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Cyclosporine immune thrombocytopenic purpura

Lucia Schwarzová, Zdenko Pirník, Natália Štecová
Primary immune thrombocytopenia (idiopathic thrombocytopenic purpura; ITP) is an autoimmune disorder characterized by isolated thrombocytopenia without abnormalities in the erythroid and myeloid/lymphoid lineages. The incidence of ITP in adults is estimated at approximately 1.6-3.9 per 100 000 persons/year. The aim of this study was to analyze a case report of a patient with diagnosed immune thrombocytopenia (D69.3) resistant to cyclosporine A therapy as well as an evaluation of factors that could contribute to an inadequate response to cyclosporine A...
2018: Ceská a Slovenská Farmacie
Spero R Cataland, Peter J Kourlas, Shangbin Yang, Susan Geyer, Leslie Witkoff, Haiwa Wu, Camila Masias, James N George, Haifeng M Wu
Although steroids are routinely used as an adjunct to plasma exchange (PEX) therapy in the treatment of immune-mediated thrombotic thrombocytopenic purpura (iTTP), limited data regarding their efficacy or effect on ADAMTS13 biomarkers are available. We report the results of a prospective, randomized study that compared the effectiveness of prednisone or cyclosporine (CSA) as adjuncts to PEX in the treatment of iTTP. A total of 26 of the planned 72 subjects were enrolled and treated from November 2007 until February 2014 before the study was halted after a planned interim analysis...
October 24, 2017: Blood Advances
Doinita Sfrijan, Simina-Maria Visan, Bianca Diaconu, Sabina Zurac, Cristian Scurtu
Sweet's Syndrome also knows as acute febrile neutrophilic dermatosis, is a rare skin's condition, that can occur either idiopathic or secondary. In the case of the latter, the syndrome can develop after certain malignancies (paraneoplastic syndrome), because of exposure to some medication or post infectious. It is more frequent in women aged between 30 and 50 years, but concerning children, the disorder is extremely rare (8% of the total number of cases), having equal sex ratio distribution. We present the case of an 11 year old male, diagnosed with systemic form of SS associated with Myelodysplastic Syndrome...
June 2016: Mædica
Bérangère S Joly, Paul Coppo, Agnès Veyradier
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving protease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies, but rarely, it is inherited via mutations of the ADAMTS13 gene...
May 25, 2017: Blood
S Sami Kartı, Necmi Eren, Mehmet Sönmez, Mustafa Yılmaz, Ümit Çobanoğlu, Halil Kavgacı, Hasan Bozkaya, Ercüment Ovalı
Thrombotic thrombocytopenic purpura (TTP) typically presents with consumptive thrombocytopenia, non-immune intravascular hemolytic anemia, renal failure, various neurologic findings and fever. It is a clinical syndrome that can be associated with drugs such as ticlopidine, quinine, mitomycin C and cyclosporine, allogeneic stem cell transplantation, pregnancy, infections, autoimmune diseases and metastatic carcinoma. Here, we describe a 48-years- old women presented with full picture of TTP and diagnosed as leiomyosarcoma with further evaluation...
September 5, 2003: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
Myoung Nam Bae, Dae Hun Kwak, Se Jun Park, Bum Soon Choi, Cheol Whee Park, Yeong Jin Choi, Jong Wook Lee, Chul Woo Yang, Yong-Soo Kim, Byung Ha Chung
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a fatal clinical syndrome characterized by excessive immune activation and inflammation. It is frequently complicated by acute kidney injury (AKI) that often develops as acute tubular necrosis (ATN). Meanwhile, renal thrombotic microangiopathy (TMA) is a rare pathologic finding that mostly occurs in hemolytic uremic syndrome or thrombotic thrombocytopenic purpura. There are only few reports on TMA developing in patients with HLH...
January 6, 2016: BMC Nephrology
Yilmaz Ay, Tuba H Karapinar, Yesim Oymak, Ersin Toret, Bengu Demirag, Dilek Ince, Esin Ozcan, Nergial Moueminoglou, Sultan A Koker, Canan Vergin
Immune thrombocytopenic purpura (ITP) results from accelerated platelet destruction mediated by autoantibodies to platelet glycoproteins. Some patients with chronic ITP are refractory to all therapies [steroids, intravenous immunoglobulin (IVIG), anti-D and immunosuppresive drugs] and have chronic low platelet counts and episodic bleeding. We retrospectively evaluated the efficacy and safety of rituximab treatment and splenectomy in paediatric patients diagnosed with chronic and refractory ITP who were unresponsive to steroids, IVIG, cyclosporine and mycophenolate mofetil...
