[Multicentric Castleman's disease with lymphoid interstitial pneumonia died of aggressive course with adult respiratory distress syndrome].

A 49-year-old man was admitted to our hospital with anemia and hypergammaglobulinemia. Physical examination revealed superficial lymph node swelling and no hepatosplenomegaly. Laboratory findings showed WBC 5,300/microliters with normal hemogram, microcytic and hypochromic anemia. Total protein was 11.5 g/dl and immunoglobulinemia (IgG 10,100 mg/dl, IgA 295 mg/dl, IgM 160 mg/dl) was observed without M-component in serum and urine. The CD4/CD8 ratio of lymphocyte subsets was 0.58 and the tuberuculin skin test was negative. Urinary protein was positive and renal biopsy disclosed plasma cell infiltration. Lymph node biopsy revealed multiple lymphoid follicles and infiltration of plasma cells in the interfollicular areas. A diagnosis of multicentric Castleman's disease (MCD) was made baredon clinical findings and lymph node biopsy. After therapy with plasmapheresis and the CHOP regimen, he was given etoposide. Although discharged with clinical improvement and a decrease of serum IgG, he was readmitted because of pyrexia after 4 days and died of pneumonia with adult respiratory distress syndrome. The autopsy revealed lymphoid interstitial pneumonia. It seems important to notice that some of MCD have poor prognoses because of accompanying immunodeficiency.