An unusual case of primary macroglobulinemia associated with PIE syndrome and erythema

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Recently we have had an unusual case of primary macroglobulinemia associated with PIE syndrome and pruritic erythema. A 77-year-old woman was admitted to this hospital In February 1977, because of productive cough and subfebrile episodes of three years' duration. Her chest x-ray film showed linear and nodular shadow in the left lower lung field and infiltration in the right lower lung field. Elevated erythemas with itching sensation in both upper extremities and over the body were noted.Investigations on admission were as follows; Bence Jones protein in the urine was negative. IgG level was 1, 300mg/dl, IgA 80mg/dl, and IgM 1, 400mg/dl. Electrophoretic picture of the serum showed monoclonal spike of IgM (κ) type. Cryoglobulin was negative. Cold agglutinin titer was within normal limits. Rheumatoid factor was positive, Mantoux reaction and DNCB negative. No osteolytic lesions were noted. Blood picture of peripheral blood as well as bone marrow showed eosinophilia with normal appearance, 34% and 14%, respectively. The bone marrow from the sternum showed lymphoid cells resembling plasma cells. These cells stained with FITC-labeled anti-μ, in which well-developed, somewhat dilated, rough endoplasmic reticulums were documented by electron microscopy. Biopsy specimens from pleura, cervical lymph node and cutaneous lesion revealed eosinophilic infiltrations with plasma cells.We have discussed the combination of primary macroglobulinemia, PIE syndrome and erythema to investigate whether there is a pathogenetic relationship among the three. It should be considered that this combination can be pathogenetically related: (1) The eosinophilia of this case might be due to hypersensitivity reactions occurring in the presence of primary macroglobulinemia which is an immunodeficiency disease. (2) Host reaction against tumor cells might induce eosinophilia.
日本臨床免疫学会の論文

An unusual case of primary macroglobulinemia associated with PIE syndrome and erythema

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