Abstract
A 62-year-old woman on hemodialysis with more than 30 years was admitted to our hospital with complaints of high fever, general malaise, pleural effusion and infected decubitus of the buttocks. She had a history of pyelonephritis (24 years of age) and inflammation of the hip and knee joints (40 years of age). One year prior to the admission, she developed renal cell carcinoma (RCC) on the right side. The right kidney was surgically extracted, but 1 year later the dissemination of RCC to the lung and bones was found. For the last decade, she had shown a good response to erythropoietin stimulating agent (ESA), maintaining Hb levels around 11-12 g/dL. However, about one year prior to the admission, she developed ESA-resistant anemia, which required frequent red blood cell transfusions. The diagnosis of lymphoproliferative disorder of granulocytes (LPDG) was based upon the diagnostic criteria for LPDG. The characteristic finding is the presence of increased numbers of lympho-granulocytes, which were rarely identified in the peripheral blood smear in our present case because of leucopenia. On bone marrow biopsy, scattered lymphoid aggregates with CD3-positive cells were determined by immune-histochemistry. Flow cytometric analysis of the bone marrow displayed clonally expanded lymphocytes with a CD3+, CD8+, CD16+, CD56- phenotype. The analysis of T-cell receptor gene rearrangement showed beta and/or gamma chain gene rearrangement using PCR techniques. According to these findings, the diagnosis of T-cell type LPDG based on the WHO classification was reconfirmed. Despite treatment with cyclosporine for LPDG, antibiotics for the infection and pleural drainage and snitinib for RCC, the patients developed septicemia and died a few months after the admission. This case suggests that despite its rarity LPDG and concurrent disorders might be found in patients on long-term dialysis, and that an early awareness of this clinical entity is crucial not only for hematologists but for also for nephrologists and general practitioners specialized in renal replacement therapy in order to help patients on dialysis live longer.
