Which conditions are included in the differential diagnoses of multisystemic Langerhans cell histiocytosis (LCH)?

Updated: Sep 16, 2020
  • Author: Cameron K Tebbi, MD; Chief Editor: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP  more...
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Answer

With multisystemic disease, Langerhans cell histiocytosis must be distinguished from familial hemophagocytic lymphohistiocytosis (FHL) and viral-associated hemophagocytic syndrome.

Lung, liver, [82] and GI involvement are often, but not always, associated with systemic disease and must be differentiated from immune deficiency, leukemia, and metastatic solid tumors. Single-system disease has been reported in the eyes [222] and lungs [24, 223] in patients, including a newborn. [224] Serum KL-6 levels have been used as a marker and appeared to be correlated with pulmonary involvement in an infant with Langerhans cell histiocytosis. [225]


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