Treatment of 14 cases of Castleman’s disease: the experience of one centre and an overview of literature

Česká verzia

Authors: Zdeněk Adam ¹; Petr Szturz ¹; Marta Krejčí ¹; Renata Koukalová ²; Eva Michalková ¹; Zdeněk Řehák ²; Eva Pourová ³; Luděk Pour ¹; Pavlína Volfová ¹; Viera Sandecká ¹; Zdeňka Čermáková ^4,9; Leoš Křen ⁵; Filip Sokol ⁵; Ivo Hanke ⁶; Igor Penka ⁶; Hana Petrášová ⁷; Sabina Ševčíková ⁸; Zdeněk Král ¹; Jiří Mayer ¹
Authors place of work: Interní hematologická a onkologická klinika LF MU a FN Brno, pracoviště Bohunice ¹; Oddělení nukleární medicíny, centrum PET, RECAMO, Masarykův onkologický ústav, Brno ²; Ordinace praktického lékaře pro dospělé, Pustiměř ³; Oddělení klinické biochemie FN Brno, pracoviště Bohunice ⁴; Ústav patologie LF MU a FN Brno, pracoviště Bohunice ⁵; Chirurgická klinika LF MU a FN Brno, pracoviště Bohunice ⁶; Radiologická klinika LF MU a FN Brno, pracoviště Bohunice ⁷; Ústav patologické fyziologie LF MU Brno ⁸; Katedra laboratorních metod LF MU, Brno ⁹
Published in the journal: Vnitř Lék 2016; 62(4): 287-298
Category: Reviews

Summary

Castleman’s disease is the term for reactive lymphocytary and plasmocytary proliferation which occurs in the unicentric (localized) form, usually without systemic symptoms, or in the generalized/multicentric form, typically with systemic symptoms (www.vzacne-diagnozy.cz). Over the past 25 years we diagnosed, treated and followed 14 histologically proven cases of Castleman’s diseases. Seven patients had the localised form of the disease. In 5 of 7 cases the pathological lesion was located intrathoracically or intraabdominally and in only 2 cases it was on the surface of the body. No clinical symptoms were present in any of the patients with the unicentric form of the disease and surgical treatment led to the total removing of the disease in all of them. As opposed to that, all 7 patients with the multicentric form of Castleman’s disease experienced febrile or subfebrile temperatures. Three of the 7 patients complained of severe troubling night sweats. Clinical expressions of vasculitis which was the cause of stroke, were present in 1 of 7 patients. Osteosclerotic changes on the skeleton were detected in 1 patient, who also suffered from fluid retention likely associated with this disease. Polyclonal propagation of immunoglobulins, predominantly immunoglobulin IgG type, was present in 5 of 7 patients with the multicentric form. In one case there was one complete molecule of monoclonal imunoglobuline present and in one case loose light chains κ were increased More than 1 sampling of material for histological examination of enlarged lymph nodes were needed in 6 of 7 patients for diagnosing the multicentric form of the disease. It has turned out beneficial with respect to diagnosing the disease to carry out surgical removal and histological examination of the nodes which accumulated the most fluorodeoxyglucose within PET-CT examination. The text describes experience of the treatment. In recent years the basis for the treatment has been the monoclonal antibody antiCD20 rituximab, or thalidomide and lenalidomide, or possibly their combination. The new medicine for these patients is interleukin-6 antibody called siltuximab (Sylvant), of which we have no own experience so far. Five of our seven patients with the multicentric form received treatment, 1 patient refused treatment and in one patient the signs of the disease activity are not expressed to such extent that would require treatment. The therapy containing rituximab reached complete remission in 2 patients and the therapy containing thalidomide and lenalidomide achieved the complete remission of the disease in 3 patients. In one of the above described cases the disease did not respond to the initial treatment with rituximab and remission was reached by thalidomide and lenalidomide and in one case the disease did not respond to the initial treatment with thalidomide and complete remission was reached with rituximab. Following the treatment, no patient with the multicentric form of Castleman’s disease has had a relapse until now.

Key words:
anemia of chronic diseases – Castleman’s disease – hyperproteinemia – lenalidomide – polyclonal hypergammaglobulinemia – rituximab – siltuximab – thalidomide

Zdroje

1. Dispenzieri A. Castleman disease. Cancer Treat Res 2008; 142: 293–330.

2. Ščudla V. Anémie chronických chorob, standardy diagnostiky a léčby. Vnitř Lék 2002; 48(5): 422–426.

