A case of extranodal NK/T-cell lymphoma, nasal type mimicking typical manifestations of adult-onset Still’s disease (AOSD) with hemophagocytic syndrome: diagnostic consideration between malignant lymphoma without lymphadenopathy and AOSD
A 25-year-old Japanese man was suffering from high fever, sore throat, arthralgia, and macular salmon-pink eruption. The superficial lymph node was not palpable, and computed tomographic scans from the neck to pelvis demonstrated hepatosplenomegaly without apparent lymphadenopathy. Therefore, the po...
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Veröffentlicht in: | Modern rheumatology 2009-12, Vol.19 (6), p.675-680 |
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creator | Kato, Takashi Tanabe, Juichi Kanemoto, Motoko Kobayashi, Chiharu Morita, Sho Karahashi, Taro |
description | A 25-year-old Japanese man was suffering from high fever, sore throat, arthralgia, and macular salmon-pink eruption. The superficial lymph node was not palpable, and computed tomographic scans from the neck to pelvis demonstrated hepatosplenomegaly without apparent lymphadenopathy. Therefore, the possibility of malignant lymphoma was considered to be extremely low. Serology for Epstein Barr virus (EBV) and cytomegalovirus showed a postinfectious state, and blood culture was negative. Serum rheumatoid factor and antinuclear antibody were negative. Leukocytopenia (2.4 × 10
3
/μl) was observed, and thus a diagnosis of adult-onset Still’s disease (AOSD) with hemophagocytic syndrome (HPS) was made. Fifty-five milligrams of prednisolone daily improved his symptoms and leukocytopenia promptly, but high fever with severe and progressive thrombocytopenia occurred 12 days later. Bone marrow aspiration revealed the presence of lymphoma cells and hemophagocytosis, and the CD45 gating analysis showed expanding population of CD2
+
, CD3
−
, and CD56
+
cells. Further, mucosal ulceration in the nasal cavity was detected. Therefore, a diagnosis of extranodal natural killer (NK)/T-cell lymphoma, nasal type, concomitant with HPS was made, and treatment with dexamethasone, etoposide, ifosfamide, carboplatin (DeVIC) regimen ameliorated his symptoms and platelet transfusion dependency. Later, a high titer of serum EBV-DNA was detected, which supported the diagnosis. Diagnosing AOSD, extranodal presentation of malignant lymphoma such as extranodal NK/T-cell lymphoma, nasal type, should be carefully considered. |
doi_str_mv | 10.1007/s10165-009-0204-y |
format | Article |
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3
/μl) was observed, and thus a diagnosis of adult-onset Still’s disease (AOSD) with hemophagocytic syndrome (HPS) was made. Fifty-five milligrams of prednisolone daily improved his symptoms and leukocytopenia promptly, but high fever with severe and progressive thrombocytopenia occurred 12 days later. Bone marrow aspiration revealed the presence of lymphoma cells and hemophagocytosis, and the CD45 gating analysis showed expanding population of CD2
+
, CD3
−
, and CD56
+
cells. Further, mucosal ulceration in the nasal cavity was detected. Therefore, a diagnosis of extranodal natural killer (NK)/T-cell lymphoma, nasal type, concomitant with HPS was made, and treatment with dexamethasone, etoposide, ifosfamide, carboplatin (DeVIC) regimen ameliorated his symptoms and platelet transfusion dependency. Later, a high titer of serum EBV-DNA was detected, which supported the diagnosis. Diagnosing AOSD, extranodal presentation of malignant lymphoma such as extranodal NK/T-cell lymphoma, nasal type, should be carefully considered.</description><identifier>ISSN: 1439-7595</identifier><identifier>EISSN: 1439-7609</identifier><identifier>DOI: 10.1007/s10165-009-0204-y</identifier><identifier>PMID: 19609486</identifier><language>eng</language><publisher>Japan: Springer Japan</publisher><subject>Adult ; Antineoplastic Combined Chemotherapy Protocols - therapeutic use ; Biomarkers, Tumor ; Carboplatin - therapeutic use ; Case Report ; Case studies ; Cytomegalovirus ; Dexamethasone - therapeutic use ; Diagnosis, Differential ; Disease Progression ; Epstein-Barr virus ; Etoposide - therapeutic use ; Herpesvirus 4, Human - isolation & purification ; Humans ; Ifosfamide - therapeutic use ; Lymph Nodes - diagnostic imaging ; Lymph