Relias Media - Continuing Medical Education Publishing

The trusted source for

healthcare information and

CONTINUING EDUCATION.

  • Sign In
  • Sign Out
  • MyAHC
    • Home
      • Home
      • Newsletters
      • Blogs
      • Archives
      • CME/CE Map
      • Shop
    • Emergency
      • All Products
      • Publications
      • Study Guides
      • Webinars
      • Group Sales
    • Hospital
      • All Products
      • Publications
      • Study Guides
      • Webinars
      • Group Sales
    • Clinical
      • All Products
      • Publications
      • Study Guides
      • Webinars
      • Group Sales
    • All Access
      • Subscribe Now
      • My Subscription
    • My Account
      • My Subscriptions
      • My Content
      • My Orders
      • My CME/CE
      • My Transcript
    Home » Is Stem Cell Transplantation a Viable Treatment Option for CIDP?

    Is Stem Cell Transplantation a Viable Treatment Option for CIDP?

    August 1, 2014
    No Comments
    Reprints
    Facebook Twitter Linkedin Share Share

    Related Articles

    Stem Cell Transplantation for MS Treatment

    Mesenchymal Stem Cell Transplantation: A Novel Approach to Treating Progressive Neurological Diseases

    Hematopoietic Stem Cell Transplantation for Adult Cerebral X-linked ALD

    Keywords

    Neurology

    ABSTRACT & COMMENTARY

    Is Stem Cell Transplantation a Viable Treatment Option for CIDP?

    By Norman Latov, MD, PhD

    Professor of Neurology and Neuroscience, Weill Cornell Medical College

    Dr. Latov reports that he receives grant/research support from Grifols; is a retained consultant for CSL Behring and Baxter; and owns stock in Therapath LLC.

    SYNOPSIS: In this small, uncontrolled trial, autologous stem cell transplantation appeared to be efficacious for the treatment of patients with refractory CIDP.

    SOURCE: Press R, et al. Autologous haematopoietic stem cell transplantation: A viable treatment option for CIDP. J Neurol Neurosurg Psychiatry 2014;85:618-624.

    The authors describe the results of autologous haematopoietic stem cell transplantation (AHSCT) treatment in 11 consecutive patients with chronic inflammatory demyelinating polyneuropathy (CIDP) refractory to first-line immunomodulatory treatments and one or more second-line treatments. The total median Inflammatory Neuropathy Cause and Treatment Score (INCAT) and Rankin scores improved significantly within 2-6 months after AHSCT. Eight of the 11 patients maintained drug-free remission, but three of the 11 relapsed during the follow-up period, requiring retransplantation with AHSCT in one. Complications occurred following six of the transplantations, but resolved spontaneously or with treatment. The authors conclude that AHSCT can be efficacious in therapy-refractory CIDP, with a manageable complication profile, although randomized controlled trials are needed.

    COMMENTARY

    CIDP is an inflammatory neuropathy that targets the myelin sheaths in the peripheral nerves. First-line therapies include intravenous immunolobulins, corticosteroids, or plasmapheresis, with a reported response rate of 66% to 87%.1,2,3,4 Those who progress despite first-line treatment are usually offered second-line therapies with anti-inflammatory agents, which have not been tested in clinical trials, but can be beneficial in 25% of patients refractory to first-line therapies.5 In the current cohort, treatments included alemtuzumab, azathioprine, cyclosporine, cyclophosphamide, beta-interferon, mycophenylate motefil, natalizumab, or rituximab.

    In those refractory to second-line therapies, hematopoietic stem cell transplantation (HST) has been reported to be beneficial in occasional patients.6 High-dose cyclophosphamide without stem cell rescue has also been reported to be efficacious in some cases.7 In general, autologous stem cells are generally preferred to allogenic stem cells as the latter can cause graft-vs-host disease, including CIDP,8 although relapses are more common following the use of autologous stem cells, possibly due to inclusion of autoreactive lymphocytes.9 In the current study, three of the 11 patients suffered relapses, with one requiring repeat AHSCT.

    The risks associated with AHSCT are mainly due to the severe pancytopenia that follows the bone marrow ablation. In the current study, early complications were seen in six to 12 transplants, and included reactivation of cytomegalovirus or Epstein-Barr virus, bacterial infection with E. coli, staphylococci, Klebsiella, pseudomonas, alpha Streptococci, or Clostridium difficile; hemorrhagic cystitis; pancreatitis; anemia; hypothyroidism; and neutropenia. All, however, resolved spontaneously or with treatment.