3. Szturz P, Adam Z, Moulis M et al. Naše zkušenosti s léčbou multicentrické plazmocelulární varianty Castlemanovy choroby s projevy vaskulitidy. Vnitř Lék 2012; 58(9): 679–690.

4. Adam Z, Szturz P, Křen L et al. PET-CT dokumentovaný rychlý nástup léčebné odpovědi cyklofosfamidu, thalidomidu a dexametazonu u multicentrické formy Castlemanovy nemoci. Popis případu a přehled informací o léčbě. Vnitř Lék 2013; 59(4): 301–312.

5. Adam Z, Szturz P, Koukalová R et al. PET-CT dokumentovaná remise multicentrické formy Castlemanovy choroby po léčbě rituximabem Popis případu a přehled literatury Vnitř Lék 2015; 61(3): 251–259.

6. Castleman B, Towe VW. Case report of the Massachusetts General Hospital weekly clinicopathological exercises, fouded by Richard C Cabot. N Engl J Med 1954; 251(10): 396–400.

7. Keller AR, Hochholzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other localisation. Cancer 1972; 29(3): 670–683.

8. Gaba AR, Stein RS, Sweet DJ et al. Multicentric giant node hyperplasia. Am J Clin Pathol 1978; 69(1): 86–90.

9. Soulier J, Grollet L, Oksenhendler E et al. Kaposi’s sarcoma-associated herpesvirus-like DNA sequences in multicentric Castleman’s disease. Blood 1995; 86(4): 1276–1280.

10. Chadbum A, Cesarman E, Nador RG et al. Kaposi´s sarcoma-associated herpesvirus sequences in benign lymphoid proliferations not associated with human immunodeficiency virus. Cancer 1997; 80(4): 788–797.

11. Riu P, Noesl LH, Droz D et al. Glomerular involvement in lymfoproliferative disorders with hyperproduction of cytokines (Castleman, POEMS). Adv Nephrol Necker Hosp 2000; 30: 305–331.

12. Masaki Y, Nakajima A, Iwao H et al. Japanese variant of multicentric castleman’s disease associated with serositis and thrombocytopenia – a report of two cases: is TAFRO syndrome (Castleman- Kojima disease) a distinct clinicopathological entity? J Clin Exp Hematop 2013; 53(1): 79–85.

13. 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. J Clin Exp Hematop 2013; 53(1): 57–61.

14. Inoue M, Ankou M, Hua J et al. Complete resolution of TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly) after immunosuppressive therapies using corticosteroids and cyclosporin A: a case report. J Clin Exp Hematop 2013; 53(1): 95–99.

15. Tedesco S, Postacchini L, Manfredi L et al. Successful treatment of a Caucasian case of multifocal Castleman´s disease with TAFRO syndrome with pathophysiology targeted therapy- case report. Exp Hematol Oncol 2015; 4(1): 3. Dostupné z DOI: <http://dx.doi.org/10.1186/2162–3619–4-3>.

16. Robinson D Jr, Reynolds M, Casper C et al. Clinical epidemiology and treatment patterns of patients with multicentric Castleman disease: results from two US treatment centres. Br J Haematol 2014; 165(1): 39–48.

17. Fajgenbaum DC, vanRhee F, Nabel CS. HHV-8 negative idiopathic multicentric Castleman disease: novel insight into biology pathogenesis and therapy. Blood 2014; 123(19): 2924–2933.

18. Bower M, Pria AD, Coyle C et al. Diagnostic criteria schemes for multicentric Castleman disease in 75 cases. J Acquir Immune Defic Syndr 2014; 65(2): e80-e82. Dostupné z DOI: http://dx.doi.org/10.1097/QAI.0b013e3182a8338f.

19. Cronin DM, Warnke RA. Castleman disease: an update on classification and the spectrum of associated lesions. Adv Anat Pathol 2009; 16(4): 236–246.

20. Kawabata H, Kadowaki N, Nishikori M et al. Clinical features and treatment of multicentric Castleman’s disease : a retrospective study of 21 Japanese patients at a single institute. J Clin Exp Hematop 2013; 53(1): 69–77.

