Immunologic Research

, Volume 65, Issue 1, pp 193–196 | Cite as

An idiopathic thrombocytopenic purpura with polyneuropathy

Therapeutic Aspects in Autoimmunity


Concurrent association of idiopathic thrombocytopenic purpura (ITP) and peripheral neuropathy is a rare condition. There are only few case reports published concerning peripheral neuropathy with ITP. One of the etiopathogenetic mechanisms proposed is intraneural hemorrhage, but the pathogenesis is not fully understood. Autoimmune nature with common antibodies to the platelets and the nerve´s myelin sheath should also be considered. Here we describe a 47-year-old woman, with a family history of autoimmune diseases. She was diagnosed 2 years ago with ITP and later developed a chronic combined inflammatory demyelinating polyneuropathy. Treatment with high-dose steroids was initiated with a poor response. The patient showed a clear improvement in platelet count and in the peripheral neuropathy symptoms when treatment with intravenous immunoglobulin was administered. Common etiologic mechanism of the two diseases should be considered, both, because of the simultaneous time of development and a similar response to intravenous immunoglobulin treatment.


Idiopathic thrombotic purpura Peripheral neuropathy Intravenous immunoglobulin Autoimmunity Autoantibodies 


  1. 1.
    Greenberg Michael K, Sonoda T. Mononeuropathy multiplex complicating idiopathic thrombocytopenic purpura. Neurology. 1991;41:1517–818.CrossRefGoogle Scholar
  2. 2.
    Silverstein A. Neuropathy in hemophilia. JAMA. 1964;190:554–5.CrossRefPubMedGoogle Scholar
  3. 3.
    Bigelow NH, Graves RN. Peripheral nerve lesions in hemorrhagic diseases. Arch Neurol Psychiat. 1952;68:819–30.CrossRefGoogle Scholar
  4. 4.
    Brun A, Caviness V, Rudnick P, Tayler HR. Hemorrhages into peripheral nerves in association with leukemia. J Neuropathol Exp Neurol. 1964;23:719–25.CrossRefPubMedGoogle Scholar
  5. 5.
    Cordinegley FT, Crawford GPM. Ulnar nerve palsy in the haemophiliac due to intraneural haemorrhage. Br Med J. 1984;289:18–9.CrossRefGoogle Scholar
  6. 6.
    Miyao S, Takano A, Teramoto J, Fujitake S, Hashizume Y. Oculomotor nerve palsy due to intraneural hemorrhage in idiopathic thrombocytopenic purpura: a case report. Eur Neurol. 1993;33:20–2.CrossRefPubMedGoogle Scholar
  7. 7.
    Gianpolo Antony J, Giuliani Michael J. ITP and neuropathy. To the editor. Neurology. 1993;43:847.CrossRefGoogle Scholar
  8. 8.
    Ward IM, Fewell AE, Ferraro DM, Morris MJ. Concurrent acute motor and sensory axonal neuropathy and immune thrombocytopenic purpura. Mil Med. 2013;178(3):e367.CrossRefPubMedGoogle Scholar
  9. 9.
    Shoenfeld Y, Gilburd B, Abu-Shakra M, Amital H, Barzilai O, Berkun Y, Blank M, Zandman-Goddard G, Katz U, Krause I, Langevitz P, Levy Y, Orbach H, Pordeus V, Ram M, Sherer Y, Toubi E, Tomer Y. The mosaic of autoimmunity: genetic factors involved in autoimmune diseases. Isr Med Assoc J. 2008;10:3–7.PubMedGoogle Scholar
  10. 10.
    Shoenfeld Y, Zandman-Goddard G, Stojanovich L, Cutolo M, Amital H, Levy Y, Abu-Shakra M, Barzilai O, Berkun Y, Blank M, Freire de Carvalho J, Doria A, Gilburd B, Katz U, Krause I, Langevitz P, Orbach H, Pordeus V, Ram M, Toubi E, Sherer Y. The mosaic of autoimmunity: hormonal and environmental factors involved in autoimmune diseases. Isr Med Assoc J. 2008;10:8–12.PubMedGoogle Scholar
  11. 11.
