title: Aggressive TAFRO syndrome with reversible cardiomyopathy successfully treated with combination chemotherapy.
abstract: TAFRO (thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly) syndrome is an atypical manifestation of Castleman's disease. However, the mechanism underlying this very rare syndrome remains unknown, and there is no established standard treatment. Here we report cases of two young females with TAFRO syndrome who showed similar clinical courses. Both cases showed severe anasarca, ascites, and thrombocytopenia. Although high-dose steroids were ineffective, combination chemotherapy showed remarkable effects. However, both patients developed severe but reversible heart failure after CHOP therapy owing to diffuse cardiomyopathy, which was presumably associated with TAFRO syndrome. Therefore, although combination chemotherapy may be very effective in the treatment of TAFRO syndrome, careful observation for cardiomyopathy development is needed, particularly when using adriamycin-containing regimens.
authors.affiliation: Department of Hematology, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan.
authors.forename: Shunichiro
authors.initials: S
authors.lastname: Yasuda
authors.affiliation: Department of Hematology, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan.
authors.forename: Keisuke
authors.initials: K
authors.lastname: Tanaka
authors.affiliation: Department of Hematology, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan.
authors.forename: Ayako
authors.initials: A
authors.lastname: Ichikawa
authors.affiliation: Department of Hematology, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan.
authors.forename: Ken
authors.initials: K
authors.lastname: Watanabe
authors.affiliation: Department of Hematology, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan.
authors.forename: Emi
authors.initials: E
authors.lastname: Uchida
authors.affiliation: Department of Hematology, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan.
authors.forename: Masahide
authors.initials: M
authors.lastname: Yamamoto
authors.affiliation: Department of Comprehensive Pathology, Tokyo Medical and Dental University, Tokyo, Japan.
authors.forename: Kouhei
authors.initials: K
authors.lastname: Yamamoto
authors.affiliation: Department of Hematology, Tokyo Teishin Hospital, Tokyo, Japan.
authors.forename: Daisuke
authors.initials: D
authors.lastname: Mizuchi
authors.affiliation: Department of Hematology, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan.
authors.forename: Osamu
authors.initials: O
authors.lastname: Miura
authors.affiliation: Department of Hematology, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan. fuku.hema@tmd.ac.jp.
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Fukuda
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D009202:Cardiomyopathies / Q000209:etiology*; D005871:Castleman Disease / Q000150:complications / Q000188:drug therapy*; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D005260:Female; D006333:Heart Failure; D006801:Humans; D011241:Prednisone / Q000627:therapeutic use; D013577:Syndrome; D014750:Vincristine / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone
keywords: Adriamycin; Cardiomyopathy; Combination chemotherapy; IL-6; TAFRO syndrome