International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease

Frits van Rhee, Peter Voorhees, Angela Dispenzieri, Alexander Fosså, Gordan Srkalovic, Makoto Ide, Nikhil Munshi, Stephen Schey, Matthew Streetly, Sheila K. Pierson, Helen L. Partridge, Sudipto Mukherjee, Dustin Shilling, Katie Stone, Amy Greenway, Jason Ruth, Mary Jo Lechowicz, Shanmuganathan Chandrakasan, Raj Jayanthan, Elaine S. JaffeHeather Leitch, Naveen Pemmaraju, Amy Chadburn, Megan S. Lim, Kojo S. Elenitoba-Johnson, Vera Krymskaya, Aaron Goodman, Christian Hoffmann, Pier Luigi Zinzani, Simone Ferrero, Louis Terriou, Yasuharu Sato, David Simpson, Raymond Wong, Jean Francois Rossi, Sunita Nasta, Kazuyuki Yoshizaki, Razelle Kurzrock, Thomas S. Uldrick, Corey Casper, Eric Oksenhendler, David C. Fajgenbaum

Research output: Contribution to journalReview article

13 Citations (Scopus)

Abstract

Castleman disease (CD) describes a group of heterogeneous hematologic disorders with characteristic histopathological features. CD can present with unicentric or multicentric (MCD) regions of lymph node enlargement. Some cases of MCD are caused by human herpesvirus-8 (HHV-8), whereas others are HHV-8-negative/idiopathic (iMCD). Treatment of iMCD is challenging, and outcomes can be poor because no uniform treatment guidelines exist, few systematic studies have been conducted, and no agreed upon response criteria have been described. The purpose of this paper is to establish consensus, evidence-based treatment guidelines based on the severity of iMCD to improve outcomes. An international Working Group of 42 experts from 10 countries was convened by the Castleman Disease Collaborative Network to establish consensus guidelines for the management of iMCD based on published literature, review of treatment effectiveness for 344 cases, and expert opinion. The anti-interleukin-6 monoclonal antibody siltuximab (or tocilizumab, if siltuximab is not available) with or without corticosteroids is the preferred first-line therapy for iMCD. In the most severe cases, adjuvant combination chemotherapy is recommended. Additional agents are recommended, tailored by disease severity, as second- and third-line therapies for treatment failures. Response criteria were formulated to facilitate the evaluation of treatment failure or success. These guidelines should help treating physicians to stratify patients based on disease severity in order to select the best available therapeutic option. An international registry for patients with CD (ACCELERATE, #NCT02817997) was established in October 2016 to collect patient outcomes to increase the evidence base for selection of therapies in the future.

Original languageEnglish (US)
Pages (from-to)2115-2124
Number of pages10
JournalBlood
Volume132
Issue number20
DOIs
StatePublished - Nov 15 2018

Fingerprint

Giant Lymph Node Hyperplasia
Guidelines
Human Herpesvirus 8
Treatment Failure
Therapeutics
Chemotherapy
Expert Testimony
Adjuvant Chemotherapy
Multi-centric Castleman's Disease
Combination Drug Therapy
Interleukin-6
Adrenal Cortex Hormones
Registries
Monoclonal Antibodies
Lymph Nodes
Physicians
siltuximab

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Cite this

van Rhee, F., Voorhees, P., Dispenzieri, A., Fosså, A., Srkalovic, G., Ide, M., ... Fajgenbaum, D. C. (2018). International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood, 132(20), 2115-2124. https://doi.org/10.1182/blood-2018-07-862334

International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. / van Rhee, Frits; Voorhees, Peter; Dispenzieri, Angela; Fosså, Alexander; Srkalovic, Gordan; Ide, Makoto; Munshi, Nikhil; Schey, Stephen; Streetly, Matthew; Pierson, Sheila K.; Partridge, Helen L.; Mukherjee, Sudipto; Shilling, Dustin; Stone, Katie; Greenway, Amy; Ruth, Jason; Lechowicz, Mary Jo; Chandrakasan, Shanmuganathan; Jayanthan, Raj; Jaffe, Elaine S.; Leitch, Heather; Pemmaraju, Naveen; Chadburn, Amy; Lim, Megan S.; Elenitoba-Johnson, Kojo S.; Krymskaya, Vera; Goodman, Aaron; Hoffmann, Christian; Zinzani, Pier Luigi; Ferrero, Simone; Terriou, Louis; Sato, Yasuharu; Simpson, David; Wong, Raymond; Rossi, Jean Francois; Nasta, Sunita; Yoshizaki, Kazuyuki; Kurzrock, Razelle; Uldrick, Thomas S.; Casper, Corey; Oksenhendler, Eric; Fajgenbaum, David C.

