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Autoimmune Thrombocytopenia

Mohammad Zeya Ansari, R Tolstoy, G Jagadeeswaran
Thrombocytopenia is a common complication in patients with chronic liver disease and is multi-factorial. It complicates the management and worsens the prognosis. Treatment options are costly and include platelet transfusion, splenectomy, splenic artery embolization, TIPPS and thrombopoietin (TPO) agonists. Here we are presenting a patient with decompensated liver disease with known chronic alcoholism and profound thrombocytopenia despite multiple platelet transfusions. On further work up we found a coexistent autoimmune etiology...
March 2018: Journal of the Association of Physicians of India
Julie Pell, Rosemary Greenwood, Jenny Ingram, Katherine Wale, Ian Thomas, Rebecca Kandiyali, Andrew Mumford, Andrew Dick, Catherine Bagot, Nichola Cooper, Quentin Hill, Charlotte Ann Bradbury
INTRODUCTION: Immune thrombocytopenia (ITP) is an autoimmune condition that may cause thrombocytopenia-related bleeding. Current first-line ITP treatment is with high-dose corticosteroids but frequent side effects, heterogeneous responses and high relapse rates are significant problems with only 20% remaining in sustained remission with this approach. Mycophenolate mofetil (MMF) is often used as the next treatment with efficacy in 50%-80% of patients and good tolerability but can take up to 2 months to work...
October 18, 2018: BMJ Open
Andris Skride, Matiss Sablinskis, Kristaps Sablinskis, Sandra Lejniece, Aivars Lejnieks, Walter Klepetko, Irene M Lang
BACKGROUND Antiphospholipid syndrome is an autoimmune disorder characterized by a hypercoagulable state associated with circulating antiphospholipid antibodies. The presence of antiphospholipid antibodies can result in a variety of clinical symptoms, such as thrombocytopenia, stillbirth, endocardial pathologies, and recurrent pulmonary embolism. CASE REPORT We present the case of a 23-year-old man with antiphospholipid syndrome and chronic thromboembolic pulmonary hypertension who developed severe thrombocytopenia...
October 19, 2018: American Journal of Case Reports
Mengxing Chen, Rong Yan, Kangxi Zhou, Xiaodong Li, Yang Zhang, Chunliang Liu, Mengxiao Jiang, Honglei Ye, Xingjun Meng, Ningbo Pang, Lili Zhao, Jun Liu, Weiling Xiao, Renping Hu, Qingya Cui, Wei Zhong, Yunxiao Zhao, Mingqing Zhu, Anning Lin, Changgeng Ruan, Kesheng Dai
Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by low platelet count which can cause fatal hemorrhage. ITP patients with antiplatelet glycoprotein (GP) Ib-IX autoantibodies appear refractory to conventional treatments, and the mechanism remains elusive. Here we show that the platelets undergo apoptosis in ITP patients with anti-GPIbα autoantibodies. Consistent with these findings, the anti-GPIbα monoclonal antibodies AN51 and SZ2 induce platelet apoptosis in vitro. We demonstrate that anti-GPIbα antibody binding activates Akt, which elicits platelet apoptosis through activation of phosphodiesterase (PDE3A) and PDE3A-mediated PKA inhibition...
October 18, 2018: Proceedings of the National Academy of Sciences of the United States of America
Liqiong Yao, Bei Liu, Li Jiang, Lanxia Zhou, Xiaoju Liu
OBJECTIVES: To investigate the association of cytotoxic T lymphocyte-associated antigen 4 (CTLA4) with immune thrombocytopenia (ITP). METHODS: A case-control association analysis of 277 Chinese Han children was performed. The tagging variants rs11571315 and rs3087243 in the CTLA4 gene were detected using polymerase chain reaction-restriction fragment length polymorphism method. The expression quantitative trait loci (eQTL) analysis and quantitative real-time polymerase chain reaction were performed to determine the relationship of CTLA4 with ITP...
