Langerhans cell histiocytosis and primary hemophagocytic lymphohistiocytosis with persistent clonal T-large granular lymphocyte proliferation
Langerhans cell histiocytosis (LCH) is a clonal neoplastic disorder characterized by hyper-active RAS-RAF-MEK-ERK pathway due to acquired BRAF or MAP2K1 mutations , whereas cytokine storm secondary to inherited genetic defects in lymphocyte granule-secretory death pathway-related genes is central to primary hemophagocytic lymphohistiocytosis (pHLH). The histiocytic cells in HLH originate from monocyte/macrophage lineage, while LCH from Langerhans cell (LC) precursors. Though LCH has been reported simultaneously with secondary HLH rarely , not with pHLH.
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The patient’s mother has signed the photography release form/consent.
Conflict of interest
The authors declare that they have no conflict of interest.
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