Hemophagocytic lymphohistiocytosis: Recent progress in the pathogenesis, diagnosis and treatment

doi:10.5315/wjh.v3.i3.71

Advanced Search

BPG is committed to discovery and dissemination of knowledge

Home / Archive / Volume 3, Issue 3

This Article

(391)

(349)

(0)

(10236)

(1)

Peer-Review Report of This Article

Citation of this article

Corresponding Author of This Article

Research Domain of This Article

Article-Type of This Article

Open-Access Policy of This Article

Times Cited Counts in Google of This Article

Journal Information of This Article

Publication Name

World Journal of Hematology

ISSN

2218-6204

Publisher of This Article

Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA

- Full Article with Cover (PDF)
- Full Article (XML)

Review

World J Hematol. Aug 6, 2014; 3(3): 71-84
Published online Aug 6, 2014. doi: 10.5315/wjh.v3.i3.71

Table 1 List of primary hemophagocytic lymphohistiocytosis

Disease	Molecular abnormalities(chromosome location)
CTL molecule dysfunction
Pore formation
FHL2	Perforin (10q21-2)
Vesicle priming fusion
FHL3	Munc13-4/Unc 13D (17q25)
FHL4	Syntaxin 11 (6q24)
FHL5	STXBP2/Munc18-2 (19p13)
Vesicle docking/trafficking
Chediak-Higashi syndrome	LYST (1q42.1-42.2)
Griscelli syndrome, type 2	Rab27a (15q21)
Hermansky-Pudlak syndrome II	AP-3 (3q24)
EBV-driven
XLP1	SAP/SH2D1A (Xq25)
XLP2 (XIAP)	BIRC4 (Xq24-25)
ITK deficiency¹	ITK (5q34)
CD27 deficiency¹	CD27 (12p13)
XMEN¹	MAGT1 (Xq21.1)

¹Major clinical features in these diseases are not hemophagocytic lymphohistiocytosis (HLH) but Epstein-Barr virus (EBV)-associated lymphoproliferative disease. FHL: Familial hemophagocytic lymphohistiocytosis; XLP: X-linked lymphoproliferative disease; ITK: IL-2-inducible T cell kinase; XMEN: X-linked immunodeficiency with Mg²⁺ defect, EBV infection and neoplasia; MAGT1: Magnesium transporter 1, LYST is also called CHS1 gene. Association of hemophagocytosis was described in some cases.

Full Size Table

Citation: Imashuku S. Hemophagocytic lymphohistiocytosis: Recent progress in the pathogenesis, diagnosis and treatment. World J Hematol 2014; 3(3): 71-84
URL: https://www.wjgnet.com/2218-6204/full/v3/i3/71.htm
DOI: https://dx.doi.org/10.5315/wjh.v3.i3.71

Imashuku S. Hemophagocytic lymphohistiocytosis: Recent progress in the pathogenesis, diagnosis and treatment. World J Hematol 2014; 3(3): 71-84 [DOI: 10.5315/wjh.v3.i3.71]

All content on this site: Copyright © 1993-2026 Baishideng Publishing Group Inc, its licensors, and contributors. All rights are reserved, including those for text and data mining, AI training, and similar technologies. For all open access content, the relevant licensing terms apply.