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dc.contributor.authorMatutes, Een_US
dc.contributor.authorWotherspoon, ACen_US
dc.contributor.authorParker, NEen_US
dc.contributor.authorOsuji, Nen_US
dc.contributor.authorIsaacson, PGen_US
dc.contributor.authorCatovsky, Den_US
dc.identifier.citationBRITISH JOURNAL OF HAEMATOLOGY, 2001, 115 pp. 801 - 806en_US
dc.description.abstractWe describe a case of T-cell large granular lymphocyte (LGL) leukaemia that transformed into a large-cell T-cell lymphoma 11 years from diagnosis. A 29-year-old asymptomatic female presented in 1989 with lymphocytosis, neutropenia and mild bone marrow infiltration. The circulating cells were LGL with a CD2+, CD3+, CD8+, CD4-, CD16+, CD56+, CD57- 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-Barr virus, cytomegalovirus, human T-lymphotropic virus-I, human herpes virus (HHV)-6 and HHV-7 was negative, Histology of the mass was consistent with the diagnosis of peripheral T-cell 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-cell receptor (TCR) gamma-chain 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-cell LGL leukaemia with no evidence of transformation. This case illustrates that transformation or Richter syndrome may occur in a minority of patients with T-cell 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-existing clone.en_US
dc.format.extent801 - 806en_US
dc.titleTransformation of T-cell large granular lymphocyte leukaemia into a high-grade large T-cell lymphomaen_US
dc.typeJournal Article
rioxxterms.typeJournal Article/Reviewen_US
pubs.notesaffiliation: Matutes, E (Reprint Author), Royal Marsden Hosp, Acad Dept Haematol & Cytogenet, Royal Marsden NHS Trust, Fulham Rd, London SW3 6JJ, England. Royal Marsden Hosp, Acad Dept Haematol & Cytogenet, Royal Marsden NHS Trust, London SW3 6JJ, England. Royal Marsden Hosp, Dept Histopathol, London SW3 6JJ, England. Whittington Hosp, Dept Haematol, London N19 5NF, England. UCL, Sch Med, Dept Histopathol, London W1N 8AA, England. keywords: T cell; large granular lymphocyte leukaemia; transformation; high-grade T-cell lymphoma keywords-plus: POLYMERASE CHAIN-REACTION; BARR-VIRUS INFECTION; MONOCLONALITY; DNA research-areas: Hematology web-of-science-categories: Hematology author-email: [email protected] number-of-cited-references: 20 times-cited: 27 usage-count-last-180-days: 0 usage-count-since-2013: 0 journal-iso: Br. J. Haematol. doc-delivery-number: 509GD unique-id: ISI:000173137200013 oa: gold_or_bronze da: 2018-09-20en_US
pubs.notesNot knownen_US
pubs.organisational-group/ICR/Primary Group
pubs.organisational-group/ICR/Primary Group/ICR Divisions
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Clinical Studies
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Clinical Studies/Molecular Haematology (including Cytogenetics Group and Cell Markers)
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Molecular Pathology
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Molecular Pathology/Molecular Haematology (including Cytogenetics Group and Cell Markers)
pubs.embargo.termsNot knownen_US
icr.researchteamMolecular Haematology (including Cytogenetics Group and Cell Markers)en_US
dc.contributor.icrauthorCatovsky, Danielen_US
dc.contributor.icrauthorMatutes, Estellaen_US
dc.contributor.icrauthorWotherspoon, Andrewen_US
dc.contributor.icrauthorOsuji, NNennaen_US

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