TY - JOUR
T1 - International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease
AU - Fajgenbaum, David C.
AU - Uldrick, Thomas S.
AU - Bagg, Adam
AU - Frank, Dale
AU - Wu, David
AU - Srkalovic, Gordan
AU - Simpson, David
AU - Liu, Amy Y.
AU - Menke, David
AU - Chandrakasan, Shanmuganathan
AU - Lechowicz, Mary Jo
AU - Wong, Raymond S.M.
AU - Pierson, Sheila
AU - Paessler, Michele
AU - Rossi, Jean François
AU - Ide, Makoto
AU - Ruth, Jason
AU - Croglio, Michael
AU - Suarez, Alexander
AU - Krymskaya, Vera
AU - Chadburn, Amy
AU - Colleoni, Gisele
AU - Nasta, Sunita
AU - Jayanthan, Raj
AU - Nabel, Christopher S.
AU - Casper, Corey
AU - Dispenzieri, Angela
AU - Fosså, Alexander
AU - Kelleher, Dermot
AU - Kurzrock, Razelle
AU - Voorhees, Peter
AU - Dogan, Ahmet
AU - Yoshizaki, Kazuyuki
AU - Van Rhee, Frits
AU - Oksenhendler, Eric
AU - Jaffe, Elaine S.
AU - Elenitoba-Johnson, Kojo S.J.
AU - Lim, Megan S.
N1 - Publisher Copyright:
© 2011 by The American Society of Hematology.
PY - 2017/3/23
Y1 - 2017/3/23
N2 - Human 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.
AB - Human 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.
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U2 - 10.1182/blood-2016-10-746933
DO - 10.1182/blood-2016-10-746933
M3 - Article
C2 - 28087540
AN - SCOPUS:85016255188
SN - 0006-4971
VL - 129
SP - 1646
EP - 1657
JO - Blood
JF - Blood
IS - 12
ER -