{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,15]],"date-time":"2026-06-15T07:31:31Z","timestamp":1781508691005,"version":"3.54.1"},"reference-count":20,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2001,12,20]],"date-time":"2001-12-20T00:00:00Z","timestamp":1008806400000},"content-version":"vor","delay-in-days":19,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2001,12]]},"abstract":"<jats:p>We describe a case of T\u2010cell large granular lymphocyte (LGL) leukaemia that transformed into a large\u2010cell T\u2010cell lymphoma 11\u2003years from diagnosis. A 29\u2010year\u2010old asymptomatic female presented in 1989 with lymphocytosis, neutropenia and mild bone marrow infiltration. The circulating cells were LGL with a CD2+, CD3+, CD8+, CD4\u2013, CD16+, CD56+, CD57\u2013 phenotype. In August 2000, she developed fever, a large submandibular mass and hepatosplenomegaly. Biochemistry showed abnormal liver function tests and raised lactate dehydrogenase (LDH) levels. A serological screen for Epstein\u2013Barr virus, cytomegalovirus, human T\u2010lymphotropic virus\u2010I, human herpes virus (HHV)\u20106 and HHV\u20107 was negative. Histology of the mass was consistent with the diagnosis of peripheral T\u2010cell lymphoma composed of large cells, and immunohistochemistry showed that the lymphoma cells had a phenotype identical to the mature LGL. Molecular analysis with the polymerase chain reaction (PCR) demonstrated rearrangement of the T\u2010cell receptor (TCR) \u03b3\u2010chain gene with a band of identical size in both bone marrow mature LGL and lymph node cells. The patient was treated with CHOP (cyclophosphamide, vincristine, doxorubicin and prednisolone), resulting in the disappearance of the mass and improvement of the hepatosplenomegaly, LDH and liver abnormalities. She underwent splenectomy, and spleen histology showed involvement by T\u2010cell LGL leukaemia with no evidence of transformation. This case illustrates that transformation or Richter syndrome may occur in a minority of patients with T\u2010cell LGL leukaemia, a disease that has a benign clinical course in most cases. This is the first case documented by molecular methods of the transformation of the pre\u2010existing clone.<\/jats:p>","DOI":"10.1046\/j.1365-2141.2001.03220.x","type":"journal-article","created":{"date-parts":[[2003,3,11]],"date-time":"2003-03-11T06:40:31Z","timestamp":1047364831000},"page":"801-806","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":40,"title":["Transformation of T\u2010cell large granular lymphocyte leukaemia into a high\u2010grade large T\u2010cell lymphoma"],"prefix":"10.1111","volume":"115","author":[{"given":"Estella","family":"Matutes","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew C.","family":"Wotherspoon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Norman 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