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Idiopathic thrombocytopenia

Vanesa Calvo-Río, Montserrat Santos-Gómez, Inmaculada Calvo, Ma Isabel González-Fernández, Berta López Montesinos, Marina Mesquida, Alfredo Adán, María Victoria Hernández, Olga Maíz, Antonio Atanes, Beatriz Bravo, Consuelo Modesto, Gisela Díaz-Cordovés, Natalia Palmou-Fontana, Javier Loricera, M C González-Vela, Rosalía Demetrio-Pablo, J L Hernández, Miguel A González-Gay, Ricardo Blanco
OBJECTIVE: To assess the efficacy of Tocilizumab (TCZ) for Juvenile Idiopathic Arthritis (JIA)-associated uveitis. METHODS: Multicenter study of patients with JIA-associated uveitis refractory to conventional immunosuppressive drugs and anti-TNFα agents. RESULTS: We assessed 25 (21 women) patients (47 affected eyes); mean age 18.5±8.3 years. Uveitis was bilateral in 22. Cystoid macular edema (CME) was present in 9 patients. Ocular sequelae found at TCZ onset included: cataracts (13), glaucoma (7), synechiae (10), band keratopathy (12), maculopathy (9), and amblyopia (5)...
October 1, 2016: Arthritis & Rheumatology
J Marcoval, C Martín-Callizo, F Valentí-Medina, M Bonfill-Ortí, L Martínez-Molina
BACKGROUND: Several studies support a strong association of Sweet syndrome (SS) with malignancy. However, only a few studies analysing the clinical features of malignancy-associated SS have been published in recent years. AIM: To retrospectively study the clinical features of SS that could predict the development of associated malignancies and to analyse the development of malignant neoplasia during long-term follow-up of patients with SS. METHODS: Clinical features of the patients diagnosed with SS syndrome between 1987 and 2013 at Bellvitge Hospital (Barcelona, Spain) were retrospectively analysed...
October 2016: Clinical and Experimental Dermatology
S Christopher N Frontario, Anna Goldenberg-Sandau, Darshan Roy, Roy Sandau
Angiosarcoma of the spleen is a rare malignancy that arises from vascular endothelial origin. This neoplasm is highly malignant and diagnosis is often delayed due to the vague presentation of clinical symptoms. A case report and concise review of the current diagnostic criteria and surgical treatment are provided to aid in the detection and treatment of this malignancy. We present a case of a 56-year-old female who presented with massive splenomegaly secondary to angiosarcoma of the spleen. The patient suffered from longstanding symptomatic anemia and thrombocytopenia...
2016: Case Reports in Surgery
Daniel C Moreira, Christopher J Ng, Ralph Quinones, Xiayuan Liang, Dominic W Chung, Jorge Di Paola
Idiopathic systemic capillary leak syndrome (SCLS) is a rare process characterized by acute and recurrent episodes of vascular leakage with severe hypotension, hypoalbuminemia, hemoconcentration and edema. Anemia and thrombocytopenia are not part of this syndrome, but here we present the case of a pediatric patient with a clinical presentation consistent with SCLS who subsequently developed microangiopathic hemolytic anemia at a time when she had significant fluid loss and anasarca. Based on serial ADAMTS-13 levels, we propose that the anemia in this patent developed as a result of ADAMTS-13 loss in the third-space fluid, a novel mechanism for acquired microangiopathic hemolytic anemia...
September 13, 2016: Journal of Thrombosis and Haemostasis: JTH
Masao Hagihara, Morihiro Inoue, Kenichiro Kodama, Tomoyuki Uchida, Jian Hua
An 80-year-old man was admitted to our hospital because of severe thrombocytopenia. He was diagnosed with idiopathic thrombocytopenia, and prednisolone together with eltrombopag was started, leading to significant improvement of platelet counts. Four years later, there was a prominent increase of peripheral blood monocytes, which was accompanied by recurrence of thrombocytopenia. Bone marrow aspirates and serum electrophoresis revealed coexistence of chronic myelomonocytic leukemia (CMML) and multiple myeloma (MM)...
2016: Case Reports in Hematology
Jennifer Mai Falk, Inge Scharrer
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a rare, episodic clinical syndrome involving the production of thrombi in the microvasculature accompanied by thrombocytopenia and symptoms of organ ischemia. Idiopathic TTP develops when a patient produces antibodies that react with the protease ADAMTS13. The course after an episode is unpredictable; patients may relapse frequently or never. There is no laboratory value that can reliably predict potential relapse. STUDY DESIGN AND METHODS: To assess diagnostic and predictive values for risk of relapse, plasma samples from 27 patients with idiopathic TTP in remission were analyzed for anti-ADAMTS13 immunoglobulin (Ig)G, ADAMTS13 activity, and ADAMTS13 inhibitor titer...
