Résumé :
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An obese (1m65, 105 kg) woman presented since the age of 53 years, in 2002, a facial weakness, initially left, then bilateral, associated with an hypoesthesia in the territory of the lower branch of the fifth cranial nerve and with a paralysis of the right phrenic nerve arisen in 2006 ( needing a night-ventilation). EMG showed myogenic signs in the left eyelid orbiculars and in the right sterno-cleidomastoen. Upper and lower limbs EMG and neuro-muscular junction study were normal. Creatine kinases, enzyme of conversion, cerebrospinal fluid, molecular study of FSH, DOP, DM1 and DM2 genes were normal. No antibody anti acetylcholine receptors was found. A relative lymphocytosis was present since the installation of the first symptoms. Treatment by corticoids was ineffective and imurel not supported. The clinical evolution was made towards the progressive worsening with a marked asthenia, an axial and 4 limbs weakness noticed in 2007. A muscle biopsy (2007, right deltoid) showed an infiltration with lymphocytes T, CD8 and CD4 +, CD7 and CD56-. Four years after the first symptoms, an hyperlymphocytosis was noticed, diagnosed as a Large Granular Lymphocytes (LGL) expansion with proliferation of lymphocytes NK type (CD2, CD7, CD16, CD57 positive). As the persistance of the haematologic changes, the patient was considered to have NK-cell lymphocytosis. A treatment by ineffective cyclophosphamide over one year was replaced by methotrexate.NK-cell large granular lymphocyte leukaemia is a rare disorder associated with autoimmune diseases and impaired hematopoiesis. When indolent disease, the diagnosis is NK-cell lymphocytosis. When there is multiorgan large granular lymphocyte infiltration and an aggressive course, the patients are considered to have NK-cell large granular lymphocyte leukaemia. The first diagnosis was stated in our patient. As the muscle did not show NK-cell type infiltration, the discussion is that of an autoimmune myopathy secondary to the NK- LGL or a fortuitous association of two diseases.
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