21. El Karoui K, Vuiblet V, Dion D et al. Renal involvement in Castleman disease. Nephrol Dial Transplant 2011; 26(2): 599–609.

22. Sydor A, Madura M, Wagrowska-Danilewicz M. Amyloid a amyloidosis and renal failure in a course of Castleman disease. Nephrology (Carlton) 2007; 12(6): 620–621.

23. Leung KT, Wong KM, Choi KS et al. Multiicentric Castleman’s disease complicated by secondary renal amyloidosis. Nephrology (Carlton) 2004; 9(6): 392–393.

24. Dispenzieri A, Buadi FK. A review of POEMS syndrome. Oncology (Williston Park) 2013; 27(12): 1242–1250.

25. Dispenzieri A. POEMS syndrome: 2014 update on diagnosis, risk-stratification, and management. Am J Hematol 2014; 89(2): 214–223.

26. Fajgenbaum DC, Rosenbach M, van Rhee F et al. Eruptive cherry hemangiomatosis associated with multicentric Castleman disease: a case report and diagnostic clue. JAMA Dermatol 2013; 149(2): 204–208. Erratum in JAMA Dermatol 2014; 150(4): 460.

27. Ruwan KP, Parakramawansha C, Wijeweera I et al. A case of POEMS syndrome with mixed hyaline vascular and plasma cell type Castleman’s disease. Ceylon Med J 2009; 54(2): 68–69.

28. Misri R, Kharkar V, Dandale A et al. Multiple capillary hemangiomas: a distinctive lesion of multicentric Castleman’s disease and POEMS syndrome. Indian J Dermatol Venereol Leprol 2008; 74(4): 364–366.

29. Garcia T, Dafer R, Hocker S et al Recurrent strokes in two patients with POEMS syndrome and Castleman’s disease. J Stroke Cerebrovasc Dis 2007; 16(6): 278–284.

30. Owens CL, Weir EG, Ali SZ. Cytopathologic findings in “POEMS” syndrome associated with Castleman disease. Diagn Cytopathol 2007; 35(8): 512–515.

31. Eisenbarth SC, Colegio OR, Iyer A et al. Images in neuro-oncology: a case of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes) in a patient with multicentric Castleman’s disease. J Neurooncol 2007; 81(2): 163–165.

32. Huang J, Wang L, Zhou W et al. Hyaline vascular Castleman disease associated with POEMS syndrome and cerebral infarction. Ann Hematol 2007; 86(1): 59–61.

33. Tal Y, Haber G, Cohen MJ et al. Autologous stem cell transplantation in a rare multicentric Castleman disease of the plasma cell variant. Int J Hematol 2011; 93(5): 677–680.

34. Dispenzieri A, Armitage JO, Loes MJ et al. The clinical spectrum of Castleman´s disease. Am J Hematol 2012; 87(11): 997–1002.

35. Robinson D jr, Reynolds M, Casper C et al. Clinical epidemiology and treatment patterns of patients with multicentric Castleman disease: results from two US treatment centres. Br J Haematol 2014; 165(1): 39–48.

36. Chronowski GM, Ha CS, Wilder RB et al. Treatment of unicentric and multicentric Castleman disease and the role of radiotherapy. Cancer 2001; 92(3): 670–676.

37. Wolf M, Van Offel JF, Van de Velde A et al. Multicentric plasma cell variant Castleman’s disease presenting with cutaneous vasculitis and pulmonary parenchymal involvement in a patient with ankylosing spondylitis: case report and review of the literature. Acta Clin Belg 2011; 66(4): 305–310.

38. Dham A, Peterson BA. Castleman disease. Curr Opin Hematol 2007; 14(4): 354–359.

39. Peker D, Martinez AE, Schwartz MA et al. Complete remission in 4 patients with human herpesvirus 8-associated multicentric Castleman disease using rituximab and liposomal doxorubicin, a novel chemotherapy combination. Clin Adv Hematol Oncol 2012; 10(3): 204–206.

40. Hoffmann C, Schmid H, Müller M et al. Improved outcome with rituximab in patients with HIV-associated multicentric Castleman disease. Blood 2011; 118(13): 3499–3503.

41. Fragasso A, Mannarella C, Ciancio A et al. Complete remission and virologic response to combined chemoimmunotherapy (R-CVP) followed by rituximab maintenance in HIV-negative, HHV-8 positive patient with multicentric Castleman disease. Leuk Lymphoma 2008; 49(11): 2224–2226.