October 14, 2018: Hematology (Amsterdam, Netherlands)
Tristan Knight, Yaddanapudi Ravindranath, Michael U Callaghan
Evans syndrome is defined by bilineal autoimmune cytopenia, typically coombs positive hemolytic anemia and thrombocytopenia. Corticosteroids are the mainstay of treatment, with rituximab and/or mycophenolate mofetil often used in steroid-refractory cases. However, no treatment methodology has ever evaluated by a randomized clinical trial. We present a 15-year-old boy with Evans syndrome and common variable immunodeficiency who experienced a severe, refractory flare 16 months postsplenectomy. After failing to respond to multiple other agents, he achieved a durable response to a bortezomib-based regimen...
October 5, 2018: Journal of Pediatric Hematology/oncology
Anastasia A Ignatova, Evgeniya A Ponomarenko, Dmitry M Polokhov, Elena V Suntsova, Pavel A Zharkov, Daria V Fedorova, Ekaterina N Balashova, Anastasia E Rudneva, Vadim V Ptushkin, Evgeniy A Nikitin, Anna Shcherbina, Alexei A Maschan, Galina A Novichkova, Mikhail A Panteleev
The ability of platelets to carry out their hemostatic function can be impaired in a wide range of inherited and acquired conditions: trauma, surgery, inflammation, pre-term birth, sepsis, hematological malignancies, solid tumors, chemotherapy, autoimmune disorders, and many others. Evaluation of this impairment is vitally important for research and clinical purposes. This problem is particularly pronounced in pediatric patients, where these conditions occur frequently, while blood volume and the choice of blood collection methods could be limited...
October 4, 2018: Platelets
Anne Kuitunen, Marjatta Sinisalo, Annukka Vahtera, Leena Hiltunen, Kaija Javela, Outi Laine
BACKGROUND: Heparin-induced thrombocytopenia (HIT) usually appears at 5 to 10 days after initiation of heparin. Autoimmune HIT can arise after discontinuation of heparin treatment (delayed-onset HIT) or without any preceding heparin exposure (spontaneous HIT syndrome). CASE REPORT: This case presents a course of autoimmune HIT with delayed onset. The patient was hospitalized due to influenza pneumonia and received low-molecular-weight heparin thromboprophylaxis for 9 days...
October 4, 2018: Transfusion
Faizan Babar, Scott D Cohen
Thrombotic microangiopathies are heterogeneous disorders characterized by microangiopathic hemolytic anemia with thrombocytopenia and renal injury. There are a variety of causes, including metabolic disorders, infections, medications, complement disorders, pregnancy, malignancy, and autoimmune disorders. This review focuses on renal thrombotic microangiopathy in the setting of rheumatologic diseases. Systemic lupus erythematosus is the most common autoimmune disease associated with thrombotic microangiopathy...
November 2018: Rheumatic Diseases Clinics of North America
Mingjian Bai, Jing Feng, Guowei Liang
Antiphospholipid syndrome (APS) is an autoimmune disorder associated with arterial/venous thrombosis and pregnancy loss; thrombocytopenia is another common manifestation of APS. In the present study, we discovered a transient ethylenediaminetetraacetic acid-dependent pseudothrombocytopenia (EDTA-PTCP) phenomenon in APS, which has not yet been reported in the literature.
September 1, 2018: Clinical Laboratory
Paola Giordano, Flavia Urbano, Giuseppe Lassandro, Francesco Paolo Bianchi, Alessandra Tolva, Paola Saracco, Giovanna Russo, Lucia Dora Notarangelo, Maria Gabelli, Simone Cesaro, Malgorzata Wasniewska, Maria Felicia Faienza
BACKGROUND: Immune thrombocytopenia (ITP) is an acquired immune-mediated disorder characterized by isolated thrombocytopenia. Pediatric ITP patients are prone to develop autoantibodies such as antithyroglobulin (TG) and antithyroperoxidase (TPO), even in the absence of clinical signs of autoimmune disease. The aim of this multicenter retrospective study was to evaluate (1) the prevalence of positivity of antithyroid antibodies (TPO and TG) in a large cohort of pediatric patients with chronic ITP; (2) the role of autoimmune thyroiditis as a prognostic factor for chronicity of ITP...
