A clinical analysis of 52 adult patients with hemophagocytic syndrome: The prognostic significance of the underlying diseases
We retrospectively analyzed 52 adult patients with hemophagocytic syndrome (HPS). The underlying diseases were heterogeneous, including malignant lymphoma (lymphoma-associated hemophagocytic syndrome [LAHS]) in 26 patients, systemic lupus erythematosus in 3 patients, viral infections in 7 patients,...
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| Veröffentlicht in: | International journal of hematology 2001-08, Vol.74 (2), p.209-213 |
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| creator | TAKAHASHI, Naoto CHUBACHI, Akihiko KUME, Masaaki HATANO, Yoshiaki KOMATSUDA, Atsushi KAWABATA, Yoshinari YANAGIYA, Norimitsu ICHIKAWA, Yoshikazu MIURA, Akira B MIURA, Ikuo |
| description | We retrospectively analyzed 52 adult patients with hemophagocytic syndrome (HPS). The underlying diseases were heterogeneous, including malignant lymphoma (lymphoma-associated hemophagocytic syndrome [LAHS]) in 26 patients, systemic lupus erythematosus in 3 patients, viral infections in 7 patients, and bacteria] or fungal infections in 6 patients. More than 83% of patients received prednisolone as an initial treatment. Multiple-agent chemotherapies (cyclophosphamide, doxorubicin, and vincristine) were administered to 96% of LAHS patients after a histopathological diagnosis of lymphoma. HPSs were controllable and remissions were achieved except for those patients with LAHS, fulminant Epstein-Barr virus-associated HPS, and an immunosuppressive state. Twenty-one (81%) of the LAHS patients had uncontrollable HPS and died of multiple organ failure and disseminated intravascular coagulation. The median survival time of LAHS patients was 83 days. In contrast, 3 (12%) of the other HPS patients died of multiple organ failure within 44 days.The clinical manifestations and the laboratory findings of LAHS and the other HPSs were too variable to establish the prognosis based only on the findings at the onset of HPS. The prognostic factors of adult HPS were found to be the underlying diseases, notably malignant lymphoma and infections, accompanied by the immunosuppressive state. |
| doi_str_mv | 10.1007/BF02982007 |
| format | Article |
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The underlying diseases were heterogeneous, including malignant lymphoma (lymphoma-associated hemophagocytic syndrome [LAHS]) in 26 patients, systemic lupus erythematosus in 3 patients, viral infections in 7 patients, and bacteria] or fungal infections in 6 patients. More than 83% of patients received prednisolone as an initial treatment. Multiple-agent chemotherapies (cyclophosphamide, doxorubicin, and vincristine) were administered to 96% of LAHS patients after a histopathological diagnosis of lymphoma. HPSs were controllable and remissions were achieved except for those patients with LAHS, fulminant Epstein-Barr virus-associated HPS, and an immunosuppressive state. Twenty-one (81%) of the LAHS patients had uncontrollable HPS and died of multiple organ failure and disseminated intravascular coagulation. The median survival time of LAHS patients was 83 days. In contrast, 3 (12%) of the other HPS patients died of multiple organ failure within 44 days.The clinical manifestations and the laboratory findings of LAHS and the other HPSs were too variable to establish the prognosis based only on the findings at the onset of HPS. The prognostic factors of adult HPS were found to be the underlying diseases, notably malignant lymphoma and infections, accompanied by the immunosuppressive state.</description><identifier>ISSN: 0925-5710</identifier><identifier>EISSN: 1865-3774</identifier><identifier>DOI: 10.1007/BF02982007</identifier><identifier>PMID: 11594524</identifier><language>eng</language><publisher>Tokyo: Springer</publisher><subject>Adult ; Aged ; Aged, 80 and over ; Biological and medical sciences ; Chronic conditions ; Coagulation ; Cyclophosphamide ; Diseases ; Disseminated intravascular coagulation ; Epstein-Barr virus ; Female ; Fungal infections ; Hematologic and hematopoietic diseases ; Hemophagocytic syndrome ; Histiocytosis, Non-Langerhans-Cell - diagnosis ; Histiocytosis, Non-Langerhans-Cell - etiology ; Histiocytosis, Non-Langerhans-Cell - mortality ; Humans ; Infection - complications ; Infection - mortality ; Infections ; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis ; Lupus