A 75 year old woman who had had rheumatoid arthritis for 21 years and Sjögren’s syndrome for 12 years developed autoimmune hemolytic anemia. Antibodies to tissue extracts, erythrocytes, cell nuclei, and altered γ-globulin were detected in the serum, although the serum γ-globulin level was low.

The bone marrow was densely infiltrated by lymphocytes having similar abnormalities to the circulating lymphocytes.

Analysis of the proteins synthesized by the peripheral lymphocytes showed a relatively low output of immunoglobulins.

The hemolytic process was controlled by azathioprine after unsuccessful treatment with prednisolone.

A hypothesis is put forward to explain the not infrequent association of multiple autoimmune disorders, lymphoproliferative disease and disorders of immunoglobulin synthesis such as hypogammaglobulinemia and paraproteinemia. It is suggested that the disturbance of immunological function in such cases occurs at the site of recruitment for immunological activity in the peripheral lymphoid organs, constituting a disturbance of terminal immunological differentiation. The basic abnormality may, therefore, be found in the supporting cells of the lymphoid organs.

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© 1968 by American Society of Hematology, Inc.
1968
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