BMC pediatrics, 2014-06, Vol.14 (1), p.139-139, Article 139
2014
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- Autor(en) / Beteiligte
- Titel
- The first report of adolescent TAFRO syndrome, a unique clinicopathologic variant of multicentric Castleman's disease
- Ist Teil von
- BMC pediatrics, 2014-06, Vol.14 (1), p.139-139, Article 139
- Ort / Verlag
- England: BioMed Central Ltd
- Erscheinungsjahr
- 2014
- Quelle
- SpringerLink
- Beschreibungen/Notizen
- 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. 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. 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.
- Sprache
- Englisch
- Identifikatoren
- ISSN: 1471-2431eISSN: 1471-2431DOI: 10.1186/1471-2431-14-139Titel-ID: cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_4088371
- Format
- –
- Schlagworte
- Acute Kidney Injury - drug therapy, Acute Kidney Injury - etiology, Adolescent, Anemia - drug therapy, Anemia - etiology, Antibodies, Antibodies, Monoclonal, Humanized - therapeutic use, Biopsy, Blood, Bone marrow, Bone Marrow - pathology, C-reactive protein, C-Reactive Protein - analysis, Case Report, Cell growth, Consent, Cytokines - blood, Diagnostic imaging, Drug dosages, Edema - diagnosis, Edema - drug therapy, Herpes viruses, Hospitals, Humans, Hypoproteinemia - diagnosis, Japan, Lymphatic Diseases - diagnosis, Lymphatic Diseases - drug therapy, Male, Medical imaging, Medical research, Medical treatment, Medicine, Medicine, Experimental, Pathogenesis, Pathology, Patients, Pediatrics, Primary Myelofibrosis - diagnosis, Primary Myelofibrosis - drug therapy, Proteins, Respiratory Insufficiency - drug therapy, Respiratory Insufficiency - etiology, Reticulin, Syndrome, Thrombocytopenia - diagnosis, Thrombocytopenia - drug therapy, Tomography, University graduates, Viral antibodies