#PAGE_PARAMS# #ADS_HEAD_SCRIPTS# #MICRODATA#

Our experience in treatment of multicentric plasma-cell Castleman disease associated with vasculitis manifestations – case report and literature review


Authors: P. Szturz 1;  Z. Adam 1;  M. Moulis 2;  L. Šmardová 1;  M. Klincová 3;  R. Šlaisová 4;  R. Koukalová 5;  Z. Řehák 5;  P. Volfová 1;  J. Chovancová 3;  O. Stehlíková 1;  J. Mayer 1
Authors‘ workplace: Interní hematologická a onkologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Mayer, CSc. 1;  Ústav patologie Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta doc. MUDr. Josef Feit, CSc. 2;  Lékařská fakulta Masarykovy univerzity, Brno, děkan prof. MUDr. Jiří Mayer, CSc. 3;  Radiologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Vlastimil Válek, CSc. 4;  Oddělení nukleární medicíny PET Centra Masarykova onkologického ústavu Brno, přednosta prim. MUDr. Karol Bolčák 5
Published in: Vnitř Lék 2012; 58(9): 679-690
Category: Case Reports

Overview

Castleman disease is a rare idiopathic non-neoplastic lymphoproliferative disorder with 2 clinical (unicentric and multicentric) and 3 histomorphological (hyaline-vascular, plasma-cell and mixed) forms identified. The case report given here describes the 3-year experience with therapy in a patient, male born 1961, diagnosed with multicentric plasma-cell Castleman disease (HIV and HHV-8 negative) with the finding of genera­lized lymphadenopathy and splenomegaly. During first line treatment (R-CHOP: rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone, 3 cycles in total, 12/2008–2/2009) the development of bilateral upper and lower limb edemas with clinical manifestation of vasculitis occurred and a restaging computed tomography (CT) examination revealed a stable finding of the lymphadenomegaly. Greater success was achieved with thalidomide regimen (CTD: cyclophosphamide, thalidomide, dexamethasone, 10 cycles, 3/2009–1/2010) leading to reduction in the size of the hypervascularized lymph nodes (almost by 50%) as well as their radiopharmaceutical (fluorodeoxyglucose) uptake as seen on a combined positron emission tomography and computed tomography (PET/CT) scan imaging. Thalidomide was given daily at doses between 100 and 200 mg. We returned to the CTD regimen again in April 2010 after a short period of monoclonal antibody tocilizumab treatment (400 mg intravenous in 2-week intervals with 50% dose reduction due to a limited supply of the drug, 5 doses in total) during which edemas reoccurred with a CT scan finding of stable lymphadenomegaly. However, the renewed regimen with thalidomide was stopped after 2.5 cycles due to adverse effects of thalidomide (neuropathy) and corticoids (Cushing syndrome). In September 2010, after enrollement in the Celgene’s Compassionate Use Program we were able to start treating the patient with the derivative of thalidomide, lenalidomide, at a dosage of 25 mg on days 1–21 in a 28-day cycle, 15 cycles in total (10/2010–12/2011). The monotherapy with lenalidomide was very well tolerated by the patient without any effects of myelotoxicity, thromboembolism or relapses of edemas and vasculitis, additionally now with apparent improvement of fatic disorder and the patient’s motor abilities. Thus, lenalidomide represents an attractive alternative agent for patients with Castleman disease after rituximab and cytostatics failures. It has a favourable safety profile and could be therefore considered for administering in first line treatment.

Key words:
Castleman disease – glucocorticoids – chemotherapy – rituximab – thalidomide – monoclonal antibody – tocilizumab – lenalidomide – positron emission tomography – computed tomography


Sources

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

2. Van Rhee F, Stone K, Szmania S et al. Castleman disease in the 21st century: an update on diagnosis, assessment, and therapy. Clin Adv Hematol Oncol 2010; 8: 486–498.

3. Castleman B, Towne VW. Case records of the Massachusetts General Hospital; weekly clinicopathological exercises; founded by Richard C. Cabot. N Engl J Med 1954; 251: 396–400.

