)]. / Wolff, H H; Janka-Schaub, Gritta.
in: MONATSSCHR KINDERH, Jahrgang 126, Nr. 7, 7, 1978, S. 425-430.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - [Abt-Letterer-Siwe disease. Diagnosis and therapy (author's transl)]
AU - Wolff, H H
AU - Janka-Schaub, Gritta
PY - 1978
Y1 - 1978
N2 - 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".
AB - 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".
M3 - SCORING: Zeitschriftenaufsatz
VL - 126
SP - 425
EP - 430
JO - MONATSSCHR KINDERH
JF - MONATSSCHR KINDERH
SN - 0026-9298
IS - 7
M1 - 7
ER -
