International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease

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dc.citation.issue12
dc.citation.volume129
dc.contributor.authorFajgenbaum, David C.
dc.contributor.authorUldrick, Thomas S.
dc.contributor.authorBagg, Adam
dc.contributor.authorFrank, Dale
dc.contributor.authorWu, David
dc.contributor.authorSrkalovic, Gordan
dc.contributor.authorSimpson, David
dc.contributor.authorLiu, Amy Y.
dc.contributor.authorMenke, David
dc.contributor.authorChandrakasan, Shanmuganathan
dc.contributor.authorLechowicz, Mary Jo
dc.contributor.authorWong, Raymond S. M.
dc.contributor.authorPierson, Sheila
dc.contributor.authorPaessler, Michele
dc.contributor.authorRossi, Jean-Francois
dc.contributor.authorIde, Makoto
dc.contributor.authorRuth, Jason
dc.contributor.authorCroglio, Michael
dc.contributor.authorSuarez, Alexander
dc.contributor.authorKrymskaya, Vera
dc.contributor.authorChadburn, Amy
dc.contributor.authorColleoni, Gisele [UNIFESP]
dc.contributor.authorNasta, Sunita
dc.contributor.authorJayanthan, Raj
dc.contributor.authorNabel, Christopher S.
dc.contributor.authorCasper, Corey
dc.contributor.authorDispenzieri, Angela
dc.contributor.authorFossa, Alexander
dc.contributor.authorKelleher, Dermot
dc.contributor.authorKurzrock, Razelle
dc.contributor.authorVoorhees, Peter
dc.contributor.authorDogan, Ahmet
dc.contributor.authorYoshizaki, Kazuyuki
dc.contributor.authorvan Rhee, Frits
dc.contributor.authorOksenhendler, Eric
dc.contributor.authorJaffe, Elaine S.
dc.contributor.authorElenitoba-Johnson, Kojo S. J.
dc.contributor.authorLim, Megan S.
dc.coverageWashington
dc.date.accessioned2020-07-17T14:02:38Z
dc.date.available2020-07-17T14:02:38Z
dc.date.issued2017
dc.description.abstractHuman herpesvirus-8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening disorder involving systemic inflammatory symptoms, polyclonal lymphoproliferation, cytopenias, and multiple organ system dysfunction caused by a cytokine storm often including interleukin-6. iMCD accounts for one third to one half of all cases ofMCDand can occur in individuals of any age. Accurate diagnosis is challenging, because no standard diagnostic criteria or diagnostic biomarkers currently exist, and there is significant overlap with malignant, autoimmune, and infectious disorders. An international working group comprising 34 pediatric and adult pathology and clinical experts in iMCD and related disorders from 8 countries, including 2 physicians that are also iMCD patients, was convened to establish iMCD diagnostic criteria. The working group reviewed data from 244 cases, met twice, and refined criteria over 15 months (June 2015 to September 2016). The proposed consensus criteria require both Major Criteria (characteristic lymph node histopathology and multicentric lymphadenopathy), at least 2 of 11Minor Criteria with at least 1 laboratory abnormality, and exclusion of infectious, malignant, and autoimmune disorders that can mimic iMCD. Characteristic histopathologic featuresmay include a constellation of regressed or hyperplastic germinal centers, follicular dendritic cell prominence, hypervascularization, and polytypic plasmacytosis. Laboratory and clinical Minor Criteria include elevatedC-reactive protein or erythrocyte sedimentation rate, anemia, thrombocytopenia or thrombocytosis, hypoalbuminemia, renal dysfunction or proteinuria, polyclonal hypergammaglobulinemia, constitutional symptoms, hepatosplenomegaly, effusions or edema, eruptive cherry hemangiomatosis or violaceous papules, and lymphocytic interstitial pneumonitis. iMCD consensus diagnostic criteria will facilitate consistent diagnosis, appropriate treatment, and collaborative research.en