Erythematosus, Systemic - complications ; Lupus Erythematosus, Systemic - mortality ; Lymphoma ; Lymphoma - complications ; Lymphoma - drug therapy ; Lymphoma - mortality ; Male ; Medical prognosis ; Medical research ; Medical sciences ; Medical treatment ; Middle Aged ; Multiple organ dysfunction syndrome ; Patients ; Prednisolone ; Prognosis ; Retrospective Studies ; Risk Factors ; Survival Rate ; Systemic lupus erythematosus ; Vincristine</subject><ispartof>International journal of hematology, 2001-08, Vol.74 (2), p.209-213</ispartof><rights>2001 INIST-CNRS</rights><rights>The Japanese Society of Hematology 2001</rights><rights>The Japanese Society of Hematology 2001.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c422t-3c13f729e5399c98f1a4bc085fad2a54be5f43b014fd005c1fc642ac70a148063</citedby><cites>FETCH-LOGICAL-c422t-3c13f729e5399c98f1a4bc085fad2a54be5f43b014fd005c1fc642ac70a148063</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27923,27924</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=1125959$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/11594524$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>TAKAHASHI, Naoto</creatorcontrib><creatorcontrib>CHUBACHI, Akihiko</creatorcontrib><creatorcontrib>KUME, Masaaki</creatorcontrib><creatorcontrib>HATANO, Yoshiaki</creatorcontrib><creatorcontrib>KOMATSUDA, Atsushi</creatorcontrib><creatorcontrib>KAWABATA, Yoshinari</creatorcontrib><creatorcontrib>YANAGIYA, Norimitsu</creatorcontrib><creatorcontrib>ICHIKAWA, Yoshikazu</creatorcontrib><creatorcontrib>MIURA, Akira B</creatorcontrib><creatorcontrib>MIURA, Ikuo</creatorcontrib><title>A clinical analysis of 52 adult patients with hemophagocytic syndrome: The prognostic significance of the underlying diseases</title><title>International journal of hematology</title><addtitle>Int J Hematol</addtitle><description>We retrospectively analyzed 52 adult patients with hemophagocytic syndrome (HPS). The underlying diseases were heterogeneous, including malignant lymphoma (lymphoma-associated hemophagocytic syndrome [LAHS]) in 26 patients, systemic lupus erythematosus in 3 patients, viral infections in 7 patients, and bacteria] or fungal infections in 6 patients. More than 83% of patients received prednisolone as an initial treatment. Multiple-agent chemotherapies (cyclophosphamide, doxorubicin, and vincristine) were administered to 96% of LAHS patients after a histopathological diagnosis of lymphoma. HPSs were controllable and remissions were achieved except for those patients with LAHS, fulminant Epstein-Barr virus-associated HPS, and an immunosuppressive state. Twenty-one (81%) of the LAHS patients had uncontrollable HPS and died of multiple organ failure and disseminated intravascular coagulation. The median survival time of LAHS patients was 83 days. In contrast, 3 (12%) of the other HPS patients died of multiple organ failure within 44 days.The clinical manifestations and the laboratory findings of LAHS and the other HPSs were too variable to establish the prognosis based only on the findings at the onset of HPS. The prognostic factors of adult HPS were found to be the underlying diseases, notably malignant lymphoma and infections, accompanied by the immunosuppressive state.</description><subject>Adult</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>Biological and medical sciences</subject><subject>Chronic conditions</subject><subject>Coagulation</subject><subject>Cyclophosphamide</subject><subject>Diseases</subject><subject>Disseminated intravascular coagulation</subject><subject>Epstein-Barr virus</subject><subject>Female</subject><subject>Fungal infections</subject><subject>Hematologic and hematopoietic diseases</subject><subject>Hemophagocytic syndrome</subject><subject>Histiocytosis, Non-Langerhans-Cell - diagnosis</subject><subject>Histiocytosis, Non-Langerhans-Cell - etiology</subject><subject>Histiocytosis, Non-Langerhans-Cell - mortality</subject><subject>Humans</subject><subject>Infection - complications</subject><subject>Infection - mortality</subject><subject>Infections</subject><subject>Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis</subject><subject>Lupus Erythematosus, Systemic - complications</subject><subject>Lupus Erythematosus, Systemic - mortality</subject><subject>Lymphoma</subject><subject>Lymphoma - complications</subject><subject>Lymphoma - drug therapy</subject><subject>Lymphoma - mortality</subject><subject>Male</subject><subject>Medical