A 76-year-old man presented with shortness of breath and the laboratory tests suggested anemia and reticulocytopenia. CBC showed only anemia, and the bone marrow aspiration smear demonstrated absence of erythroid hematopoietic cells. Consequently, pure red cell aplasia (PRCA) was diagnosed. Computed tomography (CT) showed mediastinal multiple lymph node enlargement and ground-glass opacity in both the lung fields. Biopsy specimens of the mediastinal lymph node showed mild follicular hyperplasia and polyclonal plasma cells proliferation in the interfollicular area. These findings suggest idiopathic multicentric Castleman disease plasma cell type (iMCD PC type). Ciclosporin (CyA) was administered but there was no clinical improvement after 6 weeks of therapy. Therefore, prednisolone (PSL) was started at 0.5 mg/kg/day and was very effective for the PRCA and MCD. A total of 3 cases of CD (2 cases of MCD PC type and 1 case of CD HV type) with PRCA have been previously reported. In the 2 cases of MCD PC type, anemia was improved using PSL combination therapy. However, in the single case of CD HV type, PSL was not effective and anemia was improved with CyA treatment. This case suggests the possibility of using PSL as the first-line drug for MCD PC type with PRCA.
Keywords: Castleman disease; Ciclosporin; Pure red cell aplasia.