Idiopathic thrombocytopenic purpura (ITP) – new era for an old disease

Abstract

Immune thrombocytopenia is an autoimmune hematological disorder characterized by severely decreased platelet count of peripheral cause: platelet destruction via antiplatelet antibodies which may also affect marrow megakaryocytes. Patients may present in critical situations, with cutaneous and/or mucous bleeding and possibly life-threatening organ hemorrhages (cerebral, digestive, etc.) Therefore, rapid diagnosis and therapeutic intervention are mandatory. Corticotherapy represents the first treatment option, but as in any autoimmune disorder, there is a high risk of relapse. Second line therapy options include: intravenous immunoglobulins, thrombopoietin receptor agonists, rituximab or immunosuppression, but their benefit is usually temporary. Moreover, the disease generally affects young people who need repeated and prolonged treatment and hospitalization and therefore, it is preferred to choose a long term effect therapy. Splenectomy – removal of the site of platelet destruction – represents an effective and stable treatment, with 70–80% response rate and low complications incidence. A challenging situation is the association of ITP with pregnancy, which further increases the risk due to the immunodeficiency of pregnancy, major dangers of bleeding, vital risks for mother and fetus, potential risks of medication, necessity of prompt intervention in the setting of specific obstetrical situations – delivery, pregnancy loss, obstetrical complications, etc. We present an updated review of the current clinical and laboratory data, as well as a detailed analysis of the available therapeutic options with their benefits and risks, and also particular associations (pregnancy, relapsed and refractory disease, emergency treatment).

Romiplostim and Eltrombopag in Immune Thrombocytopenia as a Second-Line Treatment

“…The normal platelet count in the general population ranges between 150 and 400×10 9 /L while in ITP patients treated with TRAs, the main treatment goal is to decrease the risk of bleeding and maintain platelet count between 50×10 9 /L and 150×10 9 /L [ 18 ]. However, in patients with a severe disease without bleeding, a platelet count of ⩾20×10 9 /L is tolerable [ 18 ]. Several studies have been done to compare the outcomes in patients treated with romiplostim to patients treated with standard treatment or placebo.…”

“…Romiplostim is a recombinant, Fc-peptide fusion protein (peptibody) given subcutaneously, while eltrombopag is an orally available drug that binds to the transmembrane region of c-MpL [ 17 ]. Recognizing that ITP pathophysiology is defined as increased destruction of platelets and decreased production, the treatment should also not be limited to focusing only on diminishing platelet destruction, but also increasing survival [ 18 ]. Therefore, the use of romiplostim and eltrombopag in ITP treatment is supported by promoting megakaryocyte survival and increasing platelet production [ 18 ].…”

“…Recognizing that ITP pathophysiology is defined as increased destruction of platelets and decreased production, the treatment should also not be limited to focusing only on diminishing platelet destruction, but also increasing survival [ 18 ]. Therefore, the use of romiplostim and eltrombopag in ITP treatment is supported by promoting megakaryocyte survival and increasing platelet production [ 18 ]. In this review article, we will review and compare the effectiveness and side effects of romiplostim and eltrombopag in ITP treatment and the validation of switching between these two drugs, based on previously published studies.…”

Cerebellar hemorrhage due to immune thrombocytopenia

“…Tiene como causa subyacente una gran variedad de enfermedades . 9 Para hablar de PTI, debemos tener un recuento plaquetario de <100 ×10 /L en sangre periférica, (3) sin afectación de las demás series hematológicas . La principal fisiopatología es el aumento de la destrucción periférica de las plaquetas y la mayoría de los pacientes presentan anticuerpos anti glicoproteína de la membrana de las plaquetas.…”

Idiopathic thrombocytopenic purpura in a patient with situs inversus totalis: case report and literature review

“…(5) The clinical picture may present as critical situations, with cutaneous and mucosal bleeding and even voluminous hemorrhage, which make the quick diagnosis and therapeutic intervention mandatory. (6) Its incidence is estimated at 1.6 to 2.7 cases per 100 thousand individuals/year, and prevalence at 9.5 to 23.6 cases per 100 thousand individuals, with predominance of the female sex. (7) This article aimed to report the case of a patient with coexistence of two rare conditions: situs inversus totalis and idiopathic thrombocytopenic purpura.…”