    Shoenfeld Y, Blank M, Abu-Shakra M, Amital H, Barzilai O, Berkun Y, Bizzaro N, Gilburd B, Zandman-Goddard G, Katz U, Krause I, Langevitz P, Mackay IR, Orbach H, Ram M, Sherer Y, Toubi E, Eric Gershwin M. The mosaic of autoimmunity: prediction, autoantibodies, and therapy in autoimmune diseases. Isr Med Assoc J. 2008;10:13–9.PubMedGoogle Scholar
  12. 12.
    Hughes RA, Cornblath DR. Guillain-Barre´ syndrome. Lancet. 2005;366:1653–66.CrossRefPubMedGoogle Scholar
  13. 13.
    Sato N, Kamata T, Akiyama N, Kuwana M, Kanda T. Acute inflammatory sensorimotor polyradiculoneuropathy associated with immune thrombocytopenic purpura. J Intern Med. 2005;257:473–7.CrossRefPubMedGoogle Scholar
  14. 14.
    Harati Y, Bosch EP. Disorders of peripheral nerves. In: Bradley WG, Daroff RB, Fenichel GM, Jankovic J, editors. Bradley’s neurology in clinical practice, vol. 2, 5th ed. Philadelphia: Butterworth-Heinemann Elsevier; 2008. p. 2288–2296.Google Scholar
  15. 15.
    Neunert C, Lim W, Crowther M, Cohen A, Solberg L Jr, Crowther MA. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood. 2011;117:4190–207.CrossRefPubMedGoogle Scholar
  16. 16.
    Van den Bergh PY, Hadden RD, Bouche P, et al. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society—first revision. Eur J Neurol. 2010;17:356.CrossRefPubMedGoogle Scholar
  17. 17.
    Patwa HS, Chaudhry V, Katzberg H, et al. Evidence-based guideline: intravenous immunoglobulin in the treatment of neuromuscular disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2012;78:1009.CrossRefPubMedGoogle Scholar
  18. 18.
    Godeau B, Chevret S, Varet B, et al. Intravenous immunoglobulin or high-dose methylprednisolone, with or without oral prednisone, for adults with untreated severe autoimmune thrombocytopenic purpura: a randomised, multicentre trial. Lancet. 2002;359:23.CrossRefPubMedGoogle Scholar
  19. 19.
    Hughes R, Bensa S, Willison H, et al. Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 2001;50:195.CrossRefPubMedGoogle Scholar
  20. 20.
    Dyck PJ, Litchy WJ, Kratz KM, et al. A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 1994;36:838.CrossRefPubMedGoogle Scholar
  21. 21.
    Eftimov F, Winer JB, Vermeulen M, et al. Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev. 2013;12:CD001797.Google Scholar
  22. 22.
    Godeau B, Caulier MT, Decuypere L, et al. Intravenous immunoglobulin for adults with autoimmune thrombocytopenic purpura: results of a randomized trial comparing 0.5 and 1 g/kg b.w. Br J Haematol. 1999;107:716.CrossRefPubMedGoogle Scholar
  23. 23.
    Schwartz-Albiez R, Monteiro RC, Rodriguez M, Binder CJ, Shoenfeld Y. Natural antibodies, intravenous immunoglobulin and their role in autoimmunity, cancer and inflammation. Clin Exp Immunol. 2009;158(Suppl 1):43–50.CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Shoenfeld Y, Fishman P. Gamma-globulin inhibits tumour spread in mice. Int Immunol. 1999;11:1247–51.CrossRefPubMedGoogle Scholar
  25. 25.
    Shapiro S, Shoenfeld Y, Gilburd B, et al. Intravenous gamma globulin inhibits the production of matrix metalloproteinase-9 in macrophages. Cancer. 2002;95:2032–7.CrossRefPubMedGoogle Scholar
  26. 26.
    Rodeghiero F, Stasi R, Gernsheimer T, et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood. 2009;113:2386.CrossRefPubMedGoogle Scholar
  27. 27.
    Ghanima W, Godeau B, Cines DB, Bussel JB. How I treat immune thrombocytopenia: the choice between splenectomy or a medical therapy as a second-line treatment. Blood. 2012;120:960.CrossRefPubMedGoogle Scholar
  28. 28.
    George JN, Woolf SH, Raskob GE, et al. Idiopathic thrombocytopenic purpura: a practice guideline developed by explicit methods for the American Society of Hematology. Blood. 1996;88:3.PubMedGoogle Scholar
