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Review

Immunology and targeted therapy in Castleman disease

ORCID Icon, ORCID Icon, ORCID Icon, ORCID Icon & ORCID Icon
Received 01 Mar 2024, Accepted 16 May 2024, Published online: 27 May 2024

References

  • Castleman B, Towne VW. CASE records of the Massachusetts general hospital weekly clinicopathological exercises: case 40011. N Engl J Med. 1954 Jan 7;250(1):26–30. doi: 10.1056/NEJM195401072500107
  • Castleman B, Iverson L, Menedez VP. Localized mediastinal lymphnode hyperplasia resembling thymoma. Cancer. 1956 Jul;9(4):822–830. doi: 10.1002/1097-0142(195607/08)9:4<822:AID-CNCR2820090430>3.0.CO;2-4
  • Flendrig JA. Benign giant lymphoma: clinicopathologic correlation study. In: Clark R Gumley R, editors. The year book of cancer. Chicago: Year Book Medical Publishers; 1970. p. 296–299.
  • Keller AR, Hochholzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer. 1972 Mar;29(3):670–683. doi: 10.1002/1097-0142(197203)
  • Mori S, Mohri N, Uchida T, et al. Idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia: a syndrome related to giant lymph node hyperplasia of plasma cell type]. J Jpn Soc Res (New York). 1980;20(suppl):55–65. [in Japanese].
  • Frizzera G, Banks PM, Massarelli G, et al. A systemic lymphoproliferative disorder with morphologic features of Castlemanʼs disease. Am J Surg Pathol. 1983 Apr;7(3):211–232. doi: 10.1097/00000478-198304000-00001
  • Chen KT. Multicentric Castlemanʼs disease and Kaposiʼs sarcoma. Am J Surg Pathol. 1984 Apr;8(4):287–294. doi: 10.1097/00000478-198404000-00006
  • Lachant NA, Sun NC, Leong LA, et al. Multicentric angiofollicular lymph node hyperplasia (Castleman’s disease) followed by Kaposi’s sarcoma in two homosexual males with the acquired immunodeficiency syndrome (AIDS). Am J Clin Pathol. 1985 Jan;83(1):27–33. doi: 10.1093/ajcp/83.1.27
  • Soulier J, Grollet L, Oksenhendler E, et al. Kaposi’s sarcoma-associated herpesvirus-like DNA sequences in multicentric Castleman’s disease. Blood. 1995 Aug 15;86(4):1276–1280. doi: 10.1182/blood.V86.4.1276.bloodjournal8641276
  • Kishimoto T. Factors affecting B-cell growth and differentiation. Annu Rev Immunol. 1985;3:133–157. doi: 10.1146/annurev.iy.03.040185.001025
  • Kishimoto T. The biology of interleukin-6. Blood. 1989 Jul;74(1):1–10. doi: 10.1182/blood.V74.1.1.1
  • Yoshizaki K, Matsuda T, Nishimoto N, et al. Pathogenic significance of interleukin-6 (IL-6/BSF-2) in Castleman’s disease. Blood. 1989 Sep;74(4):1360–1367. doi: 10.1182/blood.V74.4.1360.1360
  • Nishimoto N, Sasai M, Shima Y, et al. Improvement in Castleman’s disease by humanized anti-interleukin-6 receptor antibody therapy. Blood. 2000 Jan 1;95(1):56–61. doi: 10.1182/blood.V95.1.56
  • Nishimoto N, Kanakura Y, Aozasa K, et al. Humanized anti-interleukin-6 receptor antibody treatment of multicentric Castleman disease. Blood. 2005;106(8):2627–2632. doi: 10.1182/blood-2004-12-4602
  • Dispenzieri A, Fajgenbaum DC. Overview of Castleman disease. Blood. 2020 Apr 16;135(16):1353–1364. doi: 10.1182/blood.2019000931
  • Fajgenbaum DC, Uldrick TS, Bagg A, et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood. 2017 Mar 23;129(12):1646–1657. doi: 10.1182/blood-2016-10-746933
  • van Rhee F, Voorhees P, Dispenzieri A, et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood. 2018 Nov 15;132(20):2115–2124. doi: 10.1182/blood-2018-07-862334
  • van Rhee F, Oksenhendler E, Srkalovic G, et al. International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease. Blood Adv. 2020 Dec 8;4(23):6039–6050. doi: 10.1182/bloodadvances.2020003334
  • Takai K, Nikkuni K, Shibuya H, et al. Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly Rinsho Ketsueki. [Rinsho Ketsueki] Jpn J Clin hematol. 2010;51(5):320–325. [in Japanese].