September 30, 2018: Pediatric Blood & Cancer
Immacolata Brigida, Matteo Zoccolillo, Maria Pia Cicalese, Laurène Pfajfer, Federica Barzaghi, Serena Scala, Carmen Oleaga-Quintas, Jesus A Álvarez-Álvarez, Lucia Sereni, Stefania Giannelli, Claudia Sartirana, Francesca Dionisio, Luca Pavesi, Marta Benavides-Nieto, Luca Basso-Ricci, Paola Capasso, Benedetta Mazzi, Jeremie Rosain, Nufar Marcus, Yu Nee Lee, Raz Somech, Massimo Degano, Giuseppe Raiola, Roberta Caorsi, Paolo Picco, Marcela Moncada Velez, Joelle Khourieh, Andrés Augusto Arias, Aziz Bousfiha, Thomas Issekutz, Andrew Issekutz, Bertrand Boisson, Kerry Dobbs, Anna Villa, Angelo Lombardo, Benedicte Neven, Despina Moshous, Jean-Laurent Casanova, José Luis Franco, Luigi D Notarangelo, Cristina Scielzo, Stefano Volpi, Loïc Dupré, Jacinta Bustamante, Marco Gattorno, Alessandro Aiuti
ARPC1B is a key factor for the assembly and maintenance of the ARP2/3 complex that is involved in actin branching from an existing filament. Germline bi-allelic mutations in ARPC1B have been recently described in six patients with clinical features of combined immunodeficiency, whose neutrophils and platelets but not T lymphocytes were studied. We hypothesized that ARPC1B-deficiency may also lead to cytoskeleton and functional defects in T cells. We have identified bi-allelic mutations in ARPC1B in six unrelated patients with early-onset disease characterized by severe infections, autoimmune manifestations and thrombocytopenia...
September 25, 2018: Blood
Felix Eisinger, Harald F Langer
Platelets are known to be central regulators of haemostasis, inflammation and immune response. Formed by megakaryocytes in the bone marrow and the lungs, platelets express a broad range of adhesion receptors and release cytokines and platelet microparticles which enable them to interact with both immune cells and pathogens. In bacterial and viral infections, thrombophilia and thrombocytopenia are commonly seen symptoms, indicating the close relationship between haemostasis and immune defence. Indeed, platelets contribute both directly and via immune mediation to pathogen clearance...
September 25, 2018: Hämostaseologie
John MacIsaac, Reda Siddiqi, Erin Jamula, Na Li, Steven Baker, Kathryn E Webert, Denise Evanovitch, Nancy M Heddle, Donald M Arnold
BACKGROUND: The anti-CD20 monoclonal antibody rituximab has immune-modulatory effects similar to intravenous immunoglobulin (IVIG). We performed a systematic review and meta-analysis to determine the efficacy and safety of rituximab in autoimmune diseases that are also treated with IVIG. STUDY DESIGN AND METHODS: The most common indications for immune modulation with IVIG, as identified from a 2012 regional audit in Canada, were chronic inflammatory demyelinating polyneuropathy (CIDP), immune thrombocytopenia (ITP), myasthenia gravis, multifocal motor neuropathy, Guillain-Barré syndrome, systemic lupus erythematosus (SLE), Sjogren's syndrome, and pemphigus vulgaris...
