[Treatment of hemophagocytic lymphohistiocytosis, HLH, with bone marrow transplantation].

Abstract:

:Hemophagocytic lymphohistiocytosis (HLH) is a rare disease of infancy and young childhood. The clinical presentation includes recurrent unexplained fever with hepatosplenomegaly. Cytopenia, hypofibrinogenemia and/or hypertriglyceridemia and hemophagocytosis in bone marrow, spleen and lymphnode confirm the diagnosis. Hemophagocytosis may not be present at the beginning. In these cases, diagnosis is facilitated by a positive family history, a relapsing course of the disease, the frequent involvement of the central nervous system and positive findings on immunological work-up. Treatment by chemotherapy and immunosuppressants can achieve sustained remissions in most patients and reinduction of remission after relapse is possible. Most children however, eventually die from progressive disease. At present, allogeneic bone marrow transplantation is the only curative therapeutic option. Between August 1992 and May 1997 eleven consecutive patients with HLH received bone marrow from unrelated (n = 7) or matched sibling donors (n = 4). The conditioning regimen consisted of busulfan, VP-16 and cyclophosphamide. Patients engrafted after a median time of 16 days (13-43). Only one patient developed grade III acute GVHD, another patient, grade II acute GVHD. Although regimen-related toxicity was extensive, all patients have survived without signs of HLH after a median follow up of 20 months (8-63). One patient suffers from chronic GVHD, three patients reveal psychomotoric retardation and one patient has severe impairment with spastic tetraparesis, amaurosis and seizures. Our experience shows that HLH can be successfully treated by allogeneic BMT from unrelated donors.

journal_name

Klin Padiatr

journal_title

Klinische Padiatrie

authors

Dürken M,Schneider EM,Blütters-Sawatzki R,Stollmann-Gibbels B,Nessler G,Bretz R,Körholz D,Probst EN,Holsten-Griffin H,Harps E,Zander AR,Janka GE

doi

10.1055/s-2008-1043876

subject

Has Abstract

pub_date

1998-07-01 00:00:00

pages

180-4

issue

4

eissn

0300-8630

issn

1439-3824

journal_volume

210

pub_type

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