1982 Volume 34 Issue 1 Pages 175-192
An autopsy case of primary macroglobulinemia with gastro-duodenal multiple ulcers is reported. The patient was a 68-year-old man who complained of edema of bilateral legs when he was exposed to cold since 65 years old, and after hospitalization he developed multiple skin ulcers of bilateral legs, persistent diarrhea and pathological fracture of the fifth lumbar vertebra.
Serological studies revealed an increase in amount of monoclonal IgM (kappa) up to 5200mg/dl. Cryoglobulin was detected in the serum and CIq binding activity amounted to 39%. Urinary Bence-Jones protein was positive. Bone marrow aspiration revealed the proliferation of plasmablastlike cells. The patient died due to pneumonia and gastrointestinal bleeding 3.5 years after the onset of this disease.
Autopsy reveale d gastro-duodenal multiple ulcers. Hepatosplenomegaly and lymph node swelling were not found. The middle lobe of the right lung contained a walnut-sized abscess.
Microscopically plasmocytoid lymphocytes were found to infiltrate into almos t all organs, especially into the bone marrow. Some of these infiltrating cells contained PAS-positive, intranuclear and intracytoplasmic inclusion bodies, and PAS-positive eosinophilic homogeneous material was found to occlude the lumen of some of the submucosal capillaries and lymphatics in almost all organs, especially in the digestive tract.
Gastro-duodenal multiple ulcers seem to have been caused as a result of the occlusion by the same material of submucosal capillaries and lymphatics.
An immunofluorescent study, peroxidase-anti-peroxidase procedure and histochemical analysis revealed that this material is composed of monoclonal IgM (kappa) with an admixture of C3complement and phospholipid, Persistent diarrh ea of which this patient complained may be due to the occlusion by eosinophilic material of submucosal capillaries and lymphatics of intestine.
Of various factors involved in the formation of this materi al, the deposition of IgM may be a most important one, and in addition, the precipitation of cryoglobulin, C3 complement and of phospholipid can be enumerated.
It was exceedingly rare in cases of primary macroglobulinemia that IgM-positive materials were deposited, as noted in the present study, in the capillaries and lymphatics throughout the whole body.
