Successful treatment of a Caucasian case of multifocal Castleman’s disease with TAFRO syndrome with a pathophysiology targeted therapy - a case report

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• 作者：Silvia Tedesco (4)
  Laura Postacchini (4)
  Lucia Manfredi (4)
  Gaia Goteri (5)
  Michele M Luchetti (4) (6)
  Antonella Festa (6)
  Armando Gabrielli (4) (6)
  Giovanni Pomponio (6)
  
  4. Clinica Medica - Dipartimento di Scienze Cliniche e Molecolari ; Università Politecnica delle Marche ; Via Conca 71 ; 60126 ; Ancona ; Italy
  5. Anatomia Patologica - Dipartimento di Scienze Cliniche e Molecolari ; Università Politecnica delle Marche ; Via Conca 71 ; 60126 ; Ancona ; Italy
  6. Clinica Medica -Ospedali Riuniti di Ancona ; Via Conca 71 ; 60126 ; Ancona ; Italy
  
• 关键词：Castleman’s disease ; Multicentric ; TAFRO syndrome ; Tocilizumab ; Rituximab ; Chemotherapy ; PRES
• 刊名：Experimental Hematology & Oncology
• 出版年：2015
• 出版时间：December 2015
• 年：2015
• 卷：4
• 期：1
• 全文大小：59 KB
• 参考文献：1. Kawabata H, Takai K, Kojima M, Nakamura N, Aoki S, Nakamura S, / 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, 53:57-1. 10.3960/jslrt.53.57 CrossRef
  2. Thiele J, Kvasnicka HM, Facchetti F, Franco V, van der Walt J, Orazi A: European consensus on grading bone marrow fibrosis and assessment of cellularity. / Haematologica 2005,90(8):1128-2.
  3. Baserga M, Rosin M, Schoen M, Young G: Multifocal Castleman disease in pediatrics: case report. / J Pediatr Hematol Oncol 2005, 27:666-. 10.1097/01.mph.0000193468.06938.93 CrossRef
  4. Kubokawa I, Yachie A, Hayakawa A, Hirase S, Yamamoto N, Mori T, / et al.: The first report of adolescent TAFRO syndrome, a unique clinicopathologic variant of multicentric Castleman’s disease. / BMC pediatrics 2014, 14:139. 10.1186/1471-2431-14-139 CrossRef
  5. Masaki Y, Nakajima A, Iwao H, Kurose N, Sato T, Nakamura T, / 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, 53:79-5. 10.3960/jslrt.53.79 CrossRef
  6. Kawabata H, Kotani S, Matsumura Y, Kondo T, Katsurada T, Haga H, / et al.: Successful treatment of a patient with multicentric Castleman's disease who presented with thrombocytopenia, ascites, renal failure and myelofibrosis using Tocilizumab, an anti-interleukin-6 receptor antibody. / Intern Med 2013, 52:1503-. 10.2169/internalmedicine.52.9482 CrossRef
  7. Iwaki N, Sato Y, Takata K, Kondo E, Ohno K, Takeuchi M, / et al.: Atypical hyaline vascular-type castleman's disease with thrombocytopenia, anasarca, fever, and systemic lymphadenopathy. / J Clin Exp Hematop 2013, 53:87-3. 10.3960/jslrt.53.87 CrossRef
  8. Inoue M, Ankou M, Hua J, Iwaki Y, Hagihara M: 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, 53:95-. 10.3960/jslrt.53.95 CrossRef
  9. Ozawa T, Kosugi S, Kito M, Onishi M, Kida T, Nakata S, / et al.: Efficacy of Rituximab for TAFRO syndrome, a variant type of multicentric Castleman's disease. / Rinsho Ketsueki 2014, 55:350-.
  10. Kawabata H, Kadowaki N, Nishikori M, Kitawaki T, Kondo T, Ishikawa T, / 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, 53:69-7. 10.3960/jslrt.53.69 CrossRef
  11. Van Rhee F, Stone K, Szmania S, Barlogie B, Singh Z: Castleman disease in the 21st century: an update on diagnosis, assessment, and therapy. / Clin Adv Hematol Oncol 2010, 8:486-8.
  12. Ye B, Gao SG, Li W, Yang LH, Zhao SH, Ma K, / et al.:
• 刊物主题：Hematology; Oncology;
• 出版者：BioMed Central
• ISSN：2162-3619

文摘

Background Castleman-Kojima disease (TAFRO Syndrome) is characterized by Thrombocytopenia, Anasarca, myeloFibrosis, Renal dysfunction, Organomegaly, multiple lymphadenopathy and histopathology pattern of atypical Castleman’s disease (CD). Only few cases of this recently identified unique variant of Multicentric CD (MCD) are described in literature, all Japanese. It therefore poses serious diagnostic and therapeutic challenges. Case description We describe a 21?year old woman with fever, asthenia, bilateral pleural effusion, ascites, hypoalbuminemia, severe thrombocytopenia, anemia, renal failure and proteinuria, whereas microbiological tests, immune serology (except ANA) and bone marrow biopsy were all negative. A CT-scan showed multiple lymphadenopathy and tissue samplings of mediastinal lymph nodes was compatible with a mixed-type CD. The diagnosis of MCD with TAFRO syndrome was made, but after an initial improvement with high dose corticosteroid therapy, clinical and laboratory features worsened. Based upon the high serum IL-6 levels and the high number of CD20-lymphocytes in lymph nodes tissue, we started tocilizumab (partial benefit), followed by rituximab combined with CVP (cyclophosphamide, vincristine and prednisone) chemotherapy, achieving a complete response. A total of six cycles of R-CVP were administered monthly, followed by maintenance with monthly rituximab. A complete remission persists at the 12th month of follow-up. Conclusions In patients with massive immune system activation and lymphadenopathy it is mandatory to rule out Castleman-Kojima disease. In our patient a therapy aimed at the prominent pathophysiological abnormalities has been successful so far. However, since the rarity of TAFRO Syndrome, a multicenter registry is strongly desirable for a better understanding of the disease mechanisms, hopefully leading to evidence-based therapeutic choices.