Frits van Rhee, Myeloma Center, University of Arkansas for Medical Sciences, Little Rock, AR, United States
Eric Oksenhendler, Department of Clinical Immunology, Hospital Saint-Louis, Paris, France
Gordan Srkalovic, Sparrow Cancer Center, Edward W. Sparrow Hospital Association, Lansing, MI, United States
Peter Voorhees, Department of Hematologic Oncology and Blood Disorders, Levine Cancer Institute, Atrium Health, Charlotte, NC, United States
Megan Lim, Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States
Angela Dispenzieri, Division of Hematology, Mayo Clinic, Rochester, MN, United States
Makoto Ide, Department of Hematology, Takamatsu Red Cross Hospital, Takamatsu, Japan
Sophia Parente, Division of Translational Medicine and Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States
Stephen Schey, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom
Matthew Streetly, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom
Raymond Wong, Sir Y.K. Pao Centre for Cancer, Department of Medicine and Therapeutics, Prince of Wales Hospital, Chinese University of Hong Kong, Hong Kong
David Wu, Department of Laboratory Medicine, University of Washington, Seattle, WA, United States
Ivan Maillard, Divison of Hematology/Oncology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States
Joshua Brandstadter, Divison of Hematology/Oncology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States
Nikhil Munshi, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, United States
Wilbur Bowne, Department of General Surgery and Surgical Oncology, Thomas Jefferson University, Philadelphia, PA, United StatesFollow
Kojo S Elenitoba-Johnson, Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States
Alexander Fössa, Department of Oncology, Oslo University Hospital, Norwegian Radium Hospital, Oslo, Norway
Mary Jo Lechowicz, Department of Hematology and Medical Oncology, Emory University, Atlanta, GA, United States
Shanmuganathan Chandrakasan, Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Emory University, Atlanta, GA, United States
Sheila K Pierson, Division of Translational Medicine and Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States
Amy Greenway, Myeloma Center, University of Arkansas for Medical Sciences, Little Rock, AR, United States
Sunita Nasta, Divison of Hematology/Oncology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States
Kazuyuki Yoshizaki, Department of Organic Fine Chemicals, Institute of Scientific and Industrial Research, Osaka University, Osaka, Japan
Razelle Kurzrock, Center for Personalized Therapy and Clinical Trials Office, Moores Cancer Center, UC San Diego, San Diego, CA, United States
Thomas S Uldrick, Fred Hutchinson Cancer Research Center and Medical Oncology, University of Washington, Seattle, WA, United States
Corey Casper, Infectious Disease Research Institute, Department of Medicine and Global Health, University of Washington, Seattle, WA, United States
Amy Chadburn, Department of Pathology and Laboratory Medicine, Weill Cornell Medical College, New York, NY
David C Fajgenbaum, Division of Translational Medicine and Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA

Document Type

Article

Publication Date

12-8-2020

Comments

This research was originally published in Blood Advances. van Rhee F, Oksenhendler E, Srkalovic G, Voorhees P, Lim M, Dispenzieri A, Ide M, Parente S, Schey S, Streetly M, Wong R, Wu D, Maillard I, Brandstadter J, Munshi N, Bowne W, Elenitoba-Johnson KS, Fössa A, Lechowicz MJ, Chandrakasan S, Pierson SK, Greenway A, Nasta S, Yoshizaki K, Kurzrock R, Uldrick TS, Casper C, Chadburn A, Fajgenbaum DC. International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease. Blood Adv. 2020;Vol 4: Pages 6039-6050. © 2020 The American Society of Hematology

DOI: https://doi.org/10.1182/bloodadvances.2020003334

Abstract

Castleman disease (CD) includes a group of rare and heterogeneous disorders with characteristic lymph node histopathological abnormalities. CD can occur in a single lymph node station, which is referred to as unicentric CD (UCD). CD can also involve multicentric lymphadenopathy and inflammatory symptoms (multicentric CD [MCD]). MCD includes human herpesvirus-8 (HHV-8)-associated MCD, POEMS-associated MCD, and HHV-8-/idiopathic MCD (iMCD). The first-ever diagnostic and treatment guidelines were recently developed for iMCD by an international expert consortium convened by the Castleman Disease Collaborative Network (CDCN). The focus of this report is to establish similar guidelines for the management of UCD. To this purpose, an international working group of 42 experts from 10 countries was convened to establish consensus recommendations based on review of treatment in published cases of UCD, the CDCN ACCELERATE registry, and expert opinion. Complete surgical resection is often curative and is therefore the preferred first-line therapy, if possible. The management of unresectable UCD is more challenging. Existing evidence supports that asymptomatic unresectable UCD may be observed. The anti-interleukin-6 monoclonal antibody siltuximab should be considered for unresectable UCD patients with an inflammatory syndrome. Unresectable UCD that is symptomatic as a result of compression of vital neighboring structures may be rendered amenable to resection by medical therapy (eg, rituximab, steroids), radiotherapy, or embolization. Further research is needed in UCD patients with persisting constitutional symptoms despite complete excision and normal laboratory markers. We hope that these guidelines will improve outcomes in UCD and help treating physicians decide the best therapeutic approach for their patients.

PubMed ID

33284946

Language

English

Download

Share

COinS