We report five cases that presented with high
fever,
anasarca, hepatosplenomegaly and severe
thrombocytopenia with
reticulin fibrosis of the bone marrow. The constellation of symptoms is not compatible with any known disease, and we had difficulty in diagnosis and treatment. The age distribution was from 47 to 56 years, and two men and three women were affected. Two patients needed
hemodialysis because of renal dysfunction and
oliguria with massive
pleural effusion. Laboratory examinations showed normal
immunoglobulin levels and no monoclonal
protein. None of them showed diagnostic
autoantibodies for any
autoimmune diseases. Histological examination of the liver in three patients and spleen in two showed non-specific findings.
Lymphadenopathy was tiny and lymph node biopsy was carried out in only one case. Histologically, paracortical
hyperplasia with vascular proliferation and atrophic germinal centers resembling hyaline-vascular-type
Castleman's disease or
POEMS syndrome were detected. Without a definitive diagnosis, treatment was started with
cyclophosphamide, hydroxydaunorubicin,
vincristine and
prednisolone (
CHOP) regimen in one patient, semi-pulse
therapy with methyl-predonisolone in three and
cyclosporin A in three. Two patients achieved complete remission, two were
steroid-dependent and the remaining one died of
multiple organ failure. These findings suggest that this disease may be a novel clinical entity belonging to systemic inflammatory disorder with a background of immunological abnormality or a unique variant of
multicentric Castleman's disease. [J Clin Exp Hematop 53(1): 63-68, 2013].