4. Castleman B, Iverson L, Menendez VP. Localized mediastinal lymphnode hyperplasia resembling thymoma. Cancer 1956; 9: 822–830.

5. 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; 29: 670–683.

6. Flendrig JA, Schillings PM. Benign giant lymphoma: the clinical signs and symptoms. Folia Medica Neerlandica 1969; 12: 119–120.

7. Casper C. The aetiology and management of Castleman disease at 50 years: translating pathophysiology to patient care. Br J Haematol 2005; 129: 3–17.

8. Moore DF, Preti A, Tran SM. Prognostic implications following an indeterminate diagnostic work-up of lymphoma. Blood 1996; 88 (Suppl 1): 229a.

9. Ye B, Gao SG, Li W et al. A retrospective study of unicentric and multicentric Castleman’s disease: a report of 52 patients. Med Oncol 2010; 27: 1171–1178.

10. Kessler P. Profylaxe a léčba tromboembolické nemoci v onkologii. Vnitř Lék 2009; 55: 219–222.

11. Choi JH, Jo YJ, Gong SJ et al. Unicentric Castleman disease is not clearly distinguished from multicentric type: a case report. Clin Lymphoma Myeloma 2008; 8: 256–259.

12. Danon AD, Krishnan J, Frizzera G. Morpho--immunophenotypic diversity of Castleman’s disease, hyaline-vascular type: with emphasis on a stroma-rich variant and a new pathogenetic hypothesis. Virchows Arch A Pathol Anat Histopathol 1993; 423: 369–382.

13. Ioachim HL, Medeiros LJ. Ioachim’s lymph node pathology. Philadelphia: Lippincott Williams & Wilkins 2008.

14. McClain KL, Natkunam Y, Swerdlow SH. Atypical cellular disorders. Hematology Am Soc Hematol Educ Program 2004: 283–296.

15. McCarty MJ, Vukelja SJ, Banks PM et al. Angiofollicular lymph node hyperplasia (Castleman’s disease). Cancer Treat Rev 1995; 21: 291–310.

16. Dispenzieri A. Castleman disease. In: Ansell SM (ed.). Rare Hematological Malignancies. Boston: Springer Science + Business Media 2008: 293–330.

17. Leger-Ravet MB, Peuchmaur M, Devergne O et al. Interleukin-6 gene expression in Castleman’s disease. Blood 1991; 78: 2923–2930.

18. Yoshizaki K, Matsuda T, Nishimoto N et al. Pathogenic significance of interleukin-6 (IL-6//BSF-2) in Castleman’s disease. Blood 1989; 74: 1360–1367.

19. Hsu SM, Waldron JA, Xie SS et al. Expression of interleukin-6 in Castleman’s disease. Hum Pathol 1993; 24: 833–839.

20. Oksenhendler E, Carcelain G, Aoki Y et al. High levels of human herpesvirus 8 viral load, human interleukin-6, interleukin-10, and C reactive protein correlate with exacerbation of multicentric castleman disease in HIV-infected pa­tients. Blood 2000; 96: 2069–2073.

21. Gloddek J, Pagotto U, Paez Pereda M et al. Pituitary adenylate cyclase-activating polypeptide, interleukin-6 and glucocorticoids regulate the release of vascular endothelial growth factor in pituitary folliculostellate cells. J Endocrinol 1999; 160: 483–490.

22. van Kooten C, Rensink I, Aarden L et al. Effect of IL-4 and IL-6 on the proliferation and differentiation of B-chronic lymphocytic leukemia cells. Leukemia 1993; 7: 618–624.

23. Vinzio S, Ciarloni L, Schlienger JL et al. Isolated microcytic anemia disclosing a unicentric Castleman disease: The interleukin-6/hepcidin pathway? Eur J Intern Med 2008; 19: 367–369.

