A systemic lymphoproliferative disorder with morphologic features of Castleman's disease: clinical findings and clinicopathologic correlations in 15 patients.
Fifteen patients (11 males, four females; median age 57) manifested a disease characterized by (1) the histopathologic features of Castleman's disease, plasma cell type, in lymph node biopsies; (2) predominantly lymphadenopathic disease, involving multiple, preferentially peripheral nodal groups; (3) varied manifestations of multisystemic involvement (such as constitutional symptoms; splenomegaly and hypergammaglobulinemia; elevated ESR, anemia, and thrombocytopenia; hepatomegaly and altered liver function tests (LFTs); signs of renal disease); and (4) idiopathic nature. Two main patterns of evolution were recognized: persistent, with sustained clinical manifestations, and episodic, with recurrent exacerbations and remissions. Seventy-three percent of patients had infectious complications, and 27% developed malignancies. Complete remissions were obtained occasionally with antineoplastic agents and with splenectomy but not with glucocorticosteroids alone. The median survival time is 30 months; 60% of patients have died. Median follow-up in the six surviving patients is 97+ months. A review of 50 cases in the literature revealed similar clinical and laboratory features. Despite some similarities with autoimmune diseases, the main features of this process seem to best fit a hyperplastic-dysplastic lymphoid disorder in a setting of immunoregulatory deficit.
D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000008:administration & dosage; D001772:Blood Cell Count; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D006965:Hyperplasia; D008111:Liver Function Tests; D008198:Lymph Nodes / Q000473:pathology*; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011507:Proteinuria / Q000209:etiology; D012008:Recurrence; D013156:Splenectomy
D000305:Adrenal Cortex Hormones; D000970:Antineoplastic Agents