Autoimmune Lymphoproliferative Syndrome with Langerhans Cell
Histiocytosis Diagnosis: A Case Report
Autoimmune lymphoproliferative syndrome (ALPS) is a disorder of
lymphocyte apoptosis characterized by nonmalignant lymphadenopathy,
splenomegaly, immune dysregulation, and autoimmune cytopenias.
Langerhans cell histiocytosis (LCH) is a neoplasm of myeloid precursor
cells, primarily presenting as bone, soft tissue, lung, skin, and
pituitary lesions. The association between the two diagnoses is
uncharacterized. Here we describe a patient presenting with fever of
unknown origin, cytopenias, and failure to thrive. She was diagnosed
with ALPS, then subsequently LCH, within three months. This case
highlights the diagnostic and management challenges associated with
these two rare diagnoses.