  • Iwaki N, Fajgenbaum DC, Nabel CS, et al. Clinicopathologic analysis of TAFRO syndrome demonstrates a distinct subtype of HHV-8-negative multicentric Castleman disease. Am J Hematol. 2016 Feb;91(2):220–226. doi: 10.1002/ajh.24242
  • Kawabata H, Takai K, Kojima M, et al. Castleman-Kojima disease (TAFRO syndrome) : a novel systemic inflammatory disease characterized by a constellation of symptoms, namely, thrombocytopenia, ascites (anasarca), microcytic anemia, myelofibrosis, renal dysfunction, and organomegaly : a status report and summary of Fukushima (6 June, 2012) and Nagoya meetings (22 September, 2012). J Clin Exp Hematop. 2013;53(1):57–61. doi: 10.3960/jslrt.53.57
  • Takatsuki K, Sanada I. Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases. Jpn J Clin Oncol. 1983 Sep;13(3):543–555.
  • Nakanishi T, Sobue I, Toyokura Y, et al. The Crow-Fukase syndrome: a study of 102 cases in Japan. Neurology. 1984 Jun;34(6):712–720. doi: 10.1212/wnl.34.6.712
  • Fajgenbaum DC, van Rhee F, Nabel CS. HHV-8-negative, idiopathic multicentric Castleman disease: novel insights into biology, pathogenesis, and therapy. Blood. 2014 May 8;123(19):2924–2933. doi: 10.1182/blood-2013-12-545087
  • Takeuchi K. Idiopathic plasmacytic lymphadenopathy: a conceptual history along with a translation of the original Japanese article published in 1980. J Clin Exp Hematop. 2022;62(2):79–84. doi: 10.3960/jslrt.22011
  • Gao YH, Liu YT, Zhang MY, et al. Idiopathic multicentric Castleman disease (iMCD)-idiopathic plasmacytic lymphadenopathy: A distinct subtype of iMCD-not otherwise specified with different clinical features and better survival. Br J Haematol. 2024 Feb 15;204(5):1830–1837. doi: 10.1111/bjh.19334
  • Kojima M, Nakamura N, Tsukamoto N, et al. Clinical implications of idiopathic multicentric Castleman disease among Japanese: a report of 28 cases. Int J Surg Pathol. 2008 Oct;16(4):391–398. doi: 10.1177/1066896908315812
  • Masaki Y, Kawabata H, Takai K, et al. 2019 Updated diagnostic criteria and disease severity classification for TAFRO syndrome. Int J Hematol. 2020 Jan;111(1):155–158. doi: 10.1007/s12185-019-02780-1
  • Nishimura Y, Nishimura MF, Sato Y. International definition of iMCD-TAFRO: future perspectives. J Clin Exp Hematop. 2022 Jun 28;62(2):73–78. doi: 10.3960/jslrt.21037
  • Nishimura MF, Nishimura Y, Nishikori A, et al. Historical and pathological overview of Castleman disease. J Clin Exp Hematop. 2022 Jun 28;62(2):60–72. doi: 10.3960/jslrt.21036
  • Nishimura Y, Fajgenbaum DC, Pierson SK, et al. Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease. Am J Hematol. 2021 Oct 1;96(10):1241–1252. doi: 10.1002/ajh.26292
  • Szalat R, Munshi NC. Diagnosis of Castleman Disease. Hematol Oncol Clin North Am. 2018 Feb;32(1):53–64. doi: 10.1016/j.hoc.2017.09.005
  • Fajgenbaum DC, Wu D, Goodman A, et al. Insufficient evidence exists to use histopathologic subtype to guide treatment of idiopathic multicentric Castleman disease. Am J Hematol. 2020 Dec;95(12):1553–1561. doi: 10.1002/ajh.25992
