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Hematology. American Society of... Dec 2023Hematologists are often needed to assist with the management of microangiopathic emergencies in pregnancy. A firm understanding of the diagnosis and management of...
Hematologists are often needed to assist with the management of microangiopathic emergencies in pregnancy. A firm understanding of the diagnosis and management of preeclampsia with severe features, hemolysis elevated liver enzyme and low platelet syndrome, and disseminated intravascular coagulation, which are the most common causes of microangiopathic emergencies, is critical. However, being able to consider when other microangiopathic emergencies (acute fatty liver of pregnancy, congenital and acquired thrombotic thrombocytopenic purpura, complement mediated microangiopathy, antiphospholipid syndrome) should be considered is imperative. The hematologist and obstetric team should work together to optimize the care of common as well as rare hematologic emergencies.
Topics: Pregnancy; Female; Humans; HELLP Syndrome; Pre-Eclampsia; Emergencies; Purpura, Thrombotic Thrombocytopenic; Hemolytic-Uremic Syndrome
PubMed: 38066933
DOI: 10.1182/hematology.2023000500 -
Hematology. American Society of... Dec 2023Thrombocytopenia in ill children is common; accurately diagnosing the underlying etiology is challenging and essential for appropriate management. Triggers for... (Review)
Review
Thrombocytopenia in ill children is common; accurately diagnosing the underlying etiology is challenging and essential for appropriate management. Triggers for accelerated consumption of platelets are numerous; common downstream mechanisms of clearance include platelet trapping in microvascular thrombi, phagocytosis, and platelet activation. Thrombocytopenia with microangiopathic hemolytic anemia (MAHA) is frequently due to disseminated intravascular coagulation. Thrombotic microangiopathy (TMA) is a subgroup of MAHA. Specific TMA syndromes include thrombotic thrombocytopenic purpura, complement-mediated TMA (CM-TMA), and Shiga toxin-mediated hemolytic uremic syndrome. Isolated thrombocytopenia is characteristic of immune thrombocytopenia; however, concomitant cytopenias are frequent in critically ill patients, making the diagnosis difficult. Immune thrombocytopenia with large vessel thrombosis is a feature of heparin-induced thrombocytopenia and antiphospholipid antibody syndrome. In addition, thrombocytopenia is common with macrophage activation, which is characteristic of hemophagocytic lymphohistiocytosis. While thrombocytopenia in ill patients can be driven by hypoproliferative processes such as myelosuppression and/or bone marrow failure, this review will focus on consumptive thrombocytopenia due to immune and nonimmune causes.
Topics: Child; Humans; Purpura, Thrombocytopenic, Idiopathic; Purpura, Thrombotic Thrombocytopenic; Hemolytic-Uremic Syndrome; Thrombotic Microangiopathies; Anemia, Hemolytic; Thrombosis
PubMed: 38066886
DOI: 10.1182/hematology.2023000465 -
British Journal of Haematology Oct 2023Immune thrombocytopenia (ITP) is an acquired bleeding disorder mediated by pathogenic autoantibodies secreted by plasma cells (PCs) in many patients. In refractory ITP... (Review)
Review
Immune thrombocytopenia (ITP) is an acquired bleeding disorder mediated by pathogenic autoantibodies secreted by plasma cells (PCs) in many patients. In refractory ITP patients, the persistence of splenic and bone marrow autoreactive long-lived PCs (LLPCs) may explain primary failure of rituximab and splenectomy respectively. The reactivation of autoreactive memory B cells generating new autoreactive PCs contributes to relapses after initial response to rituximab. Emerging strategies targeting B cells and PCs aim to prevent the settlement of splenic LLPCs with the combination of anti-BAFF and rituximab, to deplete autoreactive PCs with anti-CD38 antibodies, and to induce deeper B-cell depletion in tissues with novel anti-CD20 monoclonal antibodies and anti-CD19 therapies. Alternative strategies, focused on controlling autoantibody mediated effects, have also been developed, including SYK and BTK inhibitors, complement inhibitors, FcRn blockers and inhibitors of platelet desialylation.
