Abstract
A case of Sjögren's syndrome associated with macroglobulinemia is prsesnted. The patient, 28-year-old female, was admitted to our clinic because of dry eyes and mouth, and salivary gland enlargement in September, 1973. The sicca symptoms, especcially dryness of eyes, purpura in lower extremities and polyarthralgia had developed 5 years prior to the admission. Swelling of eyelids and salivary glands had occurred 3 months before admission and she had undergone splenectomy with the diagnosis of hypersplenism in a local hospital 2 months before admission to our clinic. The diagnosis of Sjögren's syndrome was made based on these clinical symptoms and on the histologic findings of biopsied salivary gland showing dense infiltrate of small lymphocytes, concentrated in ductal areas and around venules, associated with atrophy of acinar tissue.
The presence of monoclonal macroglobulinemia with κ and λ type light chains was demonstrated by immunoelectrophoresis, and the values of IgM ranged from 700 to 850 mg/100 ml. The sedimentation constant of the the macroglobulin was 26S and 18S, and the viscosity of serum was greatly increased. Enlargement of salivary glands and lymph nodes including both pulmonary hilar nodes increased progressively. In addition, hepatic enlargement up to 6 finger breadths below right costal margin and pleural effusion caused dyspnea and general fatigue. The patient responded excellently to chlorambucil with the disappearance of sicca symptoms and lymphadenopathy and decrease of hepatomegaly.
The relationship between Sjögren's syndrome and macroglobulinemia as well as malignant lymphoma was discussed.