September 23, 2018: Transfusion
Opeyemi Folorunsho Bamidele, Richard Oluyinka Akintayo, Mathew Olumuyiwa Bojuwoye, Tolulope Oyetunde Alabi, Foluke Comfort Akintayo, Oluwakemi Victoria Bamidele
Thrombotic thrombocytopenic purpura (TTP) is an uncommon, life-threatening disease characterized by severe thrombocytopenia, microangiopathic haemolytic anaemia, neurologic abnormalities, renal insufficiency, and fever commonly associated with infections, malignancy, drugs, and autoimmune diseases. Coexistence of TTP with systemic lupus erythematosus (SLE) is extremely rare, and the emergence of the full manifestation of SLE starting with TTP is even rarer. Black people due to genetic risk factors are at increased risk of TTP...
2018: Reumatologia
Li Wang, Hua Chen, Jing Shi, Hao Tang, Hang Li, Wenjie Zheng, Fengchun Zhang
RATIONALE: Castleman disease (CD) is a nonclonal lymphoproliferative disorder sometimes manifested systemic inflammatory symptoms. Systemic lupus erythematosus (SLE) is an autoimmune disease characterized with multi-system involvement as well as broad spectrum of serum autoantibodies. When these two conditions happened to have similar clinical spectrum features, the confusion with each other occurred. PATIENT CONCERNS: A 46-year-old man suffered from chronic fever, nephrotic syndrome, acute kidney injury, anemia, thrombocytopenia and serositis, as well as hypocomplementemia and negative anti-nuclear antibody...
September 2018: Medicine (Baltimore)
Fang Wei
INTRODUCTION: Acquired hemophilia A (AHA) is a rare bleeding disease caused by autoantibodies against factor VIII (FVIII). Spontaneous bleeding symptoms usually affect the skin, musco, muscle, and internal organs, while joint hemarthrosis in AHA is an extremely rare manifestation. AHA may have an autoimmune cause and is often associated with autoimmune disease, but no demonstrable platelet impairment was found in AHA patients. We report a patient with AHA complicated with a right shoulder joint hemarthrosis and immune thrombocytopenia...
September 2018: Medicine (Baltimore)
Tanja Falter, Stephanie Herold, Veronika Weyer-Elberich, Carina Scheiner, Veronique Schmitt, Charis von Auer, Xavier Messmer, Philipp Wild, Karl J Lackner, Bernhard Lämmle, Inge Scharrer
BACKGROUND:  Autoimmune thrombotic thrombocytopenic purpura (iTTP) is caused by autoantibody-mediated severe a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13 (ADAMTS13) deficiency leading to micro-angiopathic haemolytic anaemia (MAHA) and thrombocytopenia with organ damage. Patients survive with plasma exchange (PEX), fresh frozen plasma replacement and corticosteroid treatment. Anti-CD20 monoclonal antibody rituximab is increasingly used in patients resistant to conventional PEX or relapsing after an acute bout...
October 2018: Thrombosis and Haemostasis
Ke Zhang, Zhenfeng Dai, Runzhe Liu, Fang Tian, Xi Liu, Yi Sun, Xin Zhao, Xiaoping Pu
Immune thrombocytopenia (ITP) is a common acquired autoimmune disease, and thrombopoietin (TPO) is an important cytokine that regulates the production of megakaryocytes and platelets. We have identified a biologically active component, icaritin, from a Chinese herba epimedii extract. Icaritin promotes platelet production and regulates T cell polarization, but its mechanism is not clear. In this study, the BALB/c mouse model of ITP was established by injection of an antiplatelet antibody every other day for seven total times...
2018: Mediators of Inflammation
Jan-Paul Bohn, Michael Steurer
Immune thrombocytopenia (ITP) is an acquired autoimmune phenomenon resulting in low platelet count and increased bleeding risk. Goals of upfront management include prompt control of severe bleeding-which is rare-as well as induction and maintenance of a hemostatic platelet count. Thus, optimal management of ITP patients is often challenging and requires a highly individualized approach. Many patients may not suffer significant bleeding despite severe thrombocytopenia and the risk of toxicity associated with treatment may outweigh its benefit...
2018: Memo
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