June 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
Philip Young-Ill Choi, Fernando Roncolato, Xavier Badoux, Sundra Ramanathan, Shir-Jing Ho, Beng H Chong
Promising reports of combination immunosuppression with high-dose dexamethasone and rituximab for the treatment of primary immune thrombocytopenia (ITP) have recently emerged. They suggest a potential to further optimize the efficacy of therapy. We investigate the use of a novel combination of conventional therapies in ITP given over 4 weeks. From 2011 to 2014, 20 patients were prospectively enrolled onto a single-arm phase 2b study to describe the safety, efficacy, and tolerability of oral dexamethasone 40 mg for days 1 to 4, oral cyclosporine 2...
July 23, 2015: Blood
Carl M Gay, William N William, Sa A Wang, Thein Hlaing Oo
Although the association of pure red cell aplasia (PRCA) and aplastic anemia with thymoma is well-known, acquired amegakaryocytic thrombocytopenia (AAMT) is not a recognized paraneoplastic manifestation of thymoma. This report discusses a patient with recurrent thymoma complicated by myasthenia gravis, PRCA, and AAMT. Both PRCA and AAMT are diagnosed after a thymoma recurrence, 11 years after complete resection of the initial tumor and 9 months after chemotherapy for the relapsed disease. Both PRCA and AAMT responded to immunosuppression with cyclosporine, corticosteroid, and an abbreviated course of antithymocyte globulin, achieving a very good erythroid response and a complete remission for AAMT, suggesting that AAMT, although extremely rare, can be an immune-mediated paraneoplastic manifestation of thymoma...
November 2014: Journal of the National Comprehensive Cancer Network: JNCCN
Letizia von Laer Tschudin, Valérie M Schwitzgebel, Annette von Scheven-Gête, Jean-Louis Blouin, Michael Hofer, Michael Hauschild, Marc Ansari, Sophie Stoppa-Vaucher, Franziska Phan-Hug
Type 1 diabetes (T1D) is rarely a component of primary immune dysregulation disorders. We report two cases in which T1D was associated with thrombocytopenia. The first patient, a 13-year-old boy, presented with immune thrombocytopenia (ITP), thyroiditis, and, 3 wk later, T1D. Because of severe thrombocytopenia resistant to immunoglobulins, high-dose steroids, and cyclosporine treatment, anti-cluster of differentiation (CD20) therapy was introduced, with consequent normalization of thrombocytes and weaning off of steroids...
March 2015: Pediatric Diabetes
Ashwin P Patel, Amit S Patil
Dapsone is one of the second line treatments of immune thrombocytopenic purpura (ITP). Dapsone is cheap and has response rates comparable to other second line treatment options like azathioprine, danazol, cyclophosphamide, cyclosporine, and vincristine. This retrospective analysis includes 38 patients (out of total 313 patients) of ITP treated with dapsone from 2004 to 2012. All male patients were screened for G6PD deficiency before starting dapsone. Out of 38 patients (12 children and 26 adults), one was newly diagnosed ITP, seven were persistent ITP, and 30 were chronic ITP...
2015: Platelets
Giovanni C Del Vecchio, Attilio De Santis, Lora Accettura, Domenico De Mattia, Paola Giordano
Chronic thrombocytopenias are pathological conditions defined as a persistent platelet count below the normal range for more than 6-12 months, clinically characterized by mucocutaneous bleeding. Recently, an International Working Group of expert clinicians has redefined standard terminology and definitions of primary and secondary chronic immune thrombocytopenia (ITP). A document issued on acute childhood idiopathic thrombocytopenic purpura (AIEOP) provides parents and physicians with guidelines for the management of chronic ITP and gives prominence to the periodic re-evaluation of differential diagnosis...
June 2014: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
Jiaming Li, Zhaoyue Wang, Lan Dai, Lijuan Cao, Jian Su, Mingqing Zhu, Ziqiang Yu, Xia Bai, Changgeng Ruan
We conducted this randomized trial to investigate the efficacy and safety of rapamycin treatment in adults with chronic immune thrombocytopenia (ITP). Eighty-eight patients were separated into the control (cyclosporine A plus prednisone) and experimental (rapamycin plus prednisone) groups. The CD4⁺CD25⁺CD127(low) regulatory T (Treg) cells level, Foxp3 mRNA expression, and the relevant cytokines levels were measured before and after treatment. The overall response (OR) was similar in both groups (experimental group versus control group: 58% versus 62%, P = 0...