    A caveat to keep in mind is that there is no definitive test for CIDP, so that other causes for demyelinating polyneuropathy, such as Charcot-Marie Tooth type I, anti-MAG neuropathy, or POEMS syndrome, should be definitively ruled out before proceeding with more drastic treatments. As an example, in a recent trial of CIDP, 17% of the patients were found to have an alternate diagnosis that was only made after the patients failed to respond to standard therapies.10

    REFERENCES

    1. Cocito D, et al. A nationwide retrospective analysis on the effect of immune therapies in patients with chronic inflammatory demyelinating polyradiculoneuropathy. Eur J Neurol 2010;17:289-294.
    2. Gorson KC, et al. Chronic inflammatory demyelinating polyneuropathy: Clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology 1997;48:321-328.
    3. Rajabally YA, et al. Validity of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy: A multicentre European study. J Neurol Neurosurg Psychiatry 2009;80:1364-1368.
    4. Viala K, et al. A current view of the diagnosis, clinical variants, response to treatment and prognosis of chronic inflammatory demyelinating polyradiculoneuropathy. J Peripher Nerv Syst 2010;15:50-56.
    5. Mahdi-Rogers M, et al. Immunomodulatory treatment other than corticosteroids, immunoglobulin and plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev 2013;6:CD003280.
    6. Kazmi MA, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: A role for haematopoietic stem cell transplantation? Autoimmunity 2008;41:611-615.
    7. Brannagan TH, et al. High-dose cyclophosphamide without stem-cell rescue for refractory CIDP. Neurology 2002;58:1856-1858.
    8. Lorenzoni PJ, et al. Chronic inflammatory demyelinating polyradiculoneuropathy in chronic graft-versus-host disease following allogeneic hematopoietic stem cell transplantation: Case report. Arq Neuropsiquiatr 2007;65:700-704.
    9. Burt RK, et al. The promise of hematopoietic stem cell transplantation for autoimmune diseases. Bone Marrow Transplant 2003;31:521-524.
    10. Eftimov F, et al. Long-term remission of CIDP after pulsed dexamethasone or short-term prednisolone treatment. Neurology 2012;78:1079-1084.

    Post a comment to this article

    Report Abusive Comment

    www.reliasmedia.com

    Neurology Alert

    View PDF
    Neurology Alert 2014-08-01
    August 1, 2014

    Table Of Contents

    Is Stem Cell Transplantation a Viable Treatment Option for CIDP?

    OnabotulinumtoxinA for Treatment of Chronic Migraines

    Diffusion Tensor MRI Advances Diagnostic Accuracy in ALS

    The Neurophysiological Features of Myoclonus-Dystonia

    Is Epilepsy Surgery on the Decline?

    Atrial Fibrillation and Cryptogenic Stroke: Important New Information

    Begin Test
    Buy this Issue/Course

    Shop Now: Search Products

    • Subscription Publications
    • Books & Study Guides
    • Webinars
    • Group & Site
      Licenses
    • State CME/CE
      Requirements

    Webinars And Events

    View All Events
    • Home
      • Home
      • Newsletters
      • Blogs
      • Archives
      • CME/CE Map
      • Shop
    • Emergency
      • All Products
      • Publications
      • Study Guides
      • Webinars
      • Group Sales
    • Hospital
      • All Products
      • Publications
      • Study Guides
      • Webinars
      • Group Sales
    • Clinical
      • All Products
      • Publications
      • Study Guides
      • Webinars
      • Group Sales
    • All Access
      • Subscribe Now
      • My Subscription
    • My Account
      • My Subscriptions
      • My Content
      • My Orders
      • My CME/CE
      • My Transcript
    • Help
    • Search
    • About Us
    • Sign In
    • Register
    Relias Media - Continuing Medical Education Publishing

    The trusted source for

    healthcare information and

    CONTINUING EDUCATION.

    Customer Service

    customerservice@reliasmedia.com

    U.S. and Canada: 1-800-688-2421

    International +1-404-262-5476

    Accounts Receivable

    1-800-370-9210
    ReliasMedia_AR@reliasmedia.com

    Mailing Address

    • 1010 Sync St., Suite 100
      Morrisville, NC 27560-5468
      USA

    © 2021 Relias. All rights reserved.

    Do Not Sell My Personal Information  Privacy Policy  Terms of Use  Contact Us  Reprints  Group Sales

    For DSR inquiries or complaints, please reach out to Wes Vaux, Data Privacy Officer, DPO@relias.com

    Design, CMS, Hosting & Web Development :: ePublishing