Annals of Hematology (2018) 97:1295–1296 https://doi.org/10.1007/s00277-018-3298-5 LETTER TO THE EDITOR Langerhans cell histiocytosis and primary hemophagocytic lymphohistiocytosis with persistent clonal T-large granular lymphocyte proliferation 1 2 2 Süreyya Savaşan & Janet Poulik & Ali Gabali Received: 4 November 2017 /Accepted: 9 March 2018 /Published online: 26 March 2018 Springer-Verlag GmbH Germany, part of Springer Nature 2018 Dear Editor, No pathological mutations were identified in MUNC13-4, Langerhans cell histiocytosis (LCH) is a clonal neoplastic dis- STX11, STXBP2, SH2D1A, BIRC4,and RAB27A, but double order characterized by hyper-active RAS-RAF-MEK-ERK heterozygous perforin gene (PRF1) mutations, 97 C>T pathway due to acquired BRAF or MAP2K1 mutations , (R33C), and 272 C>T (A91V) were detected with very low whereas cytokine storm secondary to inherited genetic defects perforin expression. His healthy HLA-matched twin sister is in lymphocyte granule-secretory death pathway-related genes heterozygous for A91V PRF1 mutation with borderline–low is central to primary hemophagocytic lymphohistiocytosis perforin expression. The patient was treated with vinblastine (pHLH). The histiocytic cells in HLH originate from and prednisone induction and additional 6-mercaptopurine monocyte/macrophage lineage, while LCH from Langerhans during maintenance. He completed 1-year-long course with cell (LC) precursors. Though LCH has been reported simul- resolution of findings. Due to his uneventful course,
Annals of Hematology – Springer Journals
Published: Mar 26, 2018
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