Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Clinical Considerations on the Pathogenesis of the Plasmacytic Dyscrasias in Man
—With References to Report of Two Cases Who Had Long-term Gangrene of Legs and Recklinghausen's Disease—
Shiro NAKAYAMATakashi HOSHINOMasaichi FUKASE
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1974 Volume 15 Issue 8 Pages 878-886

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Abstract
Two cases are described in which development of plasmacytic dyscrasia was preceded by long-term inflammatory or benign neoplastic disorder.
The first case, a 57-year-old male, had a history of chronic gangrene of the bilateral legs since age of 40, for which his lower legs had to be amputated one by one because of recurrent episodes of intolerably painful necrosis. Despite the operations, however, he continued to have chronic ulcerations in the distal ends of the amputated upper legs. Seventeen years after the onset of gangrene, he was found to be severely anemic, and was diagnosed as plasma cell leukemia by the presences of Bence-Jones proteinuria, M-component of γA type in the serum, and marked proliferation of abnormal plasma cells in the peripheral blood and bone marrow.
The second case was a 58-year-old female who had had Recklinghausen's disease for 45 years. When seen by us, she was complaining of severe low back pain of 5 month's duration, and laboratory examinations revealed paraproteinemia of γG type, proliferation of myeloma cells in the bone marrow and radiolucent bone lesions, which were compatible with a diagnosis of multiple myeloma.
Based on the conception that both of genetic predisposition and protracted stimulation to the reticuloendothelial systems are important factors in the pathogenesis of plasmacytic dyscrasias in man, family history of malignant neoplasmas and principal past histories which may act as chronic irritation to the RES were carefully examined in a series of 47 patients with plasmacytic dyscrasias. The results showed that the incidence of malignancies among the second-degree members of patients with plasmacytic dyscrasias (30%) was apparently higher than that in the second-degree family members of two control diseases, one with leukemia, excluding lymphocytic types, and the other with cardiovascular disease. Furthermore, an apparently larger number of patients with plasmacytic dyscrasias was found to have a positive history of chronic inflammatory, neoplastic, or other chronic disorders and toxic or allergic reactions to drugs as compared with that in the control patients.
From these observations, it is suggested that development of certain plasmacytic dyscrasia in man is initiated or facilitated by protracted stimulation in genetically susceptible or in preconditioned hosts.
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© 1974 The Japanese Society of Hematology
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