August 11, 2016: Transfusion
Takahiro Tsuji, Hiromi Mochinaga, Hiroshi Yamasaki, Hiroyuki Tsuda
An 83-year-old woman was admitted to our hospital with a severe headache and purpura. She had previously been diagnosed with idiopathic thrombocytopenia purpura (ITP) and achieved complete remission with steroid therapy. Steroid therapy had been completed one week prior to the current admission. The recurrence of severe thrombocytopenia (<1.0×10(4) platelets/μl) was detected and a CT scan revealed pituitary hemorrhage without pituitary adenoma. She received steroid therapy combined with intravenous immunoglobulin, which resulted in the amelioration of ITP and improvements in the pituitary hemorrhage...
July 2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Etsuko Matsubara, Jun Yamanouchi, Takaaki Hato, Kazuto Takeuchi, Toshiyuki Niiya, Masaki Yasukawa
An 81-year-old man presenting with fever, neurological symptoms, thrombocytopenia, and hemolytic anemia was diagnosed with acquired idiopathic thrombotic thrombocytopenic purpura (TTP). His disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13 (ADAMTS13) activity was <1% and the ADAMTS13 inhibitor titer was 3.2 BU/ml. He received plasma exchange and steroid administration until remission was achieved. Seven months later, he suffered from paralysis of the right hand, hemolytic anemia, and thrombocytopenia...
July 2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Deepak Jain, Hari K Aggarwal, Avinash Rao, Anshul Mittal, Promil Jain
Systemic onset juvenile idiopathic arthritis (sJIA) is defined as arthritis affecting one or more joint usually in the juvenile age group (< 16 years of age) with or preceded by fever of at least 2 weeks duration that is documented to be daily ("quotidian") for at least 3 days which may be associated with evanescent (non-fixed) erythematous rash or generalized lymph node enlargement or hepatomegaly/splenomegaly/both or serositis. Macrophage activation syndrome (MAS) is a life-threatening complication of sJIA marked by sudden onset of non-remitting high fever, profound depression in all three blood cell lines (i...
2016: Reumatologia
Pierre Bigot, Marine Auffret, Sophie Gautier, Marie Weinborn, Nicolas-Kader Ettahar, Patrick Coupé
Oseltamivir is a neuraminidase inhibitor approved for the prevention and treatment of influenza. Few haematological side effects have been reported with oseltamivir. We report herein the case of an unexpected platelet increase in a 46-year-old woman with idiopathic thrombocytopenia (ITP) treated with oseltamivir for influenza. The mechanism may involve the neuraminidase inhibition which decrease platelet surface sialic acid content and reduce their removal by the reticuloendothelial system. Oseltamivir may be responsible for platelet increase especially in patients with ITP...
October 2016: Fundamental & Clinical Pharmacology
Xiaohong Ruby Xu, Dan Zhang, Brigitta Elaine Oswald, Naadiya Carrim, Xiaozhong Wang, Yan Hou, Qing Zhang, Christopher Lavalle, Thomas McKeown, Alexandra H Marshall, Heyu Ni
Platelets are small anucleate blood cells generated from megakaryocytes in the bone marrow and cleared in the reticuloendothelial system. At the site of vascular injury, platelet adhesion, activation and aggregation constitute the first wave of hemostasis. Blood coagulation, which is initiated by the intrinsic or extrinsic coagulation cascades, is the second wave of hemostasis. Activated platelets can also provide negatively-charged surfaces that harbor coagulation factors and markedly potentiate cell-based thrombin generation...
December 2016: Critical Reviews in Clinical Laboratory Sciences
Abrar-Ahmad Zulfiqar, Jean-Luc Novella, Rachid Mahmoudi, Jean-Loup Pennaforte, Emmanuel Andres
There are no specific recommendations on the care of elderly patients with ITP. A retrospective study over two academic centers (Reims and Strasbourg), focused on patients over 65 who have been diagnosed idiopathic thrombocytopenic purpura in two unit of internal medicine. 41 patients were enrolled, including 27 women (66%). The median age is 76.75 years. On admission, the average thrombocytopenia is 34.45 G/L; 24 patients (58.5%) showed severe bleeding mucosal and/or visceral signs; 37 patients (90%) had a first-line treatment...
June 1, 2016: Gériatrie et Psychologie Neuropsychiatrie du Vieillissement
Martha Romero, Guido R González-Fontal, Mónica Duarte, Carlos Saavedra, Andrés F Henao-Martínez
CASE DESCRIPTION: An 82-years old Hispanic woman with a past medical history significant for pulmonary thromboembolism on oral anticoagulation, rheumatoid arthritis, and hypertension developed a new onset thrombocytopenia. CLINICAL FINDINGS: Small clonal B-cells populations (SCBP) also known as monoclonal B-cell lymphocytosis was found as part of the workup for an idiopathic thrombocytopenia and lead ultimately to the diagnosis of parotid primary follicular lymphoma coexisting with Warthin tumor involving the bone marrow in a small extent and oncocytic papilloma located in the maxillary sinus...
