A patient with IgA lambda multiple myeloma (MM) developed plasma cell leukemia (PCL), presenting as oculomotor nerve palsy. The cerebrospinal fluid (CSF) contained plasma cells, which double stained with fluoresceinated anti-IgA and anti-lambda antisera. The palsy was most probably due to meningeal myelomatosis. The neurologic disorder appeared to be refractory to the therapy used, although plasma cells disappeared from the peripheral blood. Secondary plasma cell leukemia is a rare complication of MM, usually occurring in the terminal stage of the disease. Those patients may be eligible for central nervous system (CNS) prophylaxis, as is commonly performed in patients with other types of leukemia.
The IgG subclass composition was determined of the anti-D antibodies present in the serum of 22 women who had a history of severe rhesus-D immunization and who weekly underwent small volume plasmapheresis during their current pregnancy. There was no correlation between the subclass patterns of IgG anti-D antibodies and the degree of illness of the child; the good clinical results obtained with the small volume plasmapheresis could not be explained by a consistent change in the anti-D IgG subclass composition.
A case history of a patient with primary plasma cell leukaemia is presented. Analysis of serum showed an IgDλ paraprotein, and λ-light-chains were found in the urine. Immunofluorescence studies of a bone marrow aspirate revealed intracytoplas-matic IgD of λ-type in plasma cells. Moreover J-chain could be demonstrated in these plasma cells. A complete remission of the disease, with disappearance of the paraprotein, was obtained following treatment with a combination of cyclophosphamide, vin-cristine and prednisone. 12 months later, the patient developed multiple extramedullary plasmocytoma lesions in the skin. After treatment with cyclophosphamide, vincristine, doxorubicin and prednisone, another remission was achieved. The literature on the clinical features and the response to chemotherapy of primary plasma cell leukemia is reviewed.
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