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1.
J Neurol Sci ; 413: 116777, 2020 06 15.
Article in English | MEDLINE | ID: mdl-32200107

ABSTRACT

In chronic polyneuropathies associated with hematologic malignancy (HM) the optimal treatment management is primarily focused on the HM, but the parallel response of the neuropathy is still unclear. Rituximab is a recognized therapeutic choice in anti-MAG antibody polyneuropathy, that might be useful also in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with HM. The efficacy of immunochemotherapy, which is the standard approach to malignant lymphoproliferative diseases, has been poorly investigated in polyneuropathies. We describe a six-months combined bendamustine-rituximab (BR) treatment in nine patients affected by CIDP or paraproteinemic IgM neuropathies with antibodies to peripheral nerve antigens in course of malignant HM. All patients had a long-lasting response with an average relapse free-survival (RFS) time of 31.5 months. Clinical improvement was evident at 6 months from the beginning of therapy, even earlier in 6/9 patients (<2 months). Two patients dramatically improved the disabling attitudinal and intentional tremor and pathogenic autoantibodies significantly declined in 4/5 patients. Neurological relapses occurred in three patients after a mean of 38 months of sustained stability, even if HM remitted. In such cases rituximab was administered but was associated with a shorter RFS time (1 year) compared to the previous BR scheme (3 years). In our case series, the combined BR regimen was a valid option in immune-mediated neuropathies associated with HM. Moreover, in some patients BR scheme allowed an earlier response and a long-lasting improvement than rituximab alone.


Subject(s)
Hematologic Neoplasms , Polyneuropathies , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating , Bendamustine Hydrochloride , Humans , Myelin-Associated Glycoprotein , Neoplasm Recurrence, Local , Polyneuropathies/complications , Polyneuropathies/drug therapy , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/complications , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/drug therapy , Rituximab/therapeutic use
2.
J Neuroimmunol ; 337: 577081, 2019 12 15.
Article in English | MEDLINE | ID: mdl-31677562

ABSTRACT

Rituximab is efficacious in myelin-associated glycoprotein (MAG) polyneuropathy, but the question on timing of retreatments is open. We studied 21 anti-MAG polyneuropathy patients who responded to a first cycle of rituximab, were followed-up for an average of 11.2 years, and were retreated only when relapsing. Baseline serum B-cell-activating factor (BAFF) levels were measured. Clinical improvements lasted on average 6 years, and as many as 71% of the patients resulted long-lasting responders. Severity of disease and high serum BAFF levels (cut-off ≥860 pg/mL for relapse risk) at onset seemed to predict worse prognosis. Measurements of these variables could help deal with the issue of maintenance rituximab therapy in MAG polyneuropathy.


Subject(s)
Autoantibodies/blood , Immunologic Factors/administration & dosage , Myelin-Associated Glycoprotein/blood , Polyneuropathies/blood , Polyneuropathies/drug therapy , Rituximab/administration & dosage , Adult , Aged , Aged, 80 and over , Autoantibodies/immunology , Female , Follow-Up Studies , Humans , Male , Middle Aged , Myelin-Associated Glycoprotein/immunology , Polyneuropathies/immunology , Time Factors , Treatment Outcome
3.
Article in English | MEDLINE | ID: mdl-11970342

ABSTRACT

Experimental neon spectra (in the 10-nm region), from the tokamak Tore Supra and the reversed field pinch experiment RFX, have been simulated. The spectra include lines from three neon ionization states, namely Ne(7+), Ne(6+), and Ne(5+) ions. Collisional radiative models have been built for these three Ne ions, considering electron collisional excitation and radiative decay as populating processes of the excited states. These models give photon emission coefficients for the emitted lines at electron density and temperature values corresponding to the experimental situations. Impurity modelling is performed using a one-dimensional impurity transport code, calculating the steady-state radial distribution of the Ne ions. The Ne line brightnesses are evaluated in a post-process subroutine and simulated spectra are obtained. The parts of the spectra corresponding to a single ionization state do not depend on the experimental conditions and show good agreement with the simulated single ionization state spectra. On the other hand, the superposition of the three spectra depends on the experimental conditions, as a consequence of the fact that the ion charge distribution depends not only on the radial profiles of the electron density and temperature, but also of the impurity transport coefficients. Simulations of the Ne spectra (including transport) give confidence in the atomic physics calculations; moreover, they allow the determination of the transport coefficients in the plasma region emitting the considered ionization states.

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