A T-sejtes nagy granuláris lymphocytás leukémia egy ritka, rett T-sejtes neoplázia, mely változatos klinikai lefolyást mutat és gyakran társul autoimmun szövődményekkel.
Idős férfi betegünknél lymphocytosis és neutropenia hátterében igazolt T-LGLL mellett több autoimmun betegség is kialakult, mint gluténérzékenység, primer biliáris cirrhosis, tiszta vörösvértest-aplázia. A súlyos, rendszeres vörösvértest-transzfúziót igénylő tiszta vörösvértest-aplázia a standard immunszuppreszív gyógyszerekre refrakternek bizonyult, végül alemtuzumab hatására remisszióba került.
Bemutatjuk a T-LGLL-ben előforduló autoimmun szövődmények pathomechanizmusát, illetve röviden tárgyaljuk a refrakter esetek kezelési lehetőségeit.
T-cell large granular lymphocytic leukemia is a rare, heterogeneous, mature T-cell neoplasia, often complicated with autoimmune disorders.
The old man was diagnosed with T-cell large granular lymphocytic leukemia due to lymphocytosis and neutropenia. The patient suffered from a couple of autoimmune diseases such as gluten sensitivity, primary biliary cirrhosis, and pure red cell aplasia. The severe pure red cell aplasia proved to be resistant to the standard immunosuppressive drugs, but alemtuzumab therapy induced complete remission.
We explain the pathogenesis of the immune dysregulation and recapitulate the treatment options for a refractory disease.
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