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thrombocytopenia SLE glucocorticoid

P Ungprasert, V R Chowdhary, M D Davis, A Makol
Hematological abnormalities, such as anemia, leucopenia, and thrombocytopenia, secondary to peripheral destruction, are common in systemic lupus erythematosus (SLE). However, cytopenias from autoimmune myelofibrosis (AIMF) are extremely uncommon in SLE, with less than 40 reported cases in the literature. We report the case of a 33-year-old female who presented with bullous skin lesions and pancytopenia as the presenting manifestation of what was ultimately diagnosed as SLE with AIMF. She responded well to glucocorticoids and mycophenolate mofetil...
April 2016: Lupus
Li Wang, Hua Chen, Yao Zhang, Wanli Liu, Wenjie Zheng, Xuan Zhang, Fengchun Zhang
Clinical characteristics of systemic lupus erythematosus (SLE) patients complicated with cerebral venous sinus thrombosis (CVST) between 2000 and 2013 were analyzed through this retrospective, single-centre study. Of 4747 hospitalized SLE patients, 17 (0.36%, 12 females, average age 30) had CVST. Headache (88.2%) was the most common neurological symptom followed by nausea or vomiting (47.1%), conscious disturbance (41.2%), edema of eyelids or conjunctiva (35.3%), blurred vision or diplopia (35.3%), and seizure (35...
2015: Journal of Immunology Research
Anum Fayyaz, Ann Igoe, Biji T Kurien, Debashish Danda, Judith A James, Haraldine A Stafford, R Hal Scofield
Our purpose was to compile information on the haematological manifestations of systemic lupus erythematosus (SLE), namely leucopenia, lymphopenia, thrombocytopenia, autoimmune haemolytic anaemia (AIHA), thrombotic thrombocytopenic purpura (TTP) and myelofibrosis. During our search of the English-language MEDLINE sources, we did not place a date-of-publication constraint. Hence, we have reviewed previous as well as most recent studies with the subject heading SLE in combination with each manifestation. Neutropenia can lead to morbidity and mortality from increased susceptibility to infection...
2015: Lupus Science & Medicine
Pierina Sansinanea, Sebastián Augusto Carrica, Josefina Marcos, Mercedes Argentina García
A case is presented of a protein-losing enteropathy (PLE) as the initial manifestation of systemic lupus erythematosus (SLE) in a 17 year-old female patient, who presented with ascites, edema and hypoalbuminemia. The diagnosis of SLE was based on the presence of: malar rash, oral ulcers, thrombocytopenia, antinuclear antibodies, IgM anticardiolipin antibody, and lupus anticoagulant. Renal and liver diseases were ruled out. The PLE diagnosis was confirmed with fecal alpha 1-antitrypsin clearance. The PLE was refractory to different lines of immunosuppressive agents like glucocorticoids, cyclophosphamide, azathioprine, and cyclosporine, showing a satisfactory and sustained response with rituximab, allowing steroid sparing and long term remission...
January 2016: Reumatología Clinica
Gerardo B Vieregge, Thomas J Harrington, David M Andrews, Maria F Carpintero, Dollie F Green, Ali Nayer
The catastrophic antiphospholipid syndrome (CAPS) is a rare life-threatening form of the antiphospholipid syndrome characterized by disseminated vascular thrombosis resulting in multiorgan failure. On an exceedingly rare occasion, CAPS can be associated with severe hemorrhagic manifestations. We report a young woman with a history of several spontaneous miscarriages who presented with menorrhagia and hemoptysis. The patient developed respiratory failure due to diffuse alveolar hemorrhage. Laboratory tests demonstrated severe hemolytic anemia, profound thrombocytopenia, markedly elevated fibrin degradation products, and renal failure...
2013: Case Reports in Medicine
H Hasegawa, F Mizoguchi, H Kohsaka, N Miyasaka
A 37-year-old male with systemic lupus erythematosus (SLE) presented with high fever, subcutaneous indurations, anemia, thrombocytopenia, elevated liver enzymes and hyperferritinemia. Skin biopsy revealed hemophagocytic histiocytes in the adipose tissues. The patient was diagnosed with SLE with cytophagic histiocytic panniculitis (CHP). Treatment with high-dose glucocorticoids and cyclosporine A induced remission of SLE and CHP. CHP is generally a systemic disorder affecting subcutaneous adipose tissues with a high mortality rate...
March 2013: Lupus
Ahmed Alayoud, Ouadie Qamouss, Amine Hamzi, Mohammed Benyahia, Zouhir Oualim
INTRODUCTION: Connective tissue disorders can overlap in various ways. Patients may present with features of more than one specific disease without satisfying the diagnostic criteria and thereafter evolve into a specific disease entity. Occasionally, patients may fulfil simultaneously the diagnostic criteria of two or more diseases. Several cases of systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) overlap syndrome have been reported. SLE patients often develop lupus nephritis, the treatment of which is based on immunosuppression with corticosteroids (CS) and cytotoxic drugs...
September 2012: Arab Journal of Nephrology and Transplantation
Qing-bian Ma, Wan-zhen Yao, Jian-ming Chen, Hong-xia Ge, Shu Li, Ya-an Zheng
BACKGROUND: Pulmonary embolism (PE) is rare and seldom considered in adolescent patients; however it occurs with a greater frequency than is generally recognized, and it is a potentially fatal condition. The aim of the current study was to understand its epidemiology, clinical features and the cause of delay of its diagnosis in adolescents. METHODS: A retrospective analysis of nine adolescents with acute PE admitted to the Peking University Third Hospital over the past 16-year period was performed...
March 2012: Chinese Medical Journal
Zhe Chen, Mei-