Hodgkin's disease with predominant infradiaphragmatic involvement and massive invasion of the bone marrow. A necropsic study of nine cases.
The authors report the results obtained in the necropsic study of nine cases of the so-called acute, visceral form of Hodgkin's disease (HD). Most of the patients (six men and three women, ranging from 42 to 74 years of age) lacked peripheral lymphadenopathies and had fever, weight loss, abnormality of hepatic function, and pancytopenia. Mixed cellularity was diagnosed in two, diffuse fibrosis in four, and reticular subtype of lymphocyte depletion in three cases. Despite the predominant infradiaphragmatic involvement, supradiaphragmatic lymph nodes were involved in six and tonsils in three cases. Spleen and bone marrow were involved in eight cases and the liver was involved in seven cases. In four cases there were also lesions in other extralymphoid organs. The involvement of the bone marrow was widespread and showed concurrent myelofibrosis and/or other signs of hematopoietic disturbance. There was a close relationship between the presence of vascular invasion (seven cases) and the extent of HD spread. It is concluded that despite its peculiarity, this form of HD fits the classic model of unicentric origin, lymphogenic contiguous spread, and hematogeneous dissemination, and should not be identified with any particular histologic type of HD.
D000328:Adult; D000368:Aged; D001344:Autopsy; D001835:Body Weight; D001853:Bone Marrow / Q000473:pathology*; D003964:Diaphragm; D005260:Female; D005334:Fever; D006689:Hodgkin Disease / Q000473:pathology*; D006801:Humans; D008099:Liver / Q000473:pathology; D008198:Lymph Nodes / Q000276:immunology / Q000473:pathology; D008212:Lymphocyte Depletion; D008297:Male; D008875:Middle Aged; D009135:Muscular Diseases / Q000473:pathology; D013154:Spleen / Q000473:pathology