Nodes - pathology ; Lymphatic Diseases - diagnosis ; Lymphoma ; Lymphoma, Extranodal NK-T-Cell - complications ; Lymphoma, Extranodal NK-T-Cell - diagnosis ; Lymphoma, Extranodal NK-T-Cell - drug therapy ; Male ; Medical diagnosis ; Medicine ; Medicine & Public Health ; Nose ; Orthopedics ; Radiography ; Rheumatology ; Salmonidae ; Splenomegaly - drug therapy ; Splenomegaly - etiology ; Still's Disease, Adult-Onset - diagnosis ; Treatment Outcome ; Viruses</subject><ispartof>Modern rheumatology, 2009-12, Vol.19 (6), p.675-680</ispartof><rights>Japan College of Rheumatology 2009</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c359t-f5a8e6c7e846cae7653e10d9edb2f0782ed2ee582fcef1c0ab2012bb1d4fe78f3</citedby><cites>FETCH-LOGICAL-c359t-f5a8e6c7e846cae7653e10d9edb2f0782ed2ee582fcef1c0ab2012bb1d4fe78f3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27923,27924</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/19609486$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Kato, Takashi</creatorcontrib><creatorcontrib>Tanabe, Juichi</creatorcontrib><creatorcontrib>Kanemoto, Motoko</creatorcontrib><creatorcontrib>Kobayashi, Chiharu</creatorcontrib><creatorcontrib>Morita, Sho</creatorcontrib><creatorcontrib>Karahashi, Taro</creatorcontrib><title>A case of extranodal NK/T-cell lymphoma, nasal type mimicking typical manifestations of adult-onset Still’s disease (AOSD) with hemophagocytic syndrome: diagnostic consideration between malignant lymphoma without lymphadenopathy and AOSD</title><title>Modern rheumatology</title><addtitle>Mod Rheumatol</addtitle><addtitle>Mod Rheumatol</addtitle><description>A 25-year-old Japanese man was suffering from high fever, sore throat, arthralgia, and macular salmon-pink eruption. The superficial lymph node was not palpable, and computed tomographic scans from the neck to pelvis demonstrated hepatosplenomegaly without apparent lymphadenopathy. Therefore, the possibility of malignant lymphoma was considered to be extremely low. Serology for Epstein Barr virus (EBV) and cytomegalovirus showed a postinfectious state, and blood culture was negative. Serum rheumatoid factor and antinuclear antibody were negative. Leukocytopenia (2.4 × 10
3
/μl) was observed, and thus a diagnosis of adult-onset Still’s disease (AOSD) with hemophagocytic syndrome (HPS) was made. Fifty-five milligrams of prednisolone daily improved his symptoms and leukocytopenia promptly, but high fever with severe and progressive thrombocytopenia occurred 12 days later. Bone marrow aspiration revealed the presence of lymphoma cells and hemophagocytosis, and the CD45 gating analysis showed expanding population of CD2
+
, CD3
−
, and CD56
+
cells. Further, mucosal ulceration in the nasal cavity was detected. Therefore, a diagnosis of extranodal natural killer (NK)/T-cell lymphoma, nasal type, concomitant with HPS was made, and treatment with dexamethasone, etoposide, ifosfamide, carboplatin (DeVIC) regimen ameliorated his symptoms and platelet transfusion dependency. Later, a high titer of serum EBV-DNA was detected, which supported the diagnosis. Diagnosing AOSD, extranodal presentation of malignant lymphoma such as extranodal NK/T-cell lymphoma, nasal type, should be carefully considered.</description><subject>Adult</subject><subject>Antineoplastic Combined Chemotherapy Protocols - therapeutic use</subject><subject>Biomarkers, Tumor</subject><subject>Carboplatin - therapeutic use</subject><subject>Case Report</subject><subject>Case studies</subject><subject>Cytomegalovirus</subject><subject>Dexamethasone - therapeutic use</subject><subject>Diagnosis, Differential</subject><subject>Disease Progression</subject><subject>Epstein-Barr virus</subject><subject>Etoposide - therapeutic use</subject><subject>Herpesvirus 4, Human - isolation & purification</subject><subject>Humans</subject><subject>Ifosfamide - therapeutic use</subject><subject>Lymph Nodes - diagnostic imaging</subject><subject>Lymph Nodes - pathology</subject><subject>Lymphatic Diseases - diagnosis</subject><subject>Lymphoma</subject><subject>Lymphoma, Extranodal NK-T-Cell - complications</subject><subject>Lymphoma, Extranodal NK-T-Cell - diagnosis</subject><subject>Lymphoma, Extranodal NK-T-Cell - drug therapy</subject><subject>Male</subject><subject>Medical