  • Cronin DM, Warnke RA. Castleman disease: an update on classification and the spectrum of associated lesions. Adv Anat Pathol. 2009 Jul;16(4):236–246. doi: 10.1097/PAP.0b013e3181a9d4d3
  • Dupin N, Diss TL, Kellam P, et al. HHV-8 is associated with a plasmablastic variant of Castleman disease that is linked to HHV-8-positive plasmablastic lymphoma. Blood. 2000 Feb 15;95(4):1406–1412. doi: 10.1182/blood.V95.4.1406.004k26_1406_1412
  • Lazzi S, Bellan C, Amato T, et al. Kaposi’s sarcoma-associated herpesvirus/human herpesvirus 8 infection in reactive lymphoid tissues: a model for KSHV/HHV-8-related lymphomas?. Hum Pathol. 2006 Jan;37(1):23–31. doi: 10.1016/j.humpath.2005.08.020
  • Nishikori A, Nishimura MF, Nishimura Y, et al. Idiopathic Plasmacytic Lymphadenopathy Forms an Independent Subtype of Idiopathic Multicentric Castleman Disease. Int J Mol Sci. 2022 Sep 7;23(18):10301. doi: 10.3390/ijms231810301
  • Fujiki T, Hirasawa S, Watanabe S, et al. Successful treatment by tocilizumab without steroid in a very severe case of TAFRO syndrome. CEN Case Rep. 2017 May;6(1):105–110. doi: 10.1007/s13730-017-0253-6
  • Carbone A, Pantanowitz L. TAFRO syndrome: an atypical variant of KSHV-negative multicentric Castleman disease. Am J Hematol. 2016 Feb;91(2):171–172. doi: 10.1002/ajh.24274
  • Fajgenbaum DC, Shilling D. Castleman Disease Pathogenesis. Hematol Oncol Clin North Am. 2018 Feb;32(1):11–21. doi: 10.1016/j.hoc.2017.09.002
  • Vermi W, Lonardi S, Bosisio D, et al. Identification of CXCL13 as a new marker for follicular dendritic cell sarcoma. J Pathol. 2008 Nov;216(3):356–364. doi: 10.1002/path.2420
  • Wong RSM. unicentric castleman disease. Hematol Oncol Clin North Am. 2018 Feb;32(1):65–73. doi: 10.1016/j.hoc.2017.09.006
  • Katano H, Sato Y, Kurata T, et al. Expression and localization of human herpesvirus 8-encoded proteins in primary effusion lymphoma, Kaposi’s sarcoma, and multicentric Castleman’s disease. Virology. 2000 Apr 10;269(2):335–344. doi: 10.1006/viro.2000.0196
  • Burger R, Neipel F, Fleckenstein B, et al. Human herpesvirus type 8 interleukin-6 homologue is functionally active on human myeloma cells. Blood. 1998 Mar 15;91(6):1858–1863. doi: 10.1182/blood.V91.6.1858
  • Dispenzieri A. POEMS Syndrome: 2019 update on diagnosis, risk-stratification, and management. Am J Hematol. 2019 Jul;94(7):812–827. doi: 10.1002/ajh.25495
  • Watanabe O, Arimura K, Kitajima I, et al. Greatly raised vascular endothelial growth factor (VEGF) in POEMS syndrome. Lancet. 1996 Mar 9;347(9002):702. doi: 10.1016/s0140-6736(96)91261-1
  • Kawajiri-Manako C, Mimura N, Fukuyo M, et al. Clonal immunoglobulin λ light-chain gene rearrangements detected by next generation sequencing in POEMS syndrome. Am J Hematol. 2018 Sep;93(9):1161–1168. doi: 10.1002/ajh.25213
  • D’Souza A, Hayman SR, Buadi F, et al. The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome. Blood. 2011 Oct 27;118(17):4663–4665. doi: 10.1182/blood-2011-06-362392
