Abstract:
:Five paediatric cases of haemophagocytic lymphohistiocytosis (HLH) which showed proliferation of granular atypical lymphocytoid cells in bone marrow are reported. All cases were girls aged 8 months to 4 years who had marked hepatosplenomegaly. Marker analysis on peripheral blood mononuclear cells revealed an increase in the CD3+HLADR+ subset in three cases and the CD3- CD56+ subset in one case. An Epstein-Barr virus genome was detected in three cases, and monoclonality was confirmed in two cases. A characteristic morphology of large granular lymphocytes (LGL) was identified, with elongated bizarre features that resembled horsetail-, tadpole-, cucumber- or shooting star-type configurations on the bone marrow smear. Serum concentrations of soluble interleukin-2 receptor and interferon-gamma were elevated in all cases. All five cases required multi-agent chemotherapy which resulted in two complete remission, two partial remissions and one no response. Refinement of treatment is required for these paediatric GLPD cases which probably comprise a specific high-risk subgroup among secondary HLH patients which had previously escaped notice.
journal_name
Br J Haematoljournal_title
British journal of haematologyauthors
Imashuku S,Hibi S,Morinaga S,Takagi K,Chen J,Mugishima H,Ishii T,Sako M,Arakawa H,Kato Mdoi
10.1046/j.1365-2141.1997.d01-2082.xsubject
Has Abstractpub_date
1997-03-01 00:00:00pages
708-14issue
4eissn
0007-1048issn
1365-2141journal_volume
96pub_type
杂志文章abstract::Hypercalcaemia complicating acute myelogenous leukaemia is a rare but well-recognized phenomenon. In most cases the pathogenetic mechanism causing the hypercalcaemia remains poorly understood. We recently studied in detail two patients with acute myelogenous leukaemia who developed hypercalcaemia during the course of ...
journal_title:British journal of haematology
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journal_title:British journal of haematology
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journal_title:British journal of haematology
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journal_title:British journal of haematology
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journal_title:British journal of haematology
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doi:
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journal_title:British journal of haematology
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doi:10.1046/j.1365-2141.1997.d01-2062.x
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journal_title:British journal of haematology
pub_type: 临床试验,杂志文章,随机对照试验
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journal_title:British journal of haematology
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journal_title:British journal of haematology
pub_type: 临床试验,杂志文章,随机对照试验
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journal_title:British journal of haematology
pub_type: 杂志文章
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