Abstract:
:The underlying mechanisms of reactive macrophage activation syndromes (rMAS) are not understood in detail, and there is no specific treatment. This observational study was prompted by intravenous immunoglobulin (IVIG), dramatically halting two distinct rMAS episodes in the same patient. We evaluated the potential benefits of IVIG administration in treating fulminant rMAS and the usefulness of monitoring serum ferritin levels as an indication for emergency treatment with IVIG. Ten females and 10 males experiencing 22 episodes of rMAS were recruited on the basis of serum ferritin levels >or=10,000 microg/l and/or direct evidence of haemophagocytosis in 11 intensive care units in secondary and tertiary care hospitals in Switzerland between October 1993 and May 2000. In individual patients, serially measured ferritin was closely related to disease activity. Abrupt increases of up to >100,000 microg/l could be observed within hours. Rapid and profound beneficial effects of emergency IVIG treatment were seen in 12 episodes of rMAS accompanied by a prompt decrease of serum ferritin. IVIG produced partial or delayed improvements in 5 patients. No apparent effects were seen in 5 patients. IVIG was only successful if started early during the ferritin run-up to peak values. In conclusion, IVIG is effective in at least a subgroup of adult rMAS when started at the beginning of the macrophage activation process. The monitoring of serum ferritin levels might be helpful in detecting macrophage activation in order to commence IVIG treatment early enough.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Emmenegger U,Frey U,Reimers A,Fux C,Semela D,Cottagnoud P,Spaeth PJ,Neftel KAdoi
10.1002/ajh.1141keywords:
subject
Has Abstractpub_date
2001-09-01 00:00:00pages
4-10issue
1eissn
0361-8609issn
1096-8652pii
10.1002/ajh.1141journal_volume
68pub_type
杂志文章abstract::We report on the association of Hb Dunn (alpha 6[A4]Asp----Asn) and Hb O-Arab (beta 121 [GH4]Glu----Lys) in a healthy Moroccan man. Hb Dunn had the same electrophoretic properties as Hb G-Philadelphia, but its percentage was lower. Its identification was based on sequence determination of the alpha T1 peptide. Bgl II ...
journal_title:American journal of hematology
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doi:10.1002/ajh.2830270405
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abstract::Twenty-seven lymphomas of mucosa-associated lymphoid tissue (MALT) derived from distinct anatomical sites were tested for the presence of genetic lesions commonly involved in B-cell lymphomagenesis, including activation of proto-oncogenes (BCL-1, BCL-2, BCL-6, and c-MYC), disruption of tumor suppressor loci (p53, 6q),...
journal_title:American journal of hematology
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journal_title:American journal of hematology
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pub_type: 信件,多中心研究
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journal_title:American journal of hematology
pub_type: 杂志文章,评审
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journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究,随机对照试验
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