dc.description.affiliationUniv Pennsylvania, Perelman Sch Med, Div Translat Med & Human Genet, Philadelphia, PA USA
dc.description.affiliationNatl Canc Inst, Natl Inst Hlth, Bethesda, MD USA
dc.description.affiliationUniv Pennsylvania, Perelman Sch Med, Dept Pathol & Lab Med, Philadelphia, PA USA
dc.description.affiliationUniv Washington, Dept Lab Med, Seattle, WA USA
dc.description.affiliationEdward Sparrow Hosp Assoc, Sparrow Canc Ctr, Lansing, MI USA
dc.description.affiliationNorth Shore Hosp, Div Haematol, Auckland, New Zealand
dc.description.affiliationMayo Clin, Dept Med, Div Hematol, Rochester, MN USA
dc.description.affiliationMayo Clin, Dept Lab Med & Pathol, Rochester, MN USA
dc.description.affiliationEmory Univ Sch Med, Dept Med, Atlanta, GA USA
dc.description.affiliationChinese Univ Hong Kong, Prince Wales Hosp, Sir K Pao Ctr Canc, Hong Kong, Peoples R China
dc.description.affiliationChinese Univ Hong Kong, Dept Med Therapeut, Hong Kong, Peoples R China
dc.description.affiliationChildrens Hosp Philadelphia, Dept Pathol, Philadelphia, PA USA
dc.description.affiliationHop St Eloi Univ Hosp, Dept Hematol, Montpellier, France
dc.description.affiliationTakamatsu Red Cross Hosp, Dept Hematol, Takamatsu, Kagawa, Japan
dc.description.affiliationDana Farber Canc Inst, Harvard Med Sch, Dept Med Oncol, Boston, MA USA
dc.description.affiliationSUNY Stony Brook, Sch Med, Stony Brook, NY USA
dc.description.affiliationUniv Penn, Perelman Sch Med, Div Pulm Allergy & Crit Care, Philadelphia, PA USA
dc.description.affiliationWeill Cornell Med Coll, Dept Pathol, New York, NY USA
dc.description.affiliationUniv Fed Sao Paulo, Dept Oncol Clin Expt, Sao Paulo, Brazil
dc.description.affiliationUniv Pennsylvania, Perelman Sch Med, Div Hematol Oncol, Philadelphia, PA USA
dc.description.affiliationTexas Childrens Hosp, Dept Pediat, Houston, TX USA
dc.description.affiliationBrigham & Womens Hosp, Dept Med, Boston, MA USA
dc.description.affiliationFred Hutchinson Canc Ctr, Vaccine & Infect Dis Div, Seattle, WA USA
dc.description.affiliationMayo Clin, Div Hematol Oncol, Rochester, MN USA
dc.description.affiliationOslo Univ Hosp, Norwegian Radium Hosp, Dept Oncol, Oslo, Norway
dc.description.affiliationUniv British Columbia, Fac Med, Vancouver, BC, Canada
dc.description.affiliationUC San Diego Moores Canc Ctr, Ctr Personalized Therapy, La Jolla, CA USA
dc.description.affiliationLevine Canc Inst, Dept Hematol Oncol & Blood Disorders, Carolinas HealthCare Syst, Charlotte, NC USA
dc.description.affiliationMem Sloan Kettering Canc Ctr, Dept Pathol, Lab Med, New York, NY USA
dc.description.affiliationOsaka Univ, Inst Sci & Ind Res, Dept Organ Fine Chem, Osaka, Japan
dc.description.affiliationUniv Arkansas Med Sci, Myeloma Inst, Little Rock, AR USA
dc.description.affiliationHop St Louis, Dept Clin Immunol, Paris, France
dc.description.affiliationUnifespUniv Fed Sao Paulo, Dept Oncol Clin Expt, Sao Paulo, Brazil
dc.description.sourceWeb of Science
dc.description.sponsorshipCDCN
dc.description.sponsorshipPenn Orphan Disease Center
dc.description.sponsorshipIntramural Research Program of the National Institutes of Health
dc.format.extent1646-1657
dc.identifierhttp://dx.doi.org/10.1182/blood-2016-10-746933
dc.identifier.citationBlood. Washington, v. 129, n. 12, p. 1646-1657, 2017.
dc.identifier.doi10.1182/blood-2016-10-746933
dc.identifier.issn0006-4971
dc.identifier.urihttps://repositorio.unifesp.br/handle/11600/54914
dc.identifier.wosWOS:000397297300015
dc.language.isoeng
dc.publisherAmer Soc Hematology
dc.relation.ispartofBlood
dc.rightsinfo:eu-repo/semantics/openAccess
dc.titleInternational, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman diseaseen
dc.typeinfo:eu-repo/semantics/article
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