In: Blood, Vol. 132, No. 20, 15.11.2018, p. 2115-2124.

Research output: Contribution to journalReview article

van Rhee, F, Voorhees, P, Dispenzieri, A, Fosså, A, Srkalovic, G, Ide, M, Munshi, N, Schey, S, Streetly, M, Pierson, SK, Partridge, HL, Mukherjee, S, Shilling, D, Stone, K, Greenway, A, Ruth, J, Lechowicz, MJ, Chandrakasan, S, Jayanthan, R, Jaffe, ES, Leitch, H, Pemmaraju, N, Chadburn, A, Lim, MS, Elenitoba-Johnson, KS, Krymskaya, V, Goodman, A, Hoffmann, C, Zinzani, PL, Ferrero, S, Terriou, L, Sato, Y, Simpson, D, Wong, R, Rossi, JF, Nasta, S, Yoshizaki, K, Kurzrock, R, Uldrick, TS, Casper, C, Oksenhendler, E & Fajgenbaum, DC 2018, 'International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease', Blood, vol. 132, no. 20, pp. 2115-2124. https://doi.org/10.1182/blood-2018-07-862334
van Rhee, Frits ; Voorhees, Peter ; Dispenzieri, Angela ; Fosså, Alexander ; Srkalovic, Gordan ; Ide, Makoto ; Munshi, Nikhil ; Schey, Stephen ; Streetly, Matthew ; Pierson, Sheila K. ; Partridge, Helen L. ; Mukherjee, Sudipto ; Shilling, Dustin ; Stone, Katie ; Greenway, Amy ; Ruth, Jason ; Lechowicz, Mary Jo ; Chandrakasan, Shanmuganathan ; Jayanthan, Raj ; Jaffe, Elaine S. ; Leitch, Heather ; Pemmaraju, Naveen ; Chadburn, Amy ; Lim, Megan S. ; Elenitoba-Johnson, Kojo S. ; Krymskaya, Vera ; Goodman, Aaron ; Hoffmann, Christian ; Zinzani, Pier Luigi ; Ferrero, Simone ; Terriou, Louis ; Sato, Yasuharu ; Simpson, David ; Wong, Raymond ; Rossi, Jean Francois ; Nasta, Sunita ; Yoshizaki, Kazuyuki ; Kurzrock, Razelle ; Uldrick, Thomas S. ; Casper, Corey ; Oksenhendler, Eric ; Fajgenbaum, David C. / International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. In: Blood. 2018 ; Vol. 132, No. 20. pp. 2115-2124.
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abstract = "Castleman disease (CD) describes a group of heterogeneous hematologic disorders with characteristic histopathological features. CD can present with unicentric or multicentric (MCD) regions of lymph node enlargement. Some cases of MCD are caused by human herpesvirus-8 (HHV-8), whereas others are HHV-8-negative/idiopathic (iMCD). Treatment of iMCD is challenging, and outcomes can be poor because no uniform treatment guidelines exist, few systematic studies have been conducted, and no agreed upon response criteria have been described. The purpose of this paper is to establish consensus, evidence-based treatment guidelines based on the severity of iMCD to improve outcomes. An international Working Group of 42 experts from 10 countries was convened by the Castleman Disease Collaborative Network to establish consensus guidelines for the management of iMCD based on published literature, review of treatment effectiveness for 344 cases, and expert opinion. The anti-interleukin-6 monoclonal antibody siltuximab (or tocilizumab, if siltuximab is not available) with or without corticosteroids is the preferred first-line therapy for iMCD. In the most severe cases, adjuvant combination chemotherapy is recommended. Additional agents are recommended, tailored by disease severity, as second- and third-line therapies for treatment failures. Response criteria were formulated to facilitate the evaluation of treatment failure or success. These guidelines should help treating physicians to stratify patients based on disease severity in order to select the best available therapeutic option. An international registry for patients with CD (ACCELERATE, #NCT02817997) was established in October 2016 to collect patient outcomes to increase the evidence base for selection of therapies in the future.",
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AU - van Rhee, Frits

AU - Voorhees, Peter

AU - Dispenzieri, Angela

AU - Fosså, Alexander

AU - Srkalovic, Gordan

AU - Ide, Makoto

AU - Munshi, Nikhil

AU - Schey, Stephen

AU - Streetly, Matthew

AU - Pierson, Sheila K.