  • Fujimoto S, Kawabata H, Sakai T, et al. Optimal treatments for TAFRO syndrome: a retrospective surveillance study in Japan. Int J Hematol. 2021 Jan;113(1):73–80. doi: 10.1007/s12185-020-03008-3
  • Sarmiento Bustamante M, Shyamsundar S, Coren FR, et al. Ongoing symptoms following complete surgical excision in unicentric Castleman disease. Am J Hematol. 2023 Nov;98(11):E334–E337. doi: 10.1002/ajh.27065
  • Akira S, Taga T, Kishimoto T. Interleukin-6 in biology and medicine. Adv Immunol. 1993;54:1–78. doi: 10.1016/s0065-2776(08)60532-5
  • Tanaka T, Narazaki M, Kishimoto T. Interleukin (IL-6) Immunotherapy. Cold Spring Harb Perspect Biol. 2018 Aug 1;10(8):a028456. doi: 10.1101/cshperspect.a028456
  • Choy EH, De Benedetti F, Takeuchi T, et al. Translating IL-6 biology into effective treatments. Nat Rev Rheumatol. 2020 Jun;16(6):335–345. doi: 10.1038/s41584-020-0419-z
  • Kishimoto T, Akira S, Taga T. Interleukin-6 and its receptor: a paradigm for cytokines. Science. 1992 Oct 23;258(5082):593–597. doi: 10.1126/science.1411569
  • Hibi M, Murakami M, Saito M, et al. Molecular cloning and expression of an IL-6 signal transducer, gp130. Cell. 1990 Dec 21;63(6):1149–1157. doi: 10.1016/0092-8674(90)90411-7
  • Heinrich PC, Behrmann I, Haan S, et al. Principles of interleukin (IL)-6-type cytokine signalling and its regulation. Biochem J. 2003 Aug 15;374(Pt 1):1–20. doi: 10.1042/BJ20030407
  • Yoshizaki K, Murayama S, Ito H, et al. The Role of Interleukin-6 in Castleman Disease. Hematol Oncol Clin North Am. 2018 Feb;32(1):23–36. doi: 10.1016/j.hoc.2017.09.003
  • van Rhee F, Wong RS, Munshi N, et al. Siltuximab for multicentric Castleman’s disease: a randomised, double-blind, placebo-controlled trial. Lancet Oncol. 2014;15(9):966–974. doi: 10.1016/S1470-2045(14)70319-5 Erratum in: Lancet Oncol. 2014;15(10):417.
  • Glennie MJ, French RR, Cragg MS, et al. Mechanisms of killing by anti-CD20 monoclonal antibodies. Mol Immunol. 2007 Sep;44(16):3823–3837. doi: 10.1016/j.molimm.2007.06.151
  • Corbellino M, Bestetti G, Scalamogna C, et al. Long-term remission of Kaposi sarcoma-associated herpesvirus-related multicentric Castleman disease with anti-CD20 monoclonal antibody therapy. Blood. 2001 Dec 1;98(12):3473–3475. doi: 10.1182/blood.v98.12.3473
  • Uldrick TS, Polizzotto MN, Aleman K, et al. Rituximab plus liposomal doxorubicin in HIV-infected patients with KSHV-associated multicentric Castleman disease. Blood. 2014 Dec 4;124(24):3544–3552. doi: 10.1182/blood-2014-07-586800
  • Ide M, Kawachi Y, Izumi Y, et al. Long-term remission in HIV-negative patients with multicentric Castleman’s disease using rituximab. Eur J Haematol. 2006 Feb;76(2):119–123. doi: 10.1111/j.1600-0609.2005.00570.x
  • Stepkowski SM. Molecular targets for existing and novel immunosuppressive drugs. Expert Rev Mol Med. 2000 Jun 21;2(4):1–23. doi: 10.1017/S1462399400001769
  • Faul C, Donnelly M, Merscher-Gomez S, et al. The actin cytoskeleton of kidney podocytes is a direct target of the antiproteinuric effect of cyclosporine A. Nat Med. 2008 Sep;14(9):931–938. doi: 10.1038/nm.1857
