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IgG plasma cell leukemia concomitant with IgA hypergammaglobulinemia and cutaneous lymphoproliferative disorder

Background: Primary plasma cell leukemia (PCL) is a rare and aggressive variant of plasma cell myeloma characterized by high levels of circulating plasma cells. Clinical presentation includes extramedullary infiltration of various tissues and organs as a frequent complication. the disease has a fulminant course and poor prognosis as it was the case in patient presented herein. Patient and methods: Peripheral blood, bone marrow and lymph node FNA and biopsy were examined by conventional morphology and imunocytochemical and imunohistochemical analysis at the time of hospitalisation, as well as CT bone scan, while visceral organs including skin specimen were examined post mortem. Results: A 66-year-old women was admitted to the hospital because of thrombocytopenia, hemopthysis, lymphadenopathy and skin rush. laboratory findings reveald normocytic anemia (Hb 84g/L) and decreased platelet count (26x109/L). Blood smear showed 26% of atypical plasma cells. Immunofixtion- electrophoresis detected a monoclonal band defined as IgG-lambda light chains, with a broad band polyclonal IgA. A bone marrow aspirate and biopsy showed an excess of atypical plasma cells (57%) with restriction of lambda light chain. There was no evidence of osteolytic lesions. Patient died due to splenic rupture, before the diagnostic work-up was finished. Post-mortem examination revealed that lymph nodes, spleen, liver and kidney were infiltrated with atypical plasma cells while skin biopsy revealed scattered large CD30+lymphocites in a background of neutrophils and eosinophils. Based on all these findings, PCL (IgG-lambda type) and lymphomatoid papulosis were diagnosed. Conclusions: Present case is intriguing and challenging since PCL is a rare form of myeloma. In addition in this case PCL was associated with CD30+ lymphoproliferative skin disorder and polyclonal IgA hypergammaglobulinemia.

Plasma cell leukemia ; Cutaneous lymphoproliferative disorder

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