HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.

Abstract:

:In HLH-94, the first prospective international treatment study for hemophagocytic lymphohistiocytosis (HLH), diagnosis was based on five criteria (fever, splenomegaly, bicytopenia, hypertriglyceridemia and/or hypofibrinogenemia, and hemophagocytosis). In HLH-2004 three additional criteria are introduced; low/absent NK-cell-activity, hyperferritinemia, and high-soluble interleukin-2-receptor levels. Altogether five of these eight criteria must be fulfilled, unless family history or molecular diagnosis is consistent with HLH. HLH-2004 chemo-immunotherapy includes etoposide, dexamethasone, cyclosporine A upfront and, in selected patients, intrathecal therapy with methotrexate and corticosteroids. Subsequent hematopoietic stem cell transplantation (HSCT) is recommended for patients with familial disease or molecular diagnosis, and patients with severe and persistent, or reactivated, disease. In order to hopefully further improve diagnosis, therapy and biological understanding, participation in HLH studies is encouraged.

journal_name

Pediatr Blood Cancer

journal_title

Pediatric blood & cancer

authors

Henter JI,Horne A,Aricó M,Egeler RM,Filipovich AH,Imashuku S,Ladisch S,McClain K,Webb D,Winiarski J,Janka G

doi

10.1002/pbc.21039

subject

Has Abstract

pub_date

2007-02-01 00:00:00

pages

124-31

issue

2

eissn

1545-5009

issn

1545-5017

journal_volume

48

pub_type

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