  • Bao H, Liu ZH, Xie HL, et al. Successful treatment of class V+IV lupus nephritis with multitarget therapy. J Am Soc Nephrol. 2008 Oct;19(10):2001–2010. doi: 10.1681/ASN.2007121272
  • Yamaga Y, Tokuyama K, Kato T, et al. Successful treatment with Cyclosporin a in tocilizumab-resistant TAFRO syndrome. Intern Med. 2016;55(2):185–190. doi: 10.2169/internalmedicine.55.4710
  • Shirai T, Onishi A, Waki D, et al. Successful treatment with tacrolimus in TAFRO syndrome: two case reports and literature review. Medicine (Baltimore). 2018 Jun;97(23):e11045. doi: 10.1097/MD.0000000000011045
  • Fajgenbaum DC, Langan RA, Japp AS, et al. Identifying and targeting pathogenic PI3K/AKT/mTOR signaling in IL-6-blockade-refractory idiopathic multicentric Castleman disease. J Clin Invest. 2019 Aug 13;129(10):4451–4463. doi: 10.1172/JCI126091
  • Sehgal SN. Sirolimus: its discovery, biological properties, and mechanism of action. Transplant Proc. 2003 May;35(3):S7–S14. doi: 10.1016/s0041-1345(03)00211-2
  • Teachey DT, Obzut DA, Axsom K, et al. Rapamycin improves lymphoproliferative disease in murine autoimmune lymphoproliferative syndrome (ALPS). Blood. 2006 15;108(6):1965–1971. doi: 10.1182/blood-2006-01-010124
  • McCarthy C, Gupta N, Johnson SR, et al. Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management. Lancet Respir Med. 2021 Nov;9(11):1313–1327. doi: 10.1016/S2213-2600(21)00228-9
  • Sumiyoshi R, Koga T, Furukawa K, et al. A case of tocilizumab-refractory idiopathic multicentric Castleman’s disease successfully treated with sirolimus. Clin Immunol. 2021 Dec;233:108887. doi: 10.1016/j.clim.2021.108887
  • Koga T, Takemori S, Hagimori N, et al. An open-label continuation trial of sirolimus for tocilizumab-refractory idiopathic multicentric Castleman disease: Study protocol for an investigator-initiated, multicenter, open-label trial (SPIRIT compliant). Medicine (Baltimore). 2020 Dec 11;99(50):e23291. doi: 10.1097/MD.0000000000023291
  • Arenas DJ, Floess K, Kobrin D, et al. Increased mTOR activation in idiopathic multicentric Castleman disease. Blood. 2020 May 7;135(19):1673–1684. doi: 10.1182/blood.2019002792
  • Phillips AD, Kakkis JJ, Tsao PY, et al. Increased mTORC2 pathway activation in lymph nodes of iMCD-TAFRO. J Cell Mol Med. 2022;26(11):3147–3152. doi: 10.1111/jcmm.17251
  • Ivashkiv LB, Hu X. Signaling by STATs. Arthritis Res Ther. 2004;6(4):159–168. doi: 10.1186/ar1197
  • Kang S, Tanaka T, Narazaki M, et al. Targeting Interleukin-6 Signaling in Clinic. Immunity. 2019 Apr 16;50(4):1007–1023. doi: 10.1016/j.immuni.2019.03.026
  • Pierson SK, Shenoy S, Oromendia AB, et al. Discovery and validation of a novel subgroup and therapeutic target in idiopathic multicentric Castleman disease. Blood Adv. 2021;5(17):3445–3456. doi: 10.1182/bloodadvances.2020004016
  • Killian M, Viel S, Chalayer E, et al. JAK1/2 Inhibition in Severe TAFRO Syndrome: A Case Report. Ann Intern Med. 2021 May;174(5):719–721. doi: 10.7326/L20-1051