2013: Clinical & Developmental Immunology
Chikara Ogimi, Nazuna Honma, Risa Tanaka, Tsutomu Oh-ishi
A 6-year-old girl, who had received corticosteroid and cyclosporine on the diagnosis of interstitial pneumonitis related to juvenile dermatomyositis, developed severe thrombocytopenia. Her thrombocytopenia was resistant to repeated intravenous immunoglobulin administration and methylprednisolone pulse therapy. After additional treatment with mycophenolate mofetil (MMF), instead of cyclosporine, the thrombocytopenia improved, facilitating a reduction in the dose of corticosteroid without exacerbation of the interstitial pneumonitis...
April 2012: Modern Rheumatology
Xing Wu, Wen Qu, Jun Wang, Guo-Jin Wang, Yong Liang, Rong Fu, Yu-Hong Wu, Li-Juan Li, Zong-Hong Shao
This study was aimed to investigate the clinical features of patients with immune thrombocytopenia (ITP). The clinical data, laboratory results and therapeutic effects of 125 inpatients with ITP from January 2005 to May 2010 were retrospectively analyzed. The results showed that median age of 125 inpatients was 41, and the sex ratio of male to female was 1:1.78. The average pre-treatment platelet count was (28.59 ± 23.05) × 10(9)/L. 69.1% patients (67/97) suffered from rheumatoid or immune abnormalities. The carrier rate of hepatitis B virus and parvovirus B19 were 37...
April 2011: Zhongguo Shi Yan Xue Ye Xue za Zhi
Salam Alkindi, Saif Khan, Dawood Riyami, Mahfooz Farooqi, Anil Pathare
We describe here coexistence of immune thrombocytopenic purpura (ITP) and idiopathic membranous glomerularnephritis (IMG) in one individual who presented a decade ago after delivering a macerated dead fetus. Investigations performed then revealed clinical, biochemical and histopathological evidence of IMG. A year later she presented with bleeding diathesis and was diagnosed as ITP. Thereafter, she manifested with several episodes of serious bleeding complications especially in the central nervous system with considerable frequency, inspite of receiving treatment with steroids, intravenous immunoglobulin and cyclosporine...
2010: Platelets
Hannah Tamary, Jelena Roganovic, Meera Chitlur, Diane J Nugent
The rarity of severe complications of this disease in children makes randomized clinical trials in immune thrombocytopenia (ITP) unfeasible. Therefore, the current management recommendations for ITP are largely dependent on clinical expertise and observations. As part of its discussions during the Intercontinental Cooperative ITP Study Group Expert Meeting in Basel, the Management working group recommended that the decision to treat an ITP patient be individualized and based mainly on bleeding symptoms and not on the actual platelet count number and should be supported by bleeding scores using a validated assessment tool...
July 2010: Annals of Hematology
Maisa Nazzal, Fadi Safi, Freda Arma, Mustafa Nazzal, Mahvish Muzaffar, Ragheb Assaly
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening multisystem disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia accompanied by microvascular thrombosis that causes variable degrees of tissue ischemia and infarction leading to organ dysfunction. Drug-associated thrombotic thrombocytopenic purpura and hemolytic uremic syndrome has been recognized for several years. The most commonly implicated drugs are mitomycin C, cyclosporine, quinine, clopidogrel, and ticlopidine...
November 2011: American Journal of Therapeutics
Donald M Arnold, Ishac Nazi, Aurelio Santos, Howard Chan, Nancy M Heddle, Theodore E Warkentin, John G Kelton
Treatment options for patients with chronic refractory immune thrombocytopenic purpura (ITP) are limited. Because combination immunosuppressant therapy appeared to be effective in ITP and other disorders, we used this approach in patients with particularly severe and refractory ITP. In this retrospective, observational study, we determined the response (platelet count above 30 x 10(9)/L and doubling of baseline) among 19 refractory ITP patients. Treatment consisted of azathioprine, mycophenolate mofetil, and cyclosporine...
January 7, 2010: Blood
B Einollahi
Kidney transplantation has rarely been performed in patients with immune thrombocytopenic purpura (ITP). We present two cases of kidney recipients from unrelated donors in women who had prior chronic refractory ITP. Their immunosuppression included induction therapy with antithymocyte globulin (ATG) and maintenance treatment with cyclosporine and prednisone. Kidney transplantations were safely performed without any complication. The platelet count of our patients increased gradually after the surgery, and maintenance with cyclosporine, mycophenolate mofetil, and prednisolone allowed graft function and satisfactory hemostasis...
September 2009: Transplantation Proceedings
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