|
- 2017
Immunopathology of Immune thrombocytopenia: Essentials for oncologists and haematologistsAbstract: An in depth knowledge is essential about the pathology of immune thrombocytopenias before we start managing these autoimmune disorders. It’s important to differentiate whether the patient has primary or secondary immune thrombocytopenia. If the aetiology is known, we try to eliminate the antigen which triggers further formation of autoantibodies directed against platelets. If aetiology is unknown then as per international working group criteria, we diagnose such immune thrombocytopenia as primary immune thrombocytopenias. Primary immune thrombocytopenias are managed primarily by administering intravenous immunoglobulins or steroids as first line treatment. In second line immunosuppressive drugs like azathioprine, cyclosporine, rituximab, vinca alkaloids like vincristine, cyclophosphamide or other drugs like androgens- danazol, TPO mimetics etc. are administered. And of course for refractory or relapsed ITP splenectomy remains as an important surgical option however now-a-days less popular. Keywords: ITP- immune thrombocytopenia; ALPS – Autoimmune lymphoproliferative Syndrome; SLE –Systemic lupus erythematosis; VZV- Varicella zoster virus; APS – Antiphospholipid syndrome; CVID – Common variable immune deficiency; MMR- Measles, mumps, rubella.; MDS –Myelodysplastic syndrome; GP- Glycoprotein; i.v.Ig – Intravenous immunoglobulins; TPO- Thrombopoeitin mimetics; NK-natural killer; MHC- Major histocompability Complex; APCantigen presenting cells; TCR – T cell recerptor ; Tregs –T relulatory Lymphocytes ; IL-interleukin ; TGF-transforming growth factor; IDDMinsulin dependent diabetes mellitus ; CTL- cytotoxic T lymphocyte
|