TAFRO Syndrome in a Patient of South-American Descent
KeywordsCastleman disease, TAFRO syndrome
A 31-year-old Caucasian woman of South-American descent was diagnosed with a variant of multicentric Castleman disease (MCD) that has been reported in Japan as Castleman-Kojima disease. This is a systemic inflammatory disorder known as TAFRO Syndrome which includes thrombocytopenia, polyserositis (ascites/pleural effusion), microcytic anemia, myelofibrosis, fever, renal dysfunction and organomegaly, with immunologic disorder, polyclonal hypergammaglobulinemia, and elevated levels of interleukin-6 (IL-6) and the vascular endothelial growth factor present in serum and/or effusions. Optimal therapies are not well established. The patient was treated with methylprednisolone and rituximab. Following the start of treatment, the patient has been asymptomatic for over 8 months.
This is one of only a few reports of TAFRO Syndrome in a non-Japanese patient.
Castleman B, Towne VW. Case records of the Massachusetts General Hospital. Case 40231. NEJM 1954;23:1001-1005.
Castleman B, Iverson L, Menendez VP. Localized mediastinal lymphnode hyperplasia resembling thymoma. Cancer 1956;9:822–30.
Jongsma TEF, Verburg RJ, Geelhoed-Duijvestijn PHLM. Castleman's disease: A rare lymphoproliferative disorder. Eur J of Intern Med 2007;18:87–89.
Kojima M, Shimizu K, Ikota H, Ohno Y, Motoori T, Itoh H, et al. ‘‘Follicular variant’’ of hyaline-vascular type of Castleman’s disease: histopathological and immunohistochemical study of 11 cases. J Clin Exp Hematop 2008;48:39–45.
Kojima M, Nakamura N, Tsukamoto N, Yokohama A, Kobayashi S, Kashimura M. Multicentric Castleman’s disease representing effusion at initial clinical presentation: clinicopathological study of seven cases. Lupus 2011;20:44–50.
Talat N, Schulte KM. Castleman’s disease: systematic analysis of 416 patients from the literature. Oncologist 2011;16:1316–24.
Robinson D, Casper C, Vermeulen J, Reynolds M, Dispenzieri A, Payne K, et al. Clinical epidemiology and treatment patterns of patients with multicentric Castleman disease: results from two US treatment centres. Br J Haematol 2014;165:39–48.
Muzes G, Sipos F, Csomor J, Sréter L. Multicentric Castleman’s Disease: A Challenging Diagnosis. Pathol Oncol Res 2013,19:345–351.
Soumerai JD, Sohani AR, Abramson JS. Diagnosis and Management of Castleman Disease. Cancer Control 2014;21:266-278.
Mian H, Leber B. Mixed variant multicentric Castleman disease treated with Rituximab: case report. J Pediatr Hematol Oncol 2010;32:622.
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 Meeting (22 September, 2012). J Clin Exp Hematop 2014;2:163-166.
Koduri PR, Parvez M, Kaza S, Pappu P,Anuradha S. Castleman-Kojima Disease in South Asian Adolescent. J Clin Exp Hematop 2013;53:57-61.
Marcelin AG, Aaron L, Mateus C, Gyan E, Gorin I, Viard JP, et al. Rituximab therapy for HIV-associated Castleman's disease. Blood 2003, 102:2786-2788.
Kojima M, Nakamura N, Tsukamoto N, Otuski Y, Shimizu K et al. Clinical implications of Multicentric Castleman’s disease among Japanese. A report of 28 cases. Int J Surg Pathol 2008;16:391-398.
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;1:79-85.
Tedesco S, Postacchini L, Manfredi L, Goteri G, Luchetti MM, Festa A et al. Successful treatment of a Caucasian case of multifocal Castleman's disease with TAFRO syndrome with a pathophysiology targeted therapy - a case report. Exp Hematol Oncol 2015;4:3.
Robinson D, Casper C, Vermeulen J, Reynolds M, Dispenzieri A, Payne K et al. Clinical epidemiology and treatment patterns of patients with multicentric Castleman disease: results from two US treatment centres. Br J of Haematol 2014;165:39–48.
Zhu SH, Sun JJ, Yu YH, Han DL, Li J, Zhang Y. Clinical features and outcome of patients with HIV-negative multicentric Castleman’s disease treated with combination chemotherapy: a report on 10 patients. Med Oncol 2013;30:492.
Issue: Vol. 2 No. 4 (2015) (view)