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Master Thesis

Large granular lymphocyte leukemia and pulmonary hypertension : an unusual association

Abstract : Large granular lymphocyte (LGL) leukemia is a rare lymphoproliferative disorder characterized by a clonal expansion of T/NK cells associated with cytopenia and immune diseases. This retrospective international cohort reports 9 cases of patients presenting with pulmonary hypertension (PH) proved by right heart catherization (RHC) and LGL leukemia. Among the 5 patients revalued by RHC, 4 patients showed hemodynamic PH improvement following immunosuppressive treatment of LGL Leukemia. With a median follow-up of 88 months, all patients are alive. Pathogenesis mechanisms uniting both diseases are discussed. These data suggest completing the initial PAH work-up with T cell clonality detection and to propose starting immunosuppressive therapy if LGL leukemia is concomitantly diagnosed.
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Master Thesis
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https://dumas.ccsd.cnrs.fr/dumas-01782595
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Submitted on : Thursday, June 7, 2018 - 12:47:51 PM
Last modification on : Thursday, July 9, 2020 - 12:30:44 PM

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Brieuc Chérel. Large granular lymphocyte leukemia and pulmonary hypertension : an unusual association. Life Sciences [q-bio]. 2017. ⟨dumas-01782595⟩

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