42. Casquero A, Barroso A, Fernández Guerrero ML. Use of rituximab as a salvage therapy for HIV-associated multicentric Castleman disease. Ann Hematol 2006; 85(3): 185–187.

43. 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; 76(2): 119–123.

44. Powles T, Stebbing J, Montoto S et al. Rituximab as retreatment for rituximab pretreated HIV-associated multicentric Castleman disease. Blood 2007; 110(12): 4132–4133.

45. Bestawros A, Michel R, Séguin C et al. Multicentric Castleman’s disease treated with combination chemotherapy and rituximab in four HIV-positive men: a case series. Am J Hematol 2008; 83(6): 508–511.

46. Buchler T, Dubash S, Lee V et al. Rituximab failure in fulminant multicentric HIV/human herpesvirus 8-associated Castleman’s disease with multiorgan failure: report of two cases. AIDS 2008; 22(13): 1685–1687.

47. Bower M, Veraitch O, Szydlo R et al. Cytokine changes during rituximab therapy in HIV-associated multicentric Castleman disease. Blood 2006; 113(19): 4521–4524.

48. Mian H, Leber B. Mixed variant multicentric Castleman disease treated with rituximab: case report. J Pediatr Hematol Oncol 2010; 32(8): 622.

49. Lee JP, Kim DK, Oh DY et al. Successfully treated multicentric Castleman’s disease with renal thrombotic microangiopathy using rituximab and corticosteroid. Clin Nephrol 2011; 75(2): 165–170.

50. Gérard L, Bérezné A, Galicier L et al . Prospective study of rituximab in chemotherapy-dependent human immunodeficiency virus associated multicentric Castleman’s disease: ANRS 117 CastlemaB Trial. J Clin Oncol 2007; 25(22): 3350–3356.

51. Gérard L, Michot JM, Burcheri S et al. Rituximab decreases the risk of lymphoma in patients with HIV-associated multicentric Castleman disease. Blood 2012; 119(10): 2228–2233.

52. Ramasamy K, Gandhi S, Flowers MT et al. Rituximab and thalidomide combination therapy for Castleman Disease. Brit J Haematol 2012; 158(3): 415–429.

53. Stary G, Kohrgruber N, Herneth AM et al. Complete regression of HIV-associated multicentric Castleman disease treated with rituximab and thalidomide. AIDS 2008; 22(10): 1232–1234.

54. Fishman SJ, Feins NR, D’Amato RJ et al. Long-term remission of Crohn’s disease treated with thalidomide: a seminal case report. Angiogenesis 1999; 3(3): 201–204.

55. Lee FC, Merchant SH. Alleviation of systemic manifestations of multicentric Castleman’s disease by thalidomide. Am J Hematol 2003; 73(1): 48–53.

56. Starkey CR, Joste NE, Lee FC. Near-total resolution of multicentric Castleman disease by prolonged treatment with thalidomide. Am J Hematol. 2006; 81(4): 303–304.

57. Kim SY, Lee SA, Ryoo HM et al. Thalidomide for POEMS syndrome. Ann Hematol 2006; 85(8): 545–546.

58. Wang X, Ye S, Xiong C et al. Successful treatment with bortezomib and thalidomide for POEMS syndrome associated with multicentric mixed-type Castleman’s disease. Jpn J Clin Oncol 2011; 41(10): 1221–1224.

59. Menegato MA, Canelles MF, Tonutti E et al. Remission of nephrotic syndrome after thalidomide therapy in a patient with Castleman’s disease. Clin Nephrol 2004; 61(5): 352–356.

60. Miltenyi Z, Toth J, Gonda A et al. Successful immunomodulatory therapy in castleman disease with paraneoplastic pemphigus vulgaris. Pathol Oncol Res 2009; 15(3): 375–381.

61. Zhao X, Shi R, Jin X et al. Diffuse hyperpigmented plaques as cutaneous manifestation of multicentric Castleman disease and treatment with thalidomide: report of three cases. J Am Acad Dermatol 2011; 65(2): 430–432.

62. Jung CP, Emmerich B, Goebel FD et al. Successful treatment of a patient with HIV-associated multicentric Castleman disease (MCD) with thalidomide. Am J Hematol 2004; 75(3): 176–177.