prognosis</subject><subject>Medical research</subject><subject>Medical sciences</subject><subject>Medical treatment</subject><subject>Middle Aged</subject><subject>Multiple organ dysfunction syndrome</subject><subject>Patients</subject><subject>Prednisolone</subject><subject>Prognosis</subject><subject>Retrospective Studies</subject><subject>Risk Factors</subject><subject>Survival Rate</subject><subject>Systemic lupus erythematosus</subject><subject>Vincristine</subject><issn>0925-5710</issn><issn>1865-3774</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2001</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><recordid>eNp10c9rFDEUB_AgFrtWL_4BElQ8FEZffm0m3mqxKhS81POQzSS7KTPJmjeDzMH_3axdaBF6yoP34Ut4X0JeMfjAAPTHz1fATcvr-ISsWLtWjdBaPiUrMFw1SjM4Jc8RbwGYBqmfkVPGlJGKyxX5c0HdEFN0dqA22WHBiDQHqji1_TxMdG-n6NOE9HecdnTnx7zf2W12yxQdxSX1JY_-E73ZeboveZsy_lvEbYqhpibnD3FTXc-p92VYYtrSPqK36PEFOQl2QP_y-J6Rn1dfbi6_Ndc_vn6_vLhunOR8aoRjImhuvBLGONMGZuXGQauC7blVcuNVkGIDTIYeQDkW3Fpy6zRYJltYizPy_i63fvHX7HHqxojOD4NNPs_Yac6M4BoqfPsfvM1zqXfBjq-NbOu95UG9eVQxLQwwISo6v0OuZMTiQ7cvcbRl6Rh0h966-94qfn1MnDej7-_psagK3h2BxdpVKPWyER84rowy4i_wfp7L</recordid><startdate>20010801</startdate><enddate>20010801</enddate><creator>TAKAHASHI, Naoto</creator><creator>CHUBACHI, Akihiko</creator><creator>KUME, Masaaki</creator><creator>HATANO, Yoshiaki</creator><creator>KOMATSUDA, Atsushi</creator><creator>KAWABATA, Yoshinari</creator><creator>YANAGIYA, Norimitsu</creator><creator>ICHIKAWA, Yoshikazu</creator><creator>MIURA, Akira B</creator><creator>MIURA, Ikuo</creator><general>Springer</general><general>Springer Nature B.V</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7RV</scope><scope>7T5</scope><scope>7T7</scope><scope>7TM</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FD</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>C1K</scope><scope>CCPQU</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>H94</scope><scope>K9.</scope><scope>KB0</scope><scope>M0S</scope><scope>M1P</scope><scope>NAPCQ</scope><scope>P64</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope></search><sort><creationdate>20010801</creationdate><title>A clinical analysis of 52 adult patients with hemophagocytic syndrome: The prognostic significance of the underlying diseases</title><author>TAKAHASHI, Naoto ; CHUBACHI, Akihiko ; KUME, Masaaki ; HATANO, Yoshiaki ; KOMATSUDA, Atsushi ; KAWABATA, Yoshinari ; YANAGIYA, Norimitsu ; ICHIKAWA, Yoshikazu ; MIURA, Akira B ; MIURA, Ikuo</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c422t-3c13f729e5399c98f1a4bc085fad2a54be5f43b014fd005c1fc642ac70a148063</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2001</creationdate><topic>Adult</topic><topic>Aged</topic><topic>Aged, 80 and over</topic><topic>Biological and medical sciences</topic><topic>Chronic conditions</topic><topic>Coagulation</topic><topic>Cyclophosphamide</topic><topic>Diseases</topic><topic>Disseminated intravascular coagulation</topic><topic>Epstein-Barr virus</topic><topic>Female</topic><topic>Fungal infections</topic><topic>Hematologic and hematopoietic diseases</topic><topic>Hemophagocytic syndrome</topic><topic>Histiocytosis, Non-Langerhans-Cell - diagnosis</topic><topic>Histiocytosis, Non-Langerhans-Cell - etiology</topic><topic>Histiocytosis, Non-Langerhans-Cell - mortality</topic><topic>Humans</topic><topic>Infection - complications</topic><topic>Infection - mortality</topic><topic>Infections</topic><topic>Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis</topic><topic>Lupus Erythematosus, Systemic - complications</topic><topic>Lupus Erythematosus, Systemic - mortality</topic><topic>Lymphoma</topic><topic>Lymphoma - complications</topic><topic>Lymphoma - drug therapy</topic><topic>Lymphoma - mortality</topic><topic>Male</topic><topic>Medical prognosis</topic><topic>Medical research</topic><topic>Medical sciences</topic><topic>Medical treatment</topic><topic>Middle Aged</topic><topic>Multiple organ dysfunction syndrome</topic><topic>Patients</topic><topic>Prednisolone</topic><topic>Prognosis</topic><topic>Retrospective Studies</topic><topic>Risk Factors</topic><topic>Survival Rate</topic><topic>Systemic lupus erythematosus</topic><topic>Vincristine</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>TAKAHASHI, Naoto</creatorcontrib><creatorcontrib>CHUBACHI, Akihiko</creatorcontrib><creatorcontrib>KUME, Masaaki</creatorcontrib><creatorcontrib>HATANO, Yoshiaki</creatorcontrib><creatorcontrib>KOMATSUDA, Atsushi</creatorcontrib><creatorcontrib>KAWABATA, Yoshinari</creatorcontrib><creatorcontrib>YANAGIYA, Norimitsu</creatorcontrib><creatorcontrib>ICHIKAWA, Yoshikazu</creatorcontrib><creatorcontrib>MIURA, Akira