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[Hematological manifestations]
S Kumakura1, H Ishikura, S Kobayashi
1Third Divison of Internal Medicine, Shimane Medical University.
Hematological manifestations as the clinicopathological features of connective tissue disease are described. Secondary anemia, so called anemia of chronic disorders (ACD), is one of the most common features of connective tissue disease. In addition, quantitative abnormalities of leukocytes or thrombocytes which reflect upon the pathophysiology of underlying connective tissue disease are variously observed. Patients with connective tissue disease sometimes develop hematological malignancies. Increased risk of lymphoma in Sjögren's syndrome has been known. The use of alkylating agents as immunosuppressive therapy is also associated with the risk of secondary or therapy-related myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). Pancytopenia or bicytopenia can be caused by hemophagocytosis which is related to connective tissue disease itself. Such condition is recognized as autoimmune-associated hemophagocytic syndrome (AAHS). Possible mechanism inducing hemophagocytosis, clinical features, diagnosis and treatment of AAHS are discussed.
Hematological manifestations as the clinicopathological features of connective tissue disease are described. Secondary anemia, so called anemia of chronic disorders (ACD), is one of the most common features of connective tissue disease. In addition, quantitative abnormalities of leukocytes or thrombocytes which reflect upon the pathophysiology of underlying connective tissue disease are variously observed. Patients with connective tissue disease sometimes develop hematological malignancies. Increased risk of lymphoma in Sjögren's syndrome has been known. The use of alkylating agents as immunosuppressive therapy is also associated with the risk of secondary or therapy-related myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). Pancytopenia or bicytopenia can be caused by hemophagocytosis which is related to connective tissue disease itself. Such condition is recognized as autoimmune-associated hemophagocytic syndrome (AAHS). Possible mechanism inducing hemophagocytosis, clinical features, diagnosis and treatment of AAHS are discussed.