63. Wang X, Ye S, Xiong C et al. Successful treatment with bortezomib and thalidomide for POEMS syndrome associated with multicentric mixed-type Castleman’s disease. Jpn J Clin Oncol 2011; 41(10): 1221–1224.

64. Hess G, Wagner V, Kreft A et al. Effects of bortezomib on proinflamatory cytokine levels and transfusion dependency in patient with multicentric Castleman disease. Brit J Haematol 2006; 134(5): 544–545.

65. Sobas MA, Alonso Vence N, Diaz Arias J et al. Efficacy of bortezomib in refractory form of multicentric Castleman disease associated to POEMS syndrome (MCD-POEMS variant). Ann Hematol 2010; 89(2): 217–219.

66. Galeotti C, Boucheron A, Guillaume S et al. Sustained remission of multicentric Castleman disease in children treated with tocilizumab, an anti-interleukin-6 receptor antibody. Mol Cancer Ther 2012; 11(8): 1623–1626.

67. Yuzuriha A, Saitoh T, Koiso H et al. Successful treatment of autoimmune hemolytic anemia associated with multicentric Castleman disease by anti-interleukin-6 receptor antibody (tocilizumab) therapy. Acta Haematol 2011; 126(3): 147–150.

68. Higuchi T, Nakanishi T, Takada K et al. A case of multicentric Castleman’s disease having lung lesion successfully treated with humanized anti-interleukin-6 receptor antibody, tocilizumab. J Korean Med Sci 2010; 25(9): 1364–1367.

69. Arita Y, Sakata Y, Sudo T et al. The efficacy of tocilizumab in a patient with pulmonary arterial hypertension associated with Castleman’s disease. Heart Vessels 2010; 25(5): 444–447.

70. Song SN, Tomosugi N, Kawabata H et al. Down-regulation of hepcidin resulting from long-term treatment with an anti-IL-6 receptor antibody (tocilizumab) improves anemia of inflammation in multicentric Castleman disease. Blood 2010; 116(18): 3627–3634.

71. Nishimoto N, Terao K, Mima T et al. Mechanisms and pathologic significances in increase in serum interleukin-6 (IL-6) and soluble IL-6 receptor after administration of an anti-IL-6 receptor antibody, tocilizumab, in patients with rheumatoid arthritis and Castleman disease. Blood 2008; 112(10): 3959–3964.

72. Williams SC. First IL-6-blocking drug nears approval for rare blood disorder. Nat Med 2013; 19(10): 1193.

73. Robey RC, Mletzko S, Colley C et al. The use of monoclonal antibodies to treat Castleman’s disease. Immunotherapy 2014; 6(2): 211–219.

74. Nagai K, Ueda A, Yamagata K. Successful use of tocilizumab in a case of multicentric Castleman’s disease and end-stage renal disease. Ther Apher Dial 2014; 18(2): 210–211.

75. Man L, Goudar RK. Reversal of cardiomyopathy with tocilizumab in a case of HIV-negative Castleman’s disease. Eur J Haematol 2013; 91(3): 273–276.

76. Otani N, Morishita Y, Oh I et al. Successful treatment of a mesangial proliferative glomerulonephritis with interstitial nephritis associated with Castleman’s disease by an anti-interleukin-6 receptor antibody (tocilizumab). Intern Med 2012; 51(11): 1375–1378.

77. Liu YC, Stone K, van Rhee F. Siltuximab for multicentric Castleman disease. Expert Rev Hematol 2014; 7(5): 545–557.

78. van Rhee F, Fayad L, Voorhees P et al. Siltuximab, a novel anti-interleukin-6 monoclonal antibody, for Castleman’s disease. J Clin Oncol 2010; 28(23): 3701–3708.

79. Kurzrock R, Voorhees PM, Casper C et al. A phase I, open-label study of siltuximab, an anti-IL-6 monoclonal antibody, in patients with B-cell non-Hodgkin lymphoma, multiple myeloma, or Castleman disease. Clin Cancer Res 2013; 19(13): 3659–3670.

80. van Rhee F, Wong RS, Munshi N et al. Siltuximab for multicentric Castleman´s disease. A randomized double blind placebo controlled trial. Lancet Oncol 2014; 15(9): 966–974.