Archives

  • 2018-07
  • 2018-10
  • 2018-11
  • 2019-04
  • 2019-05
  • 2019-06
  • 2019-07
  • 2019-08
  • 2019-09
  • 2019-10
  • 2019-11
  • 2019-12
  • 2020-01
  • 2020-02
  • 2020-03
  • 2020-04
  • 2020-05
  • 2020-06
  • 2020-07
  • 2020-08
  • 2020-09
  • 2020-10
  • 2020-11
  • 2020-12
  • 2021-01
  • 2021-02
  • 2021-03
  • 2021-04
  • 2021-05
  • 2021-06
  • 2021-07
  • 2021-08
  • 2021-09
  • 2021-10
  • 2021-11
  • 2021-12
  • 2022-01
  • 2022-02
  • 2022-03
  • 2022-04
  • 2022-05
  • 2022-06
  • 2022-07
  • 2022-08
  • 2022-09
  • 2022-10
  • 2022-11
  • 2022-12
  • 2023-01
  • 2023-02
  • 2023-03
  • 2023-04
  • 2023-05
  • 2023-06
  • 2023-07
  • 2023-08
  • 2023-09
  • 2023-10
  • 2023-11
  • 2023-12
  • 2024-01
  • 2024-02
  • 2024-03
  • br Discussion We were interested in knowing whether patients

    2019-05-06


    Discussion We were interested in knowing whether patients who were discharged before ANC recovery did well because they were younger, had better PS, or more likely to be in CR than patients who were not discharged before ANC recovery. However while patients with better PS (but not age) were more likely to be discharged than other patients, better PS was not associated with fewer re-admissions. Likewise patients in CR were not less likely to be re-admitted. Thus 9 of the 35 patients (26%) who were in CR or shown to be in CR at their next marrow after initial NF were readmitted for a subsequent NF within 30 days of discharge vs 4 of the 14 patients who were not in CR. The contrast between this finding and Bodey et al.\'s likely reflects the development of better AMG 925 and correspondingly less need for reliance on normally functioning neutrophils (as in patients in CR). A point made by Bodey et al. that still seems true however is that the trend in neutrophil count is a better predictor than a cutoff value such as 500. Specifically, only 3 of the 22 patients (14%) whose neutrophil count increased by 500 or more between initial admission for NF and discharge were subsequently re-admitted vs 10 of 27 patients (37%) with less of an increase (p=0.10). If we used 50 rather than 500 as the criterion of a rise, readmission rates were 7/37 (19%) for those with such an increase vs 6/12 (50%) for those without (p=0.06).
    Funding
    Introduction Large granular lymphocytic (LGL) leukemia is an uncommon clonal lymphoproliferative disorder, which has usually an indolent clinical behavior and can be associated, at diagnosis or during evolution, with a variety of autoimmune disorders including autoimmune cytopenias such as pure red cell aplasia, Sjogrens syndrome and rheumatoid arthritis (RA). Lambert–Eaton Myasthenic Syndrome (LEMS) is a rare neuromuscular autoimmune disease caused by pathogenic autoantibodies targeting the voltage-gated calcium channels (VGCC) on the presynaptic nerve terminal. It traduces by muscular weakness and autonomic dysfunction. It is strongly associated with Lung Small Cell Carcinoma (LSLC), and fulfills the paraneoplastic syndrome definition criteria. We here report on a patient with LGL leukemia, associated with a seropositive and symptomatic LEMS and a seronegative rheumatoid arthritis.
    Observation A 77 year-old male without any relevant medical history nor professional toxic exposition, presented in 2001 with a permanent cough (without detectable infectious etiology), proximal muscular weakness, unusual asthenia, and a dry mouth sensation. Clinical examination showed a complete areflexia. Paraclinical explorations showed anti N-type autoantibodies directed against VGCC strongly positives (390pmol/l; Nl<100), without anti-P/Q type VGCC antibodies. Laboratory tests showed a normal blood cells differential count. Neurophysiological tests showed a trend to lower first compound muscle action potential amplitude, with a discrete post-exercise increment, which were compatible with LEMS. An exhaustive neoplastic screening was performed, showing an asymptomatic and non-capsule disrupting prostatic adenocarcinoma. Patient was effectively treated by hormonotherapy, leading to normalization of PSA-value, but without effect on patient\'s neurological symptoms. Anti N-type VGCC-directed autoantibodies-value continued to increase (530pmoll−1 at maximum). Six months later, patient presented clinical features compatible with active rheumatoid arthritis (RA) (tenosynovitis and arthritis). Laboratory tests showed a moderate hyperlymphocytosis (4.6×109/l), predominantly composed of LGL. LGL phenotypic expression was CD3+, CD5low, CD7low, CD8+, CD4low, CD16+, CD57+, CD56− and TCRαβ gene rearrangement showed a monoclonal pattern. Patient was neutropenic (PMN: 0.8×109/l). Serum protein electrophoresis did not show hypo- nor AMG 925 hyper-gammaglobulinemia. C-reactive protein value was below 5mg/l. Autoantibodies screening showed negativity for Rheumatoid Factor, antikeratin antibodies and antinuclear antibodies, and positivity for antiphospholipid antibody. The patient was treated with steroids (initially 15mg/day) and methotrexate (7.5mg/m2/week). Clinical manifestations of RA and of neurological symptoms were quickly and dramatically improved. This first-line therapy was continued indefinitely, with a steroid-dependence at 6mg/day regarding rheumatological manifestations, and without any neurological relapse. The patient achieved a complete and persistent hematological response within 10 months. Partial molecular response was documented with a persistence of 10% of clonal TCRαβ using PCR. The anti-N type autoantibodies against VGCC-value progressively decreased (310pmoll−1 at minimum, 11 months after beginning the LGL leukemia treatment; anti-N antibodies assay was never repeated after this date). Neither Lung Small-Cell Carcinoma (LSCC), nor other preexistent carcinoma was detected during the next 9-years follow-up. Finally, the patient died in 2011, 9 years later, at the age of 86 years, due to a brain tumor without any evidence of RA/LGL leukemia nor LEMS relapse.