Langerhans cell histiocytosis and primary hemophagocytic lymphohistiocytosis with persistent clonal T-large granular lymphocyte proliferation

Langerhans cell histiocytosis and primary hemophagocytic lymphohistiocytosis with persistent... 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 [1], (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, http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Annals of Hematology Springer Journals

Langerhans cell histiocytosis and primary hemophagocytic lymphohistiocytosis with persistent clonal T-large granular lymphocyte proliferation

Loading next page...
 
/lp/springer_journal/langerhans-cell-histiocytosis-and-primary-hemophagocytic-DezamWAouW
Publisher
Springer Berlin Heidelberg
Copyright
Copyright © 2018 by Springer-Verlag GmbH Germany, part of Springer Nature
Subject
Medicine & Public Health; Hematology; Oncology
ISSN
0939-5555
eISSN
1432-0584
D.O.I.
10.1007/s00277-018-3298-5
Publisher site
See Article on Publisher Site

Abstract

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 [1], (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,

Journal

Annals of HematologySpringer Journals

Published: Mar 26, 2018

References

You’re reading a free preview. Subscribe to read the entire article.


DeepDyve is your
personal research library

It’s your single place to instantly
discover and read the research
that matters to you.

Enjoy affordable access to
over 18 million articles from more than
15,000 peer-reviewed journals.

All for just $49/month

Explore the DeepDyve Library

Search

Query the DeepDyve database, plus search all of PubMed and Google Scholar seamlessly

Organize

Save any article or search result from DeepDyve, PubMed, and Google Scholar... all in one place.

Access

Get unlimited, online access to over 18 million full-text articles from more than 15,000 scientific journals.

Your journals are on DeepDyve

Read from thousands of the leading scholarly journals from SpringerNature, Elsevier, Wiley-Blackwell, Oxford University Press and more.

All the latest content is available, no embargo periods.

See the journals in your area

DeepDyve

Freelancer

DeepDyve

Pro

Price

FREE

$49/month
$360/year

Save searches from
Google Scholar,
PubMed

Create lists to
organize your research

Export lists, citations

Read DeepDyve articles

Abstract access only

Unlimited access to over
18 million full-text articles

Print

20 pages / month

PDF Discount

20% off