A CASE OF PRIMARY MACROGLOBULINEMIA COMPLICATED BY MULTIPLE CRANIAL NERVE PARALYSIS WITH TUMOR CELLS IN THE CEREBROSPINAL FLUID
1986 Volume 9 Issue 6 Pages 534-541
Details
1986 Volume 9 Issue 6 Pages 534-541
A 64-year old man was admitted to Toyama Medical and Pharmaceutical University Hospital in July 1983 because of systemic lymphadenopathy. On physical examination, enlarged inguinal and axillary lymph nodes were palpable; they were matted together, elastic hard, and not tender. Laboratory findings were as follows: The erythrocyte sedimentation rate was 39mm/hr; The total serum protein was 7.6 g/dl with hyper γ-globulinemia (43%). Immunological studies showed elevated immunoglobulin M (IgM) level of 3, 650 mg/dl. Immunoelectrophoresis demonstrated M protein identified as IgM-κ.Bone marrow aspiration revealed an increase in abnormal lymphoid cells. Histologic and immunohistochemical studies of the biopsied inguinal lymph node revealed follicular lymphoma characterized by the tumor cells containing Ig M and κ-light chain in the cytoplasma. A diagnosis of primary macroglobulinemia was established with clinical features and lymph node biopsy. He had a good response with the chemotherapy of prednisolone and melphalan. Prior to discharge in October 1983, his serum Ig M level had decrease to 1, 150 mg/dl. However, he developed left peripheral facial paralysis in November 1983, and paralysis of right vagus nerve and right glossopharyngeal nerve in June 1984. He was readmitted for further examination in July 1984. The cerebrospinal fluid (CSF) indicated increased cellularity (993/3), total protein 116mg/dl, and glucose 119mg/dl. The cells in CSF looked lymphocytoid, and resembled tumor cells infiltrating into other organs as state before. In addition, a computed tomographic scan of his head showed multiple high density areas enhanced by contrast medium. The tumor cells invasion into central nervous system was diagnosed. While in the hospital, round July 1984, he developed neck stiffness, meningeal sign and systemic convulsion. The treatment with prednisolone was unsuccessful, and he was semiconscious, apathic, incoherent. On September 17th 1984, he expired apparently due to CNS involvement.
To our knowledge, tumor cells invasion into the cerebrospinal fluid in primary macroglobulinemia seems to be exceedingly rare.