  • Lust H, Gong S, Remiker A, et al. Idiopathic multicentric Castleman disease with TAFRO clinical subtype responsive to IL-6/JAK inhibition: A pediatric case series. Pediatr Blood Cancer. 2021;68(10):e29261. doi: 10.1002/pbc.29261
  • Pierson SK, Stonestrom AJ, Shilling D, et al. Plasma proteomics identifies a ‘chemokine storm’ in idiopathic multicentric Castleman disease. Am J Hematol. 2018 Jul;93(7):902–912. doi: 10.1002/ajh.25123
  • Pierson SK, Katz L, Williams R, et al. CXCL13 is a predictive biomarker in idiopathic multicentric Castleman disease. Nat Commun. 2022 Nov 24;13(1):7236. doi: 10.1038/s41467-022-34873-7
  • Havenar-Daughton C, Lindqvist M, Heit A, et al. CXCL13 is a plasma biomarker of germinal center activity. Proc Natl Acad Sci USA 2016 Mar 8;113(10):2702–2707. doi: 10.1073/pnas.1520112113
  • Rao DA. T cells that help B cells in chronically inflamed tissues. Front Immunol. 1924 2018 Aug 23;9. doi: 10.3389/fimmu.2018.01924
  • Nakayama S, Taguchi N, Isaka Y, et al. Glibenclamide and Therapeutic Hypothermia Have Comparable Effect on Attenuating Global Cerebral Edema Following Experimental Cardiac Arrest. Neurocrit Care. 2018 Aug;29(1):119–127. doi: 10.1007/s12028-017-0479-3
  • Yoshitomi H, Ueno H. Shared and distinct roles of T peripheral helper and T follicular helper cells in human diseases. Cell Mol Immunol. 2021 Mar;18(3):523–527. doi: 10.1038/s41423-020-00529-z
  • Bocharnikov AV, Keegan J, Wacleche VS, et al. Accelerating Medicines Partnership (AMP) RA/SLE Network. PD-1hiCXCR5- T peripheral helper cells promote B cell responses in lupus via MAF and IL-21. JCI Insight. 2019 Oct 17;4(20):e130062. doi: 10.1172/jci.insight.130062
  • Kikushige Y, Ishikawa F, Miyamoto T, et al. Self-renewing hematopoietic stem cell is the primary target in pathogenesis of human chronic lymphocytic leukemia. Cancer Cell. 2011 Aug 16;20(2):246–259. doi: 10.1016/j.ccr.2011.06.029
  • Harada T, Kikushige Y, Miyamoto T, et al. Peripheral helper-T-cell-derived CXCL13 is a crucial pathogenic factor in idiopathic multicentric Castleman disease. Nat Commun. 2023 Oct 31;14(1):6959. doi: 10.1038/s41467-023-42718-0
  • Iwaki N, Gion Y, Kondo E, et al. Elevated serum interferon γ-induced protein 10 kDa is associated with TAFRO syndrome. Sci Rep. 2017 Feb 13;7:42316. doi: 10.1038/srep42316
  • Yuan ZG, Dun XY, Li YH, et al. Treatment of multicentric Castleman’s Disease accompanying multiple myeloma with bortezomib: a case report. J Hematol Oncol. 2009 Apr 28;2:19. doi: 10.1186/1756-8722-2-19
  • Zhang L, Zhang MY, Cao XX, et al. A prospective, multicenter study of bortezomib, cyclophosphamide, and dexamethasone in relapsed/refractory iMCD. Leuk Lymphoma. 2022 Mar;63(3):618–626. doi: 10.1080/10428194.2021.1999437
  • Sato K, Tsuchiya M, Saldanha J, et al. Humanization of a mouse anti-human interleukin-6 receptor antibody comparing two methods for selecting human framework regions. Mol Immunol. 1994 Apr;31(5):371–381. doi: 10.1016/0161-5890(94)90115-5

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