AU - Partridge, Helen L.

AU - Mukherjee, Sudipto

AU - Shilling, Dustin

AU - Stone, Katie

AU - Greenway, Amy

AU - Ruth, Jason

AU - Lechowicz, Mary Jo

AU - Chandrakasan, Shanmuganathan

AU - Jayanthan, Raj

AU - Jaffe, Elaine S.

AU - Leitch, Heather

AU - Pemmaraju, Naveen

AU - Chadburn, Amy

AU - Lim, Megan S.

AU - Elenitoba-Johnson, Kojo S.

AU - Krymskaya, Vera

AU - Goodman, Aaron

AU - Hoffmann, Christian

AU - Zinzani, Pier Luigi

AU - Ferrero, Simone

AU - Terriou, Louis

AU - Sato, Yasuharu

AU - Simpson, David

AU - Wong, Raymond

AU - Rossi, Jean Francois

AU - Nasta, Sunita

AU - Yoshizaki, Kazuyuki

AU - Kurzrock, Razelle

AU - Uldrick, Thomas S.

AU - Casper, Corey

AU - Oksenhendler, Eric

AU - Fajgenbaum, David C.

PY - 2018/11/15

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N2 - Castleman disease (CD) describes a group of heterogeneous hematologic disorders with characteristic histopathological features. CD can present with unicentric or multicentric (MCD) regions of lymph node enlargement. Some cases of MCD are caused by human herpesvirus-8 (HHV-8), whereas others are HHV-8-negative/idiopathic (iMCD). Treatment of iMCD is challenging, and outcomes can be poor because no uniform treatment guidelines exist, few systematic studies have been conducted, and no agreed upon response criteria have been described. The purpose of this paper is to establish consensus, evidence-based treatment guidelines based on the severity of iMCD to improve outcomes. An international Working Group of 42 experts from 10 countries was convened by the Castleman Disease Collaborative Network to establish consensus guidelines for the management of iMCD based on published literature, review of treatment effectiveness for 344 cases, and expert opinion. The anti-interleukin-6 monoclonal antibody siltuximab (or tocilizumab, if siltuximab is not available) with or without corticosteroids is the preferred first-line therapy for iMCD. In the most severe cases, adjuvant combination chemotherapy is recommended. Additional agents are recommended, tailored by disease severity, as second- and third-line therapies for treatment failures. Response criteria were formulated to facilitate the evaluation of treatment failure or success. These guidelines should help treating physicians to stratify patients based on disease severity in order to select the best available therapeutic option. An international registry for patients with CD (ACCELERATE, #NCT02817997) was established in October 2016 to collect patient outcomes to increase the evidence base for selection of therapies in the future.

AB - Castleman disease (CD) describes a group of heterogeneous hematologic disorders with characteristic histopathological features. CD can present with unicentric or multicentric (MCD) regions of lymph node enlargement. Some cases of MCD are caused by human herpesvirus-8 (HHV-8), whereas others are HHV-8-negative/idiopathic (iMCD). Treatment of iMCD is challenging, and outcomes can be poor because no uniform treatment guidelines exist, few systematic studies have been conducted, and no agreed upon response criteria have been described. The purpose of this paper is to establish consensus, evidence-based treatment guidelines based on the severity of iMCD to improve outcomes. An international Working Group of 42 experts from 10 countries was convened by the Castleman Disease Collaborative Network to establish consensus guidelines for the management of iMCD based on published literature, review of treatment effectiveness for 344 cases, and expert opinion. The anti-interleukin-6 monoclonal antibody siltuximab (or tocilizumab, if siltuximab is not available) with or without corticosteroids is the preferred first-line therapy for iMCD. In the most severe cases, adjuvant combination chemotherapy is recommended. Additional agents are recommended, tailored by disease severity, as second- and third-line therapies for treatment failures. Response criteria were formulated to facilitate the evaluation of treatment failure or success. These guidelines should help treating physicians to stratify patients based on disease severity in order to select the best available therapeutic option. An international registry for patients with CD (ACCELERATE, #NCT02817997) was established in October 2016 to collect patient outcomes to increase the evidence base for selection of therapies in the future.

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