B</creatorcontrib><creatorcontrib>MIURA, Ikuo</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Nursing & Allied Health Database</collection><collection>Immunology Abstracts</collection><collection>Industrial and Applied Microbiology Abstracts (Microbiology A)</collection><collection>Nucleic Acids Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Technology Research Database</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central</collection><collection>Environmental Sciences and Pollution Management</collection><collection>ProQuest One Community College</collection><collection>Engineering Research Database</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Nursing & Allied Health Premium</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><jtitle>International journal of hematology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>TAKAHASHI, Naoto</au><au>CHUBACHI, Akihiko</au><au>KUME, Masaaki</au><au>HATANO, Yoshiaki</au><au>KOMATSUDA, Atsushi</au><au>KAWABATA, Yoshinari</au><au>YANAGIYA, Norimitsu</au><au>ICHIKAWA, Yoshikazu</au><au>MIURA, Akira B</au><au>MIURA, Ikuo</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>A clinical analysis of 52 adult patients with hemophagocytic syndrome: The prognostic significance of the underlying diseases</atitle><jtitle>International journal of hematology</jtitle><addtitle>Int J Hematol</addtitle><date>2001-08-01</date><risdate>2001</risdate><volume>74</volume><issue>2</issue><spage>209</spage><epage>213</epage><pages>209-213</pages><issn>0925-5710</issn><eissn>1865-3774</eissn><abstract>We retrospectively analyzed 52 adult patients with hemophagocytic syndrome (HPS). The underlying diseases were heterogeneous, including malignant lymphoma (lymphoma-associated hemophagocytic syndrome [LAHS]) in 26 patients, systemic lupus erythematosus in 3 patients, viral infections in 7 patients, and bacteria] or fungal infections in 6 patients. More than 83% of patients received prednisolone as an initial treatment. Multiple-agent chemotherapies (cyclophosphamide, doxorubicin, and vincristine) were administered to 96% of LAHS patients after a histopathological diagnosis of lymphoma. HPSs were controllable and remissions were achieved except for those patients with LAHS, fulminant Epstein-Barr virus-associated HPS, and an immunosuppressive state. Twenty-one (81%) of the LAHS patients had uncontrollable HPS and died of multiple organ failure and disseminated intravascular coagulation. The median survival time of LAHS patients was 83 days. In contrast, 3 (12%) of the other HPS patients died of multiple organ failure within 44 days.The clinical manifestations and the laboratory findings of LAHS and the other HPSs were too variable to establish the prognosis based only on the findings at the onset of HPS. The prognostic factors of adult HPS were found to be the underlying diseases, notably malignant lymphoma and infections, accompanied by the immunosuppressive state.</abstract><cop>Tokyo</cop><pub>Springer</pub><pmid>11594524</pmid><doi>10.1007/BF02982007</doi><tpages>5</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Adult Aged Aged, 80 and over Biological and medical sciences Chronic conditions Coagulation Cyclophosphamide Diseases Disseminated intravascular coagulation Epstein-Barr virus Female Fungal infections Hematologic and hematopoietic diseases Hemophagocytic syndrome Histiocytosis, Non-Langerhans-Cell - diagnosis Histiocytosis, Non-Langerhans-Cell - etiology Histiocytosis, Non-Langerhans-Cell - mortality Humans Infection - complications Infection - mortality Infections Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis Lupus Erythematosus, Systemic - complications Lupus Erythematosus, Systemic - mortality Lymphoma Lymphoma - complications Lymphoma - drug therapy Lymphoma - mortality Male Medical prognosis Medical research Medical sciences Medical treatment Middle Aged Multiple organ dysfunction syndrome Patients Prednisolone Prognosis Retrospective Studies Risk Factors Survival Rate Systemic lupus erythematosus Vincristine |
| title | A clinical analysis of 52 adult patients with hemophagocytic syndrome: The prognostic significance of the underlying diseases |
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