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Langerhans Cell Histiocytosis: Clinical Experience with 124 Patients
Author(s) -
RiveraLuna Roberto,
MartinezGuerra Guillermo,
AltamiranoAlvarez Eduardo,
MartinezAvalos Armando,
CardenasCardoz Rocio,
AyonCardenas Ana,
RuizMaldonado Ramon,
LopezCorella Eduardo
Publication year - 1988
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.1988.tb01160.x
Subject(s) - medicine , langerhans cell histiocytosis , histiocytosis , disease , organ dysfunction , lymph node , lung , lymph , chemotherapy , pathology , sepsis
We cared for 124 pediatric patients With a histologic diagnosis of Langerhans' cell histiocytosis (histiocytosis X) over a period of 14 years. Clinical, laboratory. and radiographic findings were analyzed. The most frequent manifestations were bone lesions, lymph node involvement, and skin infiltralion. Liver disease was noted in 50% of patients and lung disease in 23% he‐matologic changes were also frequent‐ Dysfunction and involvement of these three organ systems, plus age of onset, distinguished the group of patients with the highest mortality. All palienis with generalized disease or organ dysfunclion were Lrealed with systemie chemotherapy. The actuarial survival curve at 10 Years was 63%