  29. 29.
    Toltl LJ, Arnold DM. Pathophysiology and management of chronic immune thrombocytopenia: focusing on what matters. Br J Haematol. 2011;152:52.CrossRefPubMedGoogle Scholar
  30. 30.
    Mendell JR, Barohn RJ, Freimer ML, et al. Randomized controlled trial of IVIg in untreated chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 2001;56:445.CrossRefPubMedGoogle Scholar
  31. 31.
    Cornblath DR, Gorson KC, Hughes RA, Merkies IS. Observations on chronic inflammatory demyelinating polyneuropathy: a plea for a rigorous approach to diagnosis and treatment. J Neurol Sci. 2013;330:2.CrossRefPubMedGoogle Scholar
  32. 32.
    Gorson KC, van Schaik IN, Merkies IS, et al. Chronic inflammatory demyelinating polyneuropathy disease activity status: recommendations for clinical research standards and use in clinical practice. J Peripher Nerv Syst. 2010;15:326.CrossRefPubMedGoogle Scholar
  33. 33.
    Eftimov F, Vermeulen M, van Doorn PA, et al. Long-term remission of CIDP after pulsed dexamethasone or short-term prednisolone treatment. Neurology. 2012;78:1079.CrossRefPubMedGoogle Scholar
  34. 34.
    Pollard JD, Armati PJ. CIDP—the relevance of recent advances in Schwann cell/axonal neurobiology. J Peripher Nerv Syst. 2011;16:15–23.CrossRefPubMedGoogle Scholar
  35. 35.
    Dacci P, Riva N, Scarlato M, et al. Subcutaneous immunoglobulin therapy for the treatment of multifocal motor neuropathy: a case report. Neural Sci. 2010;31:829–31.CrossRefGoogle Scholar
  36. 36.
    Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society. European Federation of management of multifocal motor neuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society—first revision. J Peripher Nerv Syst. 2010;15:295–301.CrossRefGoogle Scholar
  37. 37.
    Léger JM. Immunoglobulin (IG) in multifocal motor neuropathy (MMN): update on evidence for Ig treatment in MMN. Clin Exp Immunol. 2014;178:42–4.CrossRefPubMedPubMedCentralGoogle Scholar
  38. 38.
    Léger JM, Viala K, Cancalon F, et al. Intravenous Immunoglobulin as short- and long-term therapy for multifocal motor neuropathy: a retrospective study of response to IVIg and of its predictive criteria in 40 patients. J Neural Neurosurg Psychiatry. 2008;79:93–6.CrossRefGoogle Scholar
  39. 39.
    Van den Berg-Vos RM, Franssen H, Wokke JH, Van den Berg LH. Multifocal motor neuropathy: long-term clinical and electrophysiological assessment of intravenous immunoglobulin maintenance treatment. Brain. 2002;125:1875–86.CrossRefPubMedGoogle Scholar
  40. 40.
    Terenghi F, Cappellari A, Bersano A, Carpo M, Barbieri S, Nobile-Orazio E. How long is IVIg effective in multifocal motor neuropathy? Neurology. 2004;62:666–8.CrossRefPubMedGoogle Scholar
  41. 41.
    Vucic S, Black KR, Chong PS, Cros D. Multifocal neuropathy: decrease in conduction blocks and reinnervation with long-term IVIg. Neurology. 2004;63:1264–9.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2016

Authors and Affiliations

  • Valeria Katchan
    • 1
    • 3
  • Paula David
    • 2
    • 3
  • Yehuda Shoenfeld
    • 3
    • 4
  1. 1.Departamento de Medicina InternaHospital La PazMadridSpain
  2. 2.Faculdade de Ciências MédicasUniversidade do Estado do Rio de JaneiroRio de JaneiroBrazil
  3. 3.Zabludowicz Center for Autoimmune DiseasesChaim Sheba Medical CenterTel-HashomerIsrael
  4. 4.Sackler Faculty of MedicineTel-Aviv UniversityTel-AvivIsrael

Personalised recommendations