Abstract:
:Two important syndromes of hemophagocytic lymphohistiocytosis (HLH) have to be considered in infants and young children with recurrent fever, organomegaly and cytopenias. Familial hemophagocytic lymphohistiocytosis (FHLH) is a genetically heterogeneous autosomal recessive disease with histiocytic and lymphocytic infiltrations in multiple organs and is currently curable only by bone marrow transplantation (BMT). Secondary HLH most commonly results from viral infections and some patients may be cured by treating the causative organism, others will need chemotherapy and immunosuppression. Since infections can also trigger disease episodes in FHLH, making the correct diagnosis can prove difficult. The published experience of BMT in HLH is reviewed. Taken together, cure of the majority of patients with HLH by matched related BMT, unrelated or haploidentical BMT is possible. Incomplete resolution of disease activity does not necessarily impede a successful outcome. Central nervous system involvement will eventually develop in many HLH patients and may cause considerable morbidity. Appropriate early treatment and a timely BMT will hopefully decrease mortality rates and improve neurodevelopmental outcome in this disease.
journal_name
Leuk Lymphomajournal_title
Leukemia & lymphomaauthors
Dürken M,Finckenstein FG,Janka GEdoi
10.3109/10428190109057957keywords:
subject
Has Abstractpub_date
2001-03-01 00:00:00pages
89-95issue
1-2eissn
1042-8194issn
1029-2403pii
I308J001164journal_volume
41pub_type
杂志文章,评审abstract::Since etoposide interacts with the nuclear enzyme topoisomerase II, the drug concentrations in the malignant cells during chemotherapy may have clinical correlates. Plasma protein binding of etoposide is extensive (94%) and alterations of the non-proteinbound fraction affect pharmacokinetic behavior of the drug. The p...
journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.3109/10428199309148555
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abstract::Hematopoietic progenitor cells from different sources have been widely characterized, but their ultrastructural morphology has never been described in detail. In this study, imunomagnetically separated CD34+ cells from normal bone marrow (BM), mobilized peripheral blood (PBSC) and human umbilical cord blood (CB) were ...
journal_title:Leukemia & lymphoma
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.1080/1042819031000067873
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journal_title:Leukemia & lymphoma
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journal_title:Leukemia & lymphoma
pub_type: 评论,杂志文章
doi:10.1080/10428194.2019.1613545
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abstract::Data from the large, prospective, multinational, phase 3b JUMP study were analyzed to identify factors predictive of spleen and symptom responses in myelofibrosis patients receiving ruxolitinib. Factors associated with higher spleen response rates included International Prognostic Scoring System (IPSS) low/intermediat...
journal_title:Leukemia & lymphoma
pub_type: 杂志文章
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章,多中心研究
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更新日期:2013-10-01 00:00:00
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.3109/10428199809050933
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章,评审
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journal_title:Leukemia & lymphoma
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.3109/10428199309047880
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.1080/10428190500518966
更新日期:2006-07-01 00:00:00
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journal_title:Leukemia & lymphoma
pub_type: 历史文章,杂志文章,评审
doi:10.3109/10428190903219667
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journal_title:Leukemia & lymphoma
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doi:10.3109/10428194.2012.717080
更新日期:2013-03-01 00:00:00
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journal_title:Leukemia & lymphoma
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doi:10.3109/10428194.2013.809077
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abstract::To define risk groups in children with acute myeloid leukaemia (AML), we conducted a multivariate stepwise Cox regression analysis of three consecutive multicentre studies in East Germany. The total number of patients was 240, but cytogenetics and remission status on day 15 were routinely investigated only in the most...
journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.3109/10428190109097754
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.3109/10428194.2010.520774
更新日期:2010-11-01 00:00:00
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章,评审
doi:10.1080/1042819031000077007
更新日期:2003-07-01 00:00:00
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.1080/10428190601094347
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journal_title:Leukemia & lymphoma
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.1080/10428194.2017.1298754
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章
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journal_title:Leukemia & lymphoma
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journal_title:Leukemia & lymphoma
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journal_title:Leukemia & lymphoma
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章,评审
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journal_title:Leukemia & lymphoma
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