Advertisement

A 29-year-old woman with persistent thrombocytopenia

  • Irene Di PasqualeEmail author
  • Antonella Bertomoro
  • Fabrizio Vianello
  • Piero Marson
  • Francesca Boscaro
  • Fabrizio Fabris
IM - CASE RECORD

Case presentation

Dr. Di Pasquale, Dr. Boscaro: In November 2013, a 29-year-old Moldovan woman was referred to our center with a platelet count of 4 × 109/L and bleeding symptoms such as epistaxis and petechiae. She had been diagnosed with immune thrombocytopenia (ITP) in 2002. Ever since her childhood, her thrombocytopenia had been associated with hemolytic anemia (Evans syndrome) requiring blood transfusions, and complicated by HCV-related hepatitis (anti-HCV antibodies, HCV-RNA 1257491 IU/ml, genotype 1b). Previous therapy for ITP, based on prednisone, intravenous immunoglobulin (IVIg) and azathioprine had been ineffective. In 2006, she underwent splenectomy, and achieved a stable complete remission (CR), maintained with a low daily dose of prednisone (2.5 mg).

She also suffered from Sjögren’s syndrome, with severe dry eyes, positive ANA and ENA anti-SSA titers, and monoclonal gammopathy of undetermined significance IgGK without Bence-Jones proteinuria.

Definitive diagnosis

Dr....

Notes

Compliance with ethical standards

Conflicts of interest

The authors have no conflicts of interest to disclose.

Statement of human and animal rights

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.

References

  1. 1.
    Cines DB, Bussel JB, Liebman HA, Luning Prak ET (2009) The ITP syndrome: pathogenic and clinical diversity. Blood 113(26):6511–6521CrossRefGoogle Scholar
  2. 2.
    Rodeghiero F, Stasi R, Gernsheimer T et al (2009) Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood 113(11):2386–2393CrossRefGoogle Scholar
  3. 3.
    McMillan R, Durette C (2004) Long-term outcomes in adults with chronic ITP after splenectomy failure. Blood 104(4):956–960CrossRefGoogle Scholar
  4. 4.
    Broudy VC, Lin NL (2004) AMG531 stimulates megakaryopoiesis in vitro by binding to Mpl. Cytokine 25:52–60CrossRefGoogle Scholar
  5. 5.
    Kuter DJ, Bussel JB, Lyons RM et al (2008) Efficacy of romiplostim in patients with chronic immune thrombocytopenic purpura: a double-blind randomised controlled trial. Lancet 371:395–403CrossRefGoogle Scholar
  6. 6.
    Bussel JB, Cheng G, Saleh MN et al (2007) Eltrombopag for the treatment of chronic idiopathic thrombocytopenic purpura. N Engl J Med 357:2237–2247CrossRefGoogle Scholar
  7. 7.
    Cooper N (2017) State of the art—how I manage immune thrombocytopenia. Br J Haematol 177:39–54CrossRefGoogle Scholar
  8. 8.
    Boruchov DM, Gururangan S, Driscoll MC, Bussel JB (2007) Multiagent induction and maintenance therapy for patients with refractory immune thrombocytopenic purpura (ITP). Blood 110:3526–3531CrossRefGoogle Scholar
  9. 9.
    Mahévas M, Gerfaud-Valentin M, Moulis G, Terriou L, Audia S, Guenin S, Le Guenno G, Salles G, Lambotte O, Limal N, Viallard JF, Cheze S, Tomowiak C, Royer B, Neel A, Debouverie O, Hot A, Durieu I, Perlat A, Cliquennois M, Deteix C, Michel M, Godeau B (2016) Characteristics, outcome, and response to therapy of multirefractory chronic immune thrombocytopenia. Blood 128(12):1625–1630CrossRefGoogle Scholar
  10. 10.
    Tauchi T, Suzuki A, Fujimura T, Iwabuchi H, Ohyashiki JH, Ohyashiki K et al (1989) Refractory idiopathic thrombocytopenic purpura which responded to protein A column. Rinsho Ketsueki 30(8):1289–1293Google Scholar
  11. 11.
    Fabris F, Luzzatto G, Ramon R, Randi ML, De Silvestro G, Girolami A (2000) Treatment of refractory ITP with extracorporeal immunoadsorption over a protein-A sepharose column: a report of two cases. Haematologica 85(8):888–890Google Scholar
  12. 12.
    Kurtoğlu E, Karakuş V, Deveci B (2013) Refractory idiopathic thrombocytopenic purpura treated with immunoadsorption using tryptophan column. Transfus Apheres Sci 48:311–312CrossRefGoogle Scholar
  13. 13.
    Kotb R, Pinganaud C, Trichet C et al (2005) Efficacy of mycophenolate mofetil in adult refractory autoimmune cytopenias: a single-center preliminary study. Eur J Haematol 75(1):60–64CrossRefGoogle Scholar
  14. 14.
    Taylor A, Neave L, Solanki S, Westwood JP, Terrinonive I, McGuckin S, Kothari J, Cooper N, Stasi R, Scully M (2015) Mycophenolate mofetil therapy for severe immune thrombocytopenia. Br J Haematol 171:625–630CrossRefGoogle Scholar

Copyright information

© Società Italiana di Medicina Interna (SIMI) 2019

Authors and Affiliations

  1. 1.Department of MedicineUniversity of PaduaPaduaItaly
  2. 2.Department of Medicine, Hematology and Clinical Immunology UnitUniversity of PaduaPaduaItaly
  3. 3.Apheresis UnitUniversity of PaduaPaduaItaly

Personalised recommendations