2016: Colombia Médica: CM
T Patiroglu, C Klein, H Eke Gungor, M A Ozdemir, M Witzel, M Karakukcu, J Sawalle-Belohradsky, R Conca, E Unal
AIM: The Wiskott-Aldrich syndrome (WAS) is an X-linked immunodeficiency characterized by micro-thrombocytopenia, eczema, and recurrent infections. We aimed to share our experience with six children with WAS, including two patients with two novel mutations. MATERIAL AND METHOD: We present phenotypical and laboratory description of six patients with WAS. The initial clinical presentation, biochemical and radiological features, molecular diagnosis together with long-term follow-up data are provided...
2016: Genetic Counseling
Carrie Lester, Johanna Cooper, Rachel M Peters, Cynthia R L Webster
OBJECTIVE: To characterize the clinical presentation and outcome of dogs with acute liver failure (ALF). DESIGN: Retrospective case series from January 1995 to December 2012. SETTING: University teaching hospital. ANIMALS: Forty-nine dogs were diagnosed with ALF defined as the acute onset of clinical signs accompanied by serum hyperbilirubinemia and coagulopathy (prothrombin time >1.5 times the upper limit of the reference interval) with or without signs of hepatic encephalopathy...
July 2016: Journal of Veterinary Emergency and Critical Care
Katie H Faella, David Whiting, Francis Fynn-Thompson, Gregory S Matte
The direct thrombin inhibitor bivalirudin is an option for anticoagulation in patients with heparin induced thrombocytopenia (HIT) requiring cardiopulmonary bypass (CPB). There are a limited number of reports of pediatric patients in which bivalirudin has been used for anticoagulation for CPB. We present the case of an 11 year old male with acute onset heart failure secondary to idiopathic dilated cardiomyopathy that developed heparin induced thrombocytopenia with thrombosis (HITT). The patient was anticoagulated in the operating room with bivalirudin and placed on CPB for insertion of a HeartWare(®) Ventricular Assist Device (Heartware(®))...
March 2016: Journal of Extra-corporeal Technology
Kai Ding, Rong Fu, Hui Liu, Deepak Anil Nachnani, Zong-Hong Shao
Thrombocytopenia is a common, often fatal complication experienced by patients with myelodysplastic syndromes (MDS). 5-aza-2'-deoxycytidine (decitabine) has been used to treat MDS patients with thrombocytopenia with a response rate of 45-50%. However, the mechanism of its effects on megakaryocytes remains unclear. In the present study, the effect of decitabine on megakaryocyte maturation was investigated. A total of 20 MDS patients diagnosed with thrombocytopenia were enrolled, including 16 refractory anemia with excess blasts (RAEB)-1 patients and 4 RAEB-2 patients], in addition to 20 leukemia patients that had achieved complete remission and 20 healthy donors...
April 2016: Oncology Letters
Joji Nagasaki, Masahiro Manabe, Kentaro Ido, Hiroyoshi Ichihara, Yasutaka Aoyama, Tadanobu Ohta, Yoshio Furukawa, Atsuko Mugitani
The etiologies of secondary idiopathic thrombocytopenic purpura (ITP) include infection, autoimmune disease, and immunodeficiency. We report the cases of three elderly patients who developed ITP after receiving influenza vaccinations. The platelet count of an 81-year-old woman fell to 27,000/μL after she received an influenza vaccination. A 75-year-old woman developed thrombocytopenia (5,000 platelets/μL) after receiving an influenza vaccination. An 87-year-old woman whose laboratory test values included a platelet count of 2,000/μL experienced genital bleeding after receiving an influenza vaccination...
2016: Case Reports in Hematology
Martha Casallas-Rivera, Álvaro Faccini-Martínez, Natalia Perdomo-Beltrán, Carlos Botero-García, Juan Bravo, Carlos Pérez-Díaz
We report the case of a 56-year-old female patient, with a three-day history of hematemesis, melena, abdominal wall hematoma and epistaxis associated with thrombocytopenia and anemia. Idiopathic thrombocytopenic purpura was diagnosed and she was treated with dexamethasone for four days. The patient developed acute respiratory failure with signs of systemic inflammatory response. Blood and pleural fluid cultures grew Pasteurella canis. This is the first case, to our knowledge, of P. canis empyema associated with hemorrhagic septicemia without epidemiological background and the third case of septicemia caused by P...
February 2016: Revista Chilena de Infectología: órgano Oficial de la Sociedad Chilena de Infectología
Alexandra J Baumann, David S Wheeler, Gabor Varadi, Eyob Feyssa
We present a patient with hepatitis C virus (HCV) and cirrhosis who was treated with eltrombopag for idiopathic thrombocytopenic purpura and was incidentally found to have a right atrial thrombus with extension into the left internal jugular vein. Eltrombopag was discontinued and the patient was treated with thrombectomy and anticoagulation. Given the proposed use of eltrombopag in HCV-associated thrombocytopenia, we advise caution when treating cirrhotics who are at higher intrinsic risk of thrombosis.
January 2016: ACG Case Reports Journal
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