Topics: Humans; Rituximab; Purpura, Thrombocytopenic, Idiopathic; B-Lymphocytes; Plasma Cells; Autoantibodies
PubMed: 37002711
DOI: 10.1111/bjh.18773 -
British Journal of Haematology Aug 2023
Randomized Controlled Trial
Topics: Humans; Platelet Count; Purpura, Thrombocytopenic, Idiopathic; Benzoates; Hydrazines
PubMed: 37339869
DOI: 10.1111/bjh.18908 -
Hamostaseologie Feb 2024
Topics: Humans; Purpura, Thrombocytopenic, Idiopathic; Thrombocytopenia; Thrombosis
PubMed: 38417798
DOI: 10.1055/s-0044-1782593 -
Tidsskrift For Den Norske Laegeforening... May 2024
Topics: Humans; Purpura; Golf
PubMed: 38738575
DOI: 10.4045/tidsskr.24.0262 -
Drugs Apr 2024Apadamtase alfa (ADAMTS13, recombinant-krhn; ADZYNMA), a human recombinant form of a disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13), is being... (Review)
Review
Apadamtase alfa (ADAMTS13, recombinant-krhn; ADZYNMA), a human recombinant form of a disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13), is being developed by Takeda under license from KM biologics for thrombotic thrombocytopenic purpura (TTP) and sickle cell disease. On 9 November 2023, apadamtase alfa was approved in the USA for prophylactic and on-demand enzyme replacement therapy (ERT) in paediatric and adult patients with congenital TTP. Apadamtase alfa is under regulatory review for congenital TTP in the EU and Japan, and is under clinical development for immune-mediated TTP in several countries worldwide. Clinical development of apadamtase alfa for vaso-occlusive crisis related to sickle cell anaemia is underway in the USA. This article summarizes the milestones in the development of apadamtase alfa leading to this first approval in the USA for congenital TTP.
Topics: Humans; Drug Approval; Purpura, Thrombotic Thrombocytopenic; ADAMTS13 Protein; Enzyme Replacement Therapy; Anemia, Sickle Cell; United States; Recombinant Proteins
PubMed: 38418772
DOI: 10.1007/s40265-024-02007-6 -
Hamostaseologie Apr 2024
Review
Topics: Humans; Purpura, Thrombocytopenic, Idiopathic; Autoantibodies
PubMed: 38688267
DOI: 10.1055/a-2280-1083 -
The American Journal of the Medical... Jul 2024
Topics: Humans; Purpura; Tongue; Tongue Diseases; Male; Female; Middle Aged
PubMed: 38460925
DOI: 10.1016/j.amjms.2024.03.003 -
La Tunisie Medicale Jan 2024Autoimmune cytopenias are defined by autoantibodies' immune destruction of one or more blood elements. Most often it is autoimmune hemolytic anemia or immune...
Autoimmune cytopenias are defined by autoantibodies' immune destruction of one or more blood elements. Most often it is autoimmune hemolytic anemia or immune thrombocytopenia or both that define Evans syndrome. It may be secondary to infection or to underlying pathology such as systemic autoimmune disease or primary immunodeficiency, especially when it becomes chronic over several years. Primary Immunodeficiencies or inborn errors of immunity (IEI) are no longer defined solely by infections: autoimmunity is part of the clinical features of several of these diseases. It is dominated by autoimmune cytopenias, in particular, immune thrombocytopenia (ITP) and autoimmune hemolytic anaemia (AIHA). The challenges for the clinician are the situations where autoimmune cytopenias are chronic, recurrent and/or refractory to the various long-term therapeutic options. Most of these therapies are similar in action and generally consist of non-mediated immune suppression or modulation. In these situations, primary Immunodeficiencies must be diagnosed as soon as possible to allow the initiation of a targeted treatment and to avoid several ineffective therapeutic lines.
Topics: Child; Humans; Purpura, Thrombocytopenic, Idiopathic; Cytopenia; Anemia, Hemolytic, Autoimmune; Thrombocytopenia
PubMed: 38545722
DOI: 10.62438/tunismed.v102i1.4503