24. Nemeth E, Valore EV, Territo M et al. Hepcidin, a putative mediator of anemia of inflammation, is a type II acute-phase protein. Blood 2003; 101: 2461–2463.

25. Nemeth E, Rivera S, Gabayan V et al. IL-6 mediates hypoferremia of inflammation by inducing the synthesis of the iron regulatory hormone hepcidin. J Clin Invest 2004; 113: 1271–1276.

26. Ganz T. Hepcidin – a regulator of intestinal iron absorption and iron recycling by macrophages. Best Pract Res Clin Haematol 2005; 18: 171–182.

27. Kawabata H, Tomosugi N, Kanda J et al. Anti-interleukin 6 receptor antibody tocilizumab reduces the level of serum hepcidin in patients with multicentric Castleman’s disease. Haematologica 2007; 92: 857–858.

28. 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; 269: 335–344.

29. Moore PS, Boshoff C, Weiss RA et al. Molecular mimicry of human cytokine and cytokine response pathway genes by KSHV. Science 1996; 274: 1739–1744.

30. Veldhuis GJ, van der Leest AH, de Wolf JT et al. A case of localized Castleman’s disease with systemic involvement: treatment and pathogenetic aspects. Ann Hematol 1996; 73: 47–50.

31. Nishimoto N, Kanakura Y, Aozasa K et al. Humanized anti-interleukin-6 receptor antibody treatment of multicentric Castleman disease. Blood 2005; 106: 2627–2632.

32. Bowne WB, Lewis JJ, Filippa DA et al. The management of unicentric and multicentric Castleman’s disease: a report of 16 cases and a review of the literature. Cancer 1999; 85: 706–717.

33. Gaba AR, Stein RS, Sweet DL et al. Multicentric giant lymph node hyperplasia. Am J Clin Pathol 1978; 69: 86–90.

34. Ko SF, Wan YL, Ng SH et al. Imaging features of atypical thoracic Castleman disease. Clin Imaging 2004; 28: 280–285.

35. Gangopadhyay K, Mahasin ZZ, Kfoury H. Pathologic quiz case 2. Castleman disease (giant lymph node hyperplasia). Arch Otolaryngol Head Neck Surg 1997; 123: 1137–1139.

36. Johkoh T, Müller NL, Ichikado K et al. Intrathoracic multicentric Castleman disease: CT findings in 12 patients. Radiology 1998; 209: 477–481.

37. Herrada J, Cabanillas F, Rice L et al. The clinical behavior of localized and multicentric Castleman disease. Ann Intern Med 1998; 128: 657–662.

38. Frizzera G. Castleman’s disease and related disorders. Semin Diagn Pathol 1988; 5: 346–364.

39. Menke DM, Camoriano JK, Banks PM. Angio­follicular lymph node hyperplasia: a comparison of unicentric, multicentric, hyaline vascular, and plasma cell types of disease by morphometric and clinical analysis. Mod Pathol 1992; 5: 525–530.

40. Frizzera G, Peterson BA, Bayrd ED et al. A systemic lymphoproliferative disorder with morphologic features of Castleman’s disease: clinical findings and clinicopathologic correlations in 15 patients. J Clin Oncol 1985; 3: 1202–1216.

41. Peterson BA, Frizzera G. Multicentric Castleman’s disease. Semin Oncol 1993; 20: 636–647.

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

43. Sagaert X, De Wolf-Peeters C. De ziekte van Castleman: twee afzonderlijke ziekte-entiteinten. Tijdschr Geneesk 2004; 60: 949–956.

44. Bower M, Powles T, Williams S et al. Brief communication: rituximab in HIV-associated multicentric Castleman disease. Ann Intern Med 2007; 147: 836–839.

45. Feigert JM, Sweet DL, Coleman M et al. Multi­centric angiofollicular lymph node hyperplasia with peripheral neuropathy, pseudotumor cerebri, IgA dysproteinemia, and thrombocytosis in women. A distinct syndrome. Ann Intern Med 1990; 113: 362–367.

46. Bitter MA, Komaiko W, Franklin WA. Giant lymph node hyperplasia with osteoblastic bone lesions and the POEMS (Takatsuki’s) syndrome. Cancer 1985; 56: 188–194.

47. Mandler RN, Kerrigan DP, Smart J et al. Castleman’s disease in POEMS syndrome with elevated interleukin-6. Cancer 1992; 69: 2697–2703.

48. Bélec L, Mohamed AS, Authier FJ et al. Human herpesvirus 8 infection in patients with POEMS syndrome-associated multicentric Castleman’s disease. Blood 1999; 93: 3643–3653.

49. Wang SH, Ruan Z, Huang HL et al. A rare case of Castleman disease presenting as pulmonary mass mimicking central pulmonary malignancy. Chin Med J (Engl) 2009; 122: 990–991.

50. Halac M, Ergul N, Sager S et al. PET/CT findings in a multicentric form of Castleman’s disease. Hell J Nucl Med 2007; 10: 172–174.

51. Murphy SP, Nathan MA, Karwal MW. FDG-PET appearance of pelvic Castleman’s disease. J Nucl Med 1997; 38: 1211–1212.

52. Leskinen-Kallio S, Ruotsalainen U, Någren K et al. Uptake of carbon-11-methionine and fluorodeoxyglucose in nehodgkin’s lymphoma: a PET study. J Nucl Med 1991; 32: 1211–1218.

53. Mohanna S, Sanchez J, Ferrufino JC et al. Characteristics of Castleman’s disease in Peru. Eur J Intern Med 2006; 17: 170–174.

54. Seco JL, Velasco F, Manuel JS et al. Retroperitoneal Castleman’s disease. Surgery 1992; 112: 850–855.

55. Larroche C, Cacoub P, Godeau P. Castle­man’s disease. Rev Med Interne 1996; 17: 1003–1013.

56. Ebisuno S, Yamauchi T, Fukatani T et al. Retroperitoneal Castleman’s disease: a case report and brief review of tumors of the pararenal area. Urol Int 1989; 44: 169–172.

57. Takihara H, Yamakawa G, Baba Y et al. Castle­man disease. Unusual retroperitoneal location indistinguishable from malignant tumor in preoperative angiographic appearance. Urology 1993; 41: 162–164.

58. Kiguchi H, Ishii T, Ishikawa Y et al. Castleman’s disease of the abdomen and pelvis: report of three cases and a review of the literature. J Gastroenterol 1995; 30: 661–666.

59. Bartkowski DP, Ferrigni RG. Castleman’s disease: an unusual retroperitoneal mass. J Urol 1988; 139: 118–120.

60. Skolnik G, Wiklund LM, Risberg B. Castle­man’s tumor with retroperitoneal location: a malignant-appearing benign tumor. J Surg Oncol 1985; 28: 153–155.

61. d’Agay MF, Miclea JM, Clauvel JP et al. Castleman’s disease: a well defined histological pattern for a widely divergent clinical spectrum. Nouv Rev Fr Hematol 1989; 31: 145–148.

62. Nordstrom DG, Tewfik HH, Latourette HB. Giant lymph node hyperplasia: a review of literature and report of two cases of plasma cell va­riant responding to radiation therapy. Int J Radiat Oncol Biol Phys 1978; 4: 1045–1048.

63. Adam Z, Krejčí M, Vorlíček J. Hematologie: přehled maligních hematologických nemocí. Praha: Grada Publishing 2008.

64. Estephan FF, Elghetany MT, Berry M et al. Complete remission with anti-CD20 therapy for unicentric, non-HIV-associated, hyaline-vascular type, Castleman’s disease. Cancer Invest 2005; 23: 191.

65. Bandera B, Ainsworth C, Shikle J et al. Treatment of unicentric Castleman disease with neoadjuvant rituximab. Chest 2010; 138: 1239–1241.

66. Marti S, Pahissa A, Guardia J et al. Multicentric giant follicular lymph node hyperplasia. Favorable response to radiotherapy. Cancer 1983; 51: 808–810.

67. Sethi T, Joshi K, Sharma SC et al. Radiation therapy in the management of giant lymph node hyperplasia. Br J Radiol 1990; 63: 648–650.

68. Matsuyama M, Suzuki T, Tsuboi H et al. Anti-interleukin-6 receptor antibody (tocilizumab) treatment of multicentric Castleman’s disease. Intern Med 2007; 46: 771–774.

69. Nishimoto N, Sasai M, Shima Y et al. Improvement in Castleman’s disease by humanized anti-interleukin-6 receptor antibody therapy. Blood 2000; 95: 56–61.

70. Beck JT, Hsu SM, Wijdenes J et al. Brief report: alleviation of systemic manifestations of Castleman’s disease by monoclonal anti-interleukin-6 antibody. N Engl J Med 1994; 330: 602–605.

71. Ide M, Ogawa E, Kasagi K et al. Successful treatment of multicentric Castleman’s disease with bilateral orbital tumour using rituximab. Br J Haematol 2003; 121: 818–819.

72. Gholam D, Vantelon JM, Al-Jijakli A et al. A case of multicentric Castleman’s disease associated with advanced systemic amyloidosis treated with chemotherapy and anti-CD20 monoclonal antibody. Ann Hematol 2003; 82: 766–768.

73. Ocio EM, Sanchez-Guijo FM, Diez-Campelo M et al. Efficacy of rituximab in an aggressive form of multicentric Castleman disease associated with immune phenomena. Am J Hematol 2005; 78: 302–305.

74. 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: 2224–2226.

75. Adam Z, Krejčí M, Tichý M et al. Léčba selhání ledvin u mnohočetného myelomu. Vnitř Lék 2009; 55: 570–582.

76. 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: 201–204.

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

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

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

80. 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: 352–356.

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

82. Palumbo A, Rajkumar SV. Multiple myeloma: chemotherapy or transplantation in the era of new drugs. Eur J Haematol 2010; 84: 379–390.

83. Vallet S, Palumbo A, Raje N et al. Thalidomide and lenalidomide: Mechanism-based potential drug combinations. Leuk Lymphoma 2008; 49: 1238–1245.

84. Szturz P, Adam Z, Rehák Z et al. Lenalidomide proved effective in multisystem Langerhans cell histiocytosis. Acta Oncol 2012; 51: 412–415.

85. Casper C, Nichols WG, Huang ML et al. Remission of HHV-8 and HIV-associated multicentric Castleman disease with ganciclovir treatment. Blood 2004; 103: 1632–1634.

86. Oksenhendler E, Duarte M, Soulier J et al. Multicentric Castleman’s disease in HIV infection: a clinical and pathological study of 20 patients. AIDS 1996; 10: 61–67.

87. Loi S, Goldstein D, Clezy K et al. Castleman’s disease and HIV infection in Australia. HIV Med 2004; 5: 157–162.

88. Kumari P, Schechter GP, Saini N et al. Successful treatment of human immunodeficiency virus-related Castleman’s disease with interferon-alpha. Clin Infect Dis 2000; 31: 602–604.

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

90. 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: 176–177.

91. Lachmann HJ, Gilbertson JA, Gillmore JD et al. Unicentric Castleman’s disease complicated by systemic AA amyloidosis: a curable disease. QJM 2002; 95: 211–218.

92. Weisenburger DD, Nathwani BN, Winberg CD et al. Multicentric angiofollicular lymph node hyperplasia: a clinicopathologic study of 16 cases. Hum Pathol 1985; 16: 162–172.

93. Oksenhendler E, Duarte M, Soulier J et al. Multicentric Castleman’s disease in HIV infection: a clinical and pathological study of 20 patients. AIDS 1996; 10: 61–67.

94. 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; 95: 1406–1412.

Labels
Diabetology Endocrinology Internal medicine
Topics Journals
Login
Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account

#ADS_BOTTOM_SCRIPTS#