A Case of Waldenström's Macroglobulinemia with Cold Agglutinin Disease
1986 Volume 27 Issue 3 Pages 376-380
Details
1986 Volume 27 Issue 3 Pages 376-380
An 82-year-old man admitted to our hospital with complaints of pyrexia and dyspnea in January, 1984. He had had exertional dyspnea and acrocyanosis on cold stimulation for several years.
Examination revealed the following abnormalities to establish a diagnosis of Waldenström's macroglobulinemia with cold agglutinin disease. (1) tuberculous pneumonitis and pleurisy, (2) hepatosplenomegaly and superficial lymphadenopathy, (3) paraproteinemia of IgM K type, (4) high cold agglutinin (CA) titer (IgM K type, anti-I specificity), hemolytic anemia, and IBL-like appearance of biopsied lymph node. He was treated with chemotherapy for tuberculosis, prednisolone and warming, without anti-neoplastic agents. He died of cachexia 10 months after admission.
Autopsy disclosed; (1) IBL-like lymphadenopathy with monoclonality of IgM K type, (2) infiltration of monoclonal lymphoid-plasmacytoid cell in the liver, spleen, Kidneys, bone marrow, etc. (3) active tuberculous lesion in the lung, (4) amyloid deposit in the lung, pancreas and lymph nodes.
We showed that the M-protein had CA activity, and discussed the possibility that the M-protein with CA activity was produced by neoplastic B-lymphocytes.
