International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease

被引：162

作者：

van Rhee, Frits ^{[1
]}

Oksenhendler, Eric ^{[2
]}

Srkalovic, Gordan ^{[3
]}

Voorhees, Peter ^{[4
]}

Lim, Megan ^{[5
]}

Dispenzieri, Angela ^{[6
]}

Ide, Makoto ^{[7
]}

Parente, Sophia ^{[8
]}

Schey, Stephen ^{[9
]}

Streetly, Matthew ^{[9
]}

Wong, Raymond ^{[10
]}

Wu, David ^{[11
]}

Maillard, Ivan ^{[12
]}

Brandstadter, Joshua ^{[12
]}

Munshi, Nikhil ^{[13
]}

Bowne, Wilbur ^{[14
]}

Elenitoba-Johnson, Kojo S. ^{[5
]}

Fossa, Alexander ^{[15
]}

Lechowicz, Mary Jo ^{[16
]}

Chandrakasan, Shanmuganathan ^{[17
]}

Pierson, Sheila K. ^{[8
]}

Greenway, Amy ^{[1
]}

Nasta, Sunita ^{[12
]}

Yoshizaki, Kazuyuki ^{[18
]}

Kurzrock, Razelle ^{[19
,20
]}

Uldrick, Thomas S. ^{[21
,22
]}

Casper, Corey ^{[23
]}

Chadburn, Amy ^{[24
]}

Fajgenbaum, David C. ^{[8
]}

机构：

[1] Univ Arkansas Med Sci, Myeloma Ctr, 4301 W Markham 816, Little Rock, AR 72205 USA

[2] Hosp St Louis, Dept Clin Immunol, Paris, France

[3] Edward W Sparrow Hosp Assoc, Sparrow Canc Ctr, Lansing, MI USA

[4] Atrium Hlth, Dept Hematol Oncol & Blood Disorders, Levine Canc Inst, Charlotte, NC USA

[5] Univ Penn, Perelman Sch Med, Dept Pathol & Lab Med, Philadelphia, PA 19104 USA

[6] Mayo Clin, Div Hematol, Rochester, MN USA

[7] Takamatsu Red Cross Hosp, Dept Hematol, Takamatsu, Kagawa, Japan

[8] Univ Penn, Perelman Sch Med, Div Translat Med & Human Genet, Philadelphia, PA 19104 USA

[9] Guys & St Thomas NHS Fdn Trust, London, England

[10] Chinese Univ Hong Kong, Prince Wales Hosp, Sir YK Pao Ctr Canc, Dept Med & Therapeut, Hong Kong, Peoples R China

[11] Univ Washington, Dept Lab Med, Seattle, WA 98195 USA

[12] Univ Penn, Perelman Sch Med, Div Hematol Oncol, Philadelphia, PA 19104 USA

[13] Harvard Med Sch, Dana Farber Canc Inst, Boston, MA 02115 USA

[14] Thomas Jefferson Univ, Dept Gen Surg & Surg Oncol, Philadelphia, PA 19107 USA

[15] Oslo Univ Hosp, Dept Oncol, Norwegian Radium Hosp, Oslo, Norway

[16] Emory Univ, Dept Hematol & Med Oncol, Atlanta, GA 30322 USA

[17] Emory Univ, Aflac Canc & Blood Disorders Ctr, Childrens Healthcare Atlanta, Atlanta, GA 30322 USA

[18] Osaka Univ, Inst Sci & Ind Res, Dept Organ Fine Chem, Osaka, Japan

[19] Univ Calif San Diego, Moores Canc Ctr, Ctr Personalized Therapy, La Jolla, CA USA

[20] Univ Calif San Diego, Moores Canc Ctr, Clin Trials Off, La Jolla, CA USA

[21] Univ Washington, Fred Hutchinson Canc Res Ctr, Seattle, WA 98195 USA

[22] Univ Washington, Med Oncol, Seattle, WA 98195 USA

[23] Univ Washington, Infect Dis Res Inst, Dept Med & Global Hlth, Seattle, WA 98195 USA

[24] Weill Cornell Med Coll, Dept Pathol & Lab Med, New York, NY USA

来源：

BLOOD ADVANCES | 2020年 / 4卷 / 23期

关键词：

LYMPH-NODE HYPERPLASIA; DENDRITIC CELL-SARCOMA; HODGKINS-DISEASE; LYMPHOPROLIFERATIVE DISORDER; PREOPERATIVE EMBOLIZATION; PARANEOPLASTIC PEMPHIGUS; SURGICAL RESECTION; RADIATION-THERAPY; AA AMYLOIDOSIS; MANAGEMENT;

D O I：

10.1182/bloodadvances.2020003334

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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-82 /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.

引用

页码：6039 / 6050

页数：12

共 115 条

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