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

doi:10.5315/wjh.v3.i3.71

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World Journal of Hematology

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2218-6204

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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.

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