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Open Access Case report

The first report of adolescent TAFRO syndrome, a unique clinicopathologic variant of multicentric Castleman’s disease

Ikuko Kubokawa1*, Akihiro Yachie2, Akira Hayakawa1, Satoshi Hirase1, Nobuyuki Yamamoto1, Takeshi Mori1, Tomoko Yanai1, Yasuhiro Takeshima1, Eiryu Kyo3, Goichi Kageyama4, Hiroshi Nagai5, Keiichiro Uehara6, Masaru Kojima7 and Kazumoto Iijima1

Author Affiliations

1 Department of Pediatrics, Kobe University Graduate School of Medicine, 7-5-2 Kusunoki-Cho, Chuo-ku, Kobe 650-0017, Japan

2 Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, 13-1 Takaramachi, Kanazawa 920-8641, Japan

3 Department of Pediatrics, Nishiwaki Municipal Hospital, 652-1 Shimo-toda, Nishiwaki 677-0043, Japan

4 Department of Rheumatology, Kobe University Graduate School of Medicine, 7-5-2 Kusunoki-Cho, Chuo-ku, Kobe 650-0017, Japan

5 Department of Dermatology, Kobe University Graduate School of Medicine, 7-5-2 Kusunoki-Cho, Chuo-ku, Kobe 650-0017, Japan

6 Department of Diagnostic Pathology, Kobe University Hospital, 7-5-2 Kusunoki-Cho, Chuo-ku, Kobe 650-0017, Japan

7 Department of Diagnostic and Anatomic Pathology, Dokkyo Medical University School of Medicine, 880 Kitakobayashi, Mibu-machi, Shimotsuga-gun, Tochigi 321-0293, Japan

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BMC Pediatrics 2014, 14:139  doi:10.1186/1471-2431-14-139

Published: 2 June 2014

Abstract

Background

TAFRO syndrome is a unique clinicopathologic variant of multicentric Castleman’s disease that has recently been identified in Japan. It is characterized by a constellation of symptoms: Thrombocytopenia, Anasarca, reticulin Fibrosis of the bone marrow, Renal dysfunction and Organomegaly (TAFRO). Previous reports have shown that affected patients usually respond to immunosuppressive therapy, but the disease sometimes has a fatal course. TAFRO syndrome occurs in the middle-aged and elderly and there are no prior reports of the disease in adolescents. Here we report the first adolescent case, successfully treated with anti-IL-6 receptor antibody (tocilizumab, TCZ) and monitored with serial cytokine profiles.

Case presentation

A 15-year-old Japanese boy was referred to us with fever of unknown origin. Whole body computed tomography demonstrated systemic lymphadenopathy, organomegaly and anasarca. Laboratory tests showed elevated C-reactive protein and hypoproteinemia. Bone marrow biopsy revealed a hyperplastic marrow with megakaryocytic hyperplasia and mild reticulin fibrosis. Despite methylprednisolone pulse therapy, the disease progressed markedly to respiratory distress, acute renal failure, anemia and thrombocytopenia. Serum and plasma levels of cytokines, including IL-6, vascular endothelial growth factor, neopterin and soluble tumor necrosis factor receptors I and II, were markedly elevated. Repeated weekly TCZ administration dramatically improved the patient’s symptoms and laboratory tests showed decreasing cytokine levels.

Conclusion

To our knowledge, this is the first report of TAFRO syndrome in a young patient, suggesting that this disease can occur even in adolescence. The patient was successfully treated with TCZ. During our patient’s clinical course, monitoring cytokine profiles was useful to assess the disease activity of TAFRO syndrome.

Keywords:
Thrombocytopenia; Anasarca; reticulin Fibrosis of the bone marrow; Renal dysfunction; Organomegaly; Tocilizumab; IL-6; VEGF; Neopterin; Soluble TNF-receptors