Abstract
A 79-year-old female was admitted to Iwaki Kyoritsu General Hospital because of chest and back oppressive sense and vomiting. Nodular and diffuse skin lesions were found on her face, auricle, and toes. Serological examination revealed elevated monoclonal IgM. Histopathological study on the skin biopsy showed the picture of lymphoplasmacytoma. Cytoplasmic immunoglobulin of μ, κ type was detected in the lymphoma cells. Protein A plaque forming cell assay (PFC assay) was performed for skin, peripheral blood and bone marrow mononuclear cells, and markedly elevated production of IgM was found. A diagnosis of primary macroglobulinemia was made and COP (vincristine, cyclophosphamide and prednisolone) therapy was performed resulting in an improvement in the skin lesions.
This is the first case of primary macroglobulinemia with specific skin lesions where elevated production of IgM was shown by PFC assay.