diagnosis</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Nose</subject><subject>Orthopedics</subject><subject>Radiography</subject><subject>Rheumatology</subject><subject>Salmonidae</subject><subject>Splenomegaly - drug therapy</subject><subject>Splenomegaly - etiology</subject><subject>Still's Disease, Adult-Onset - diagnosis</subject><subject>Treatment Outcome</subject><subject>Viruses</subject><issn>1439-7595</issn><issn>1439-7609</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2009</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>8G5</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><sourceid>GUQSH</sourceid><sourceid>M2O</sourceid><recordid>eNp9kstu1TAQhiMEoqXwAGyQxYKLRKjtXJywOypXUdFFy9qa2JMcl8QOsaOSHa_B6_ECvAJOzylILFjZM_Ppn_H4T5KHjL5klIpjzygri5TSOqWc5ulyKzlkeVanoqT17Zt7URcHyT3vLynNirqq7yYHrI5AXpWHya8NUeCRuJbgtzCBdRp68unj8UWqsO9Jvwzj1g3wgljwsRKWEclgBqO-GNutoVExPYA1LfoAwTjrVzXQcx_SGGAg58H0_c_vPzzRxuPa7tnm7Pz1c3JlwpZscXDjFjqnlmAU8YvVkxvwVYShs86vSRWFjMbpWp80GK4Qbezam86CDX_GvFZ08z4BGq0bIWwXAlaTtef95E4LvccH-_Mo-fz2zcXJ-_T07N2Hk81pquKOQtoWUGGpBFZ5qQBFWWTIqK5RN7ylouKoOWJR8VZhyxSFhlPGm4bpvEVRtdlR8nSnO07u6xw3Iwfj142CRTd7KbKciTLnWSSf_JfkjBdlzVgEH_8DXrp5svEVkmdc5KUQq9qjPTQ3A2o5TmaAaZE3Px4BvgN8LNkOp78qjMrVVnJnKxltJVdbySX7Db1qxa0</recordid><startdate>20091201</startdate><enddate>20091201</enddate><creator>Kato, Takashi</creator><creator>Tanabe, Juichi</creator><creator>Kanemoto, Motoko</creator><creator>Kobayashi, Chiharu</creator><creator>Morita, Sho</creator><creator>Karahashi, Taro</creator><general>Springer Japan</general><general>Informa Healthcare</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>3V.</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>8G5</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>GUQSH</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>M2O</scope><scope>MBDVC</scope><scope>PADUT</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>Q9U</scope><scope>7T5</scope><scope>7U9</scope><scope>H94</scope><scope>7X8</scope></search><sort><creationdate>20091201</creationdate><title>A case of extranodal NK/T-cell lymphoma, nasal type mimicking typical manifestations of adult-onset Still’s disease (AOSD) with hemophagocytic syndrome: diagnostic consideration between malignant lymphoma without lymphadenopathy and AOSD</title><author>Kato, Takashi ; Tanabe, Juichi ; Kanemoto, Motoko ; Kobayashi, Chiharu ; Morita, Sho ; Karahashi, Taro</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c359t-f5a8e6c7e846cae7653e10d9edb2f0782ed2ee582fcef1c0ab2012bb1d4fe78f3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2009</creationdate><topic>Adult</topic><topic>Antineoplastic Combined Chemotherapy Protocols - therapeutic use</topic><topic>Biomarkers, Tumor</topic><topic>Carboplatin - therapeutic use</topic><topic>Case Report</topic><topic>Case studies</topic><topic>Cytomegalovirus</topic><topic>Dexamethasone - therapeutic use</topic><topic>Diagnosis, Differential</topic><topic>Disease Progression</topic><topic>Epstein-Barr virus</topic><topic>Etoposide - therapeutic use</topic><topic>Herpesvirus 4, Human - isolation & purification</topic><topic>Humans</topic><topic>Ifosfamide - therapeutic use</topic><topic>Lymph Nodes - diagnostic imaging</topic><topic>Lymph Nodes - pathology</topic><topic>Lymphatic Diseases - diagnosis</topic><topic>Lymphoma</topic><topic>Lymphoma, Extranodal NK-T-Cell - complications</topic><topic>Lymphoma, Extranodal NK-T-Cell - diagnosis</topic><topic>Lymphoma, Extranodal NK-T-Cell - drug therapy</topic><topic>Male</topic><topic>Medical diagnosis</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Nose</topic><topic>Orthopedics</topic><topic>Radiography</topic><topic>Rheumatology</topic><topic>Salmonidae</topic><topic>Splenomegaly - drug therapy</topic><topic>Splenomegaly - etiology</topic><topic>Still's Disease, Adult-Onset - diagnosis</topic><topic>Treatment Outcome</topic><topic>Viruses</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Kato, Takashi</creatorcontrib><creatorcontrib>Tanabe, Juichi</creatorcontrib><creatorcontrib>Kanemoto, Motoko</creatorcontrib><creatorcontrib>Kobayashi, Chiharu</creatorcontrib><creatorcontrib>Morita, Sho</creatorcontrib><creatorcontrib>Karahashi, Taro</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>ProQuest Central (Corporate)</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>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>Research Library (Alumni Edition)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>Research Library Prep</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Research Library</collection><collection>Research Library (Corporate)</collection><collection>Research Library China</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>ProQuest Central Basic</collection><collection>Immunology Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Modern rheumatology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Kato, Takashi</au><au>Tanabe, Juichi</au><au>Kanemoto, Motoko</au><au>Kobayashi, Chiharu</au><au>Morita, Sho</au><au>Karahashi, Taro</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>A case of extranodal NK/T-cell lymphoma, nasal type mimicking typical manifestations of adult-onset Still’s disease (AOSD) with hemophagocytic syndrome: diagnostic consideration between malignant lymphoma without lymphadenopathy and AOSD</atitle><jtitle>Modern rheumatology</jtitle><stitle>Mod Rheumatol</stitle><addtitle>Mod Rheumatol</addtitle><date>2009-12-01</date><risdate>2009</risdate><volume>19</volume><issue>6</issue><spage>675</spage><epage>680</epage><pages>675-680</pages><issn>1439-7595</issn><eissn>1439-7609</eissn><abstract>A 25-year-old Japanese man was suffering from high fever, sore throat, arthralgia, and macular salmon-pink eruption. The superficial lymph node was not palpable, and computed tomographic scans from the neck to pelvis demonstrated hepatosplenomegaly without apparent lymphadenopathy. Therefore, the possibility of malignant lymphoma was considered to be extremely low. Serology for Epstein Barr virus (EBV) and cytomegalovirus showed a postinfectious state, and blood culture was negative. Serum rheumatoid factor and antinuclear antibody were negative. Leukocytopenia (2.4 × 10
3
/μl) was observed, and thus a diagnosis of adult-onset Still’s disease (AOSD) with hemophagocytic syndrome (HPS) was made. Fifty-five milligrams of prednisolone daily improved his symptoms and leukocytopenia promptly, but high fever with severe and progressive thrombocytopenia occurred 12 days later. Bone marrow aspiration revealed the presence of lymphoma cells and hemophagocytosis, and the CD45 gating analysis showed expanding population of CD2
+
, CD3
−
, and CD56
+
cells. Further, mucosal ulceration in the nasal cavity was detected. Therefore, a diagnosis of extranodal natural killer (NK)/T-cell lymphoma, nasal type, concomitant with HPS was made, and treatment with dexamethasone, etoposide, ifosfamide, carboplatin (DeVIC) regimen ameliorated his symptoms and platelet transfusion dependency. Later, a high titer of serum EBV-DNA was detected, which supported the diagnosis. Diagnosing AOSD, extranodal presentation of malignant lymphoma such as extranodal NK/T-cell lymphoma, nasal type, should be carefully considered.</abstract><cop>Japan</cop><pub>Springer Japan</pub><pmid>19609486</pmid><doi>10.1007/s10165-009-0204-y</doi><tpages>6</tpages></addata></record> |
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subjects | Adult Antineoplastic Combined Chemotherapy Protocols - therapeutic use Biomarkers, Tumor Carboplatin - therapeutic use Case Report Case studies Cytomegalovirus Dexamethasone - therapeutic use Diagnosis, Differential Disease Progression Epstein-Barr virus Etoposide - therapeutic use Herpesvirus 4, Human - isolation & purification Humans Ifosfamide - therapeutic use Lymph Nodes - diagnostic imaging Lymph Nodes - pathology Lymphatic Diseases - diagnosis Lymphoma Lymphoma, Extranodal NK-T-Cell - complications Lymphoma, Extranodal NK-T-Cell - diagnosis Lymphoma, Extranodal NK-T-Cell - drug therapy Male Medical diagnosis Medicine Medicine & Public Health Nose Orthopedics Radiography Rheumatology Salmonidae Splenomegaly - drug therapy Splenomegaly - etiology Still's Disease, Adult-Onset - diagnosis Treatment Outcome Viruses |
title | A case of extranodal NK/T-cell lymphoma, nasal type mimicking typical manifestations of adult-onset Still’s disease (AOSD) with hemophagocytic syndrome: diagnostic consideration between malignant lymphoma without lymphadenopathy and AOSD |
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