Eltrombopag is a second-line treatment in primary immune thrombocytopenia (ITP). However, its role in secondary ITP is unknown. We evaluated the efficacy and safety of eltrombopag in secondary ITP in daily clinical practice. Eighty-seven secondary ITP patients (46 with ITP secondary to autoimmune syndromes, 23 with ITP secondary to a neoplastic disease subtype: lymphoproliferative disorders [LPDs] and 18 with ITP secondary to viral infections) who had been treated with eltrombopag were retrospectively evaluated. Forty-four patients (38%) had a platelet response, including 40 (35%) with complete responses. Median time to platelet response was 15 days (95% confidence interval, 7-28 days), and was longer in the LPD-ITP group. Platelet response rate was significantly lower in the LPD-ITP than in other groups. However, having achieved response, there were no significant differences between the durable response of the groups. Forty-three patients (49·4%) experienced adverse events (mainly grade 1-2), the commonest being hepatobiliary laboratory abnormalities. There were 10 deaths in this case series, all of which were related to pre-existing medical conditions. In routine clinical practice, eltrombopag is effective and well-tolerated in unselected patients with ITP secondary to both immune and infectious disorders. However, the response rate in LPD-ITP is low.
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http://dx.doi.org/10.1111/bjh.14788 | DOI Listing |
Cureus
November 2024
Hematology, Cheikh Khalifa International University Hospital, Mohammed VI University of Sciences and Health, Casablanca, MAR.
Evans syndrome (ES) is a rare syndrome characterised by the association of autoimmune idiopathic hemolytic anemia (AIHA) with immune thrombocytopenia (ITP) and, less commonly, autoimmune neutropenia (AIN). ES may be primary or secondary to some aetiology, including, exceptionally, tuberculosis. We describe a case of association between pulmonary and medullary tuberculosis and Evans syndrome with an effective response to antitubercular treatment and corticosteroids.
View Article and Find Full Text PDFThromb Haemost
December 2024
McMaster University, Hamilton, Canada.
The McMaster Immune Thrombocytopenia (ITP) Summit was an educational seminar from leading experts in immune thrombocytopenia and related disorders geared towards hematologists, internists, immunologists, and clinical and translational scientists. The focus of the Summit was to review the mechanisms, diagnosis and treatment of primary versus secondary ITP. Specific objectives were to describe the unique features of secondary ITP, and to review its mechanisms in the context of autoimmune disease and infection.
View Article and Find Full Text PDFRheumatol Int
December 2024
Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
Cold agglutinin disease, a subtype of cold-type autoimmune hemolytic anemia, is referred to as cold agglutinin syndrome (CAS) when it develops secondary to other conditions. Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by the peripheral destruction of platelets. While both CAS and ITP can occur in patients with rheumatoid arthritis (RA), their concurrent manifestation in a single patient has not been reported, leaving the optimal treatment strategy for such a complex case unclear.
View Article and Find Full Text PDFJ Blood Med
November 2024
Kidney Institute of PLA, Department of Nephrology, Shanghai Changzheng Hospital, Naval Medical University, Shanghai, People's Republic of China.
Background: Severe acute respiratory syndrome coronavirus (SARS-CoV-2) infection has been a global threat since the end of 2019. Although the main clinical manifestation of coronavirus disease 2019 (COVID-19) is respiratory, its range of clinical manifestation is extensive and may include various systems, including hematological disorders, such as lymphopenia, thrombotic events, thrombocytopenia and immune thrombocytopenic purpura (ITP). The present case was the first one that aimed to raise awareness of ITP induced by COVID-19 in patients undergoing maintenance hemodialysis.
View Article and Find Full Text PDFJ Med Cases
December 2024
Department of Hematology/Oncology, Scripps Clinic/Scripps Green Hospital, La Jolla, CA 92037, USA.
Autoimmune neutropenia (AIN) refers to the immune-mediated destruction of neutrophils. It is a rare condition with an estimated prevalence of less than 1 case per 100,000 per year. Typical treatment involves supportive care with granulocyte colony-stimulating factor (G-CSF) and management of secondary infections with antibiotics.
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