A case of multicentric castleman's disease of mixed-type, which showed constellation of symptoms, i.e., thrombocytopenia, anasarca, anemia, fever, myelofibrosis, and lymphadenopathy.

Multicentric Castleman’s disease (MCD) is a systemic disease characterized by generalized lymphadenopathy and the proliferation of plasma cells. Recently, Takai et al. reported three patients showing a constellation of symptoms ; thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly (TAFRO syndrome). We present a case of MCD developing in a 78-year-old woman with a 17-year history of sarcoidosis, which had several features of TAFRO syndrome. The first part of this patient’s clinical course has already been reported. Here, we report the all parts of the clinical course, from diagnosis to death. The administration of steroid-pulse therapy and prednisolone was ineffective, although treatment with tocilizumab proved highly effective. But 15 months after initiation of tocilizumab, the patient died of disease progression and complications. A 60-year-old woman was diagnosed as sarcoidosis in 1993 according to the criteria by the laboratory data (the elevation of serum angiotensin-converting enzyme to 23.6 IU/L and lysozyme to 10.8 μg/mL) and skin and breast biopsies (non-caseating epithelioid cell granulomas). She was followed by observation without the administration of any treatment but in 2003, she developed third-degree atrioventricular block as a result of cardiac sarcoidosis. A pacemaker was implanted, and again she was followed by observation. In December 2010, at the age of 77, the patient came to our hospital with a slight fever and epigastric pain. Two weeks later, she developed dyspnea and systemic edema and was admitted to our hospital. Her weight had increased from 48.0 to 57.1 kg in one month. A physical examination revealed bilateral cervical and axillary lymph node swelling and legpitting edema ; however, no rheumatological, dermatological, neurological or ophthalmological abnormalities were observed. The laboratory findings showed a white blood cell count of 8,600/μL ; however, the C-reactive protein level had increased to 15.94 mg/dL. A coagulation disorder was evident with a fibrin/fibrinogen degradation product level of 51.3 μg/mL and a D-dimer level of 24.9 μg/mL. While the serum levels of calcium, angiotensin-converting enzyme, lysozyme, IgG and IgG4 were within normal limits, the serum interleukin-6 (IL-6) level was found to have increased slightly to 18 pg/mL. The serum level of vascular endothelial growth factor increased to 69.4 pg/ml. Human herpesvirus-8 (HHV8) DNA was not found in the serum. Table 1 shows additional data. A serological test for human immunodeficiency virus (HIV) was negative, as were both quantiFERON®-TB and tuberculin tests. The arterial blood gas test was unremarkable, and cultures of blood, sputum and the fluids of ascites and pleural effusion were sterile. A closer inspection of the ascites and pleural effusion revealed unremarkable results, except for a marked increase in the IL-6 levels (Fig. 3). Chest radiography showed bilateral hilar lymphadenopathy and dull costophrenic angles ; however, no abnormal opacities were