LAUSR

A 71-year-old man was admitted to our hospital with bilateral cervical lymphadenopathy. Whole-body computed tomography scans showed cervical, mediastinal and para-aortic lymphadenopathy. A full blood count showed: red blood cell count 1 37 9 10/l, haemoglobin concentration 46 g/l, MCV 88 3 fl, reticulocytes 6 8%, white blood cell count 3 86 9 10/l (50% neutrophils, 22% lymphocytes) and platelet count 56 9 10/l. Serum biochemical analysis showed aspartate aminotransferase 19 u/l, alanine aminotransferase 8 u/l, lactate dehydrogenase 550 u/l, total bilirubin 68 4 lmol/l, indirect bilirubin 61 6 lmol/l and haptoglobin <0 1 g/l. A direct Coombs test was positive with the presence of a warm immunoglobulin G (IgG) antibody. The serum platelet-associated IgG level was 581 ng/10 cells (normal, <46 ng/10 cells). The patient was negative for other autoantibodies. Bone marrow examination showed increased erythropoiesis and megakaryocytes, without lymphoid infiltration or haemophagocytosis. Based on these findings, the patient was diagnosed with Evans syndrome (ES). Histological examination of cervical lymph node specimens showed angioimmunoblastic T-cell lymphoma (AITL) (left), and the tumour cells were positive for interleukin-17 (IL17) (right). The patient was treated with THP-COP (tetrahydropyranyl doxorubicin, cyclophosphamide, vincristine and prednisolone), and showed a marked reduction in lymphadenopathy, and recovery of anaemia and thrombocytopenia following the first course. Finally, after six courses of THP-COP, total regression of lymphadenopathy was achieved. Angioimmunoblastic T-cell lymphoma is a neoplasm of mature T follicular helper (Tfh) cells. The proinflammatory cytokine IL17, produced by T helper 17 cells and Tfh cells, has been reported to be linked with the pathogenesis of both autoimmune haemolytic anaemia and autoimmune thrombocytopenia. This patient with AITL and ES, in whom lymphoma cells showed positive staining for IL17, suggests that IL17 produced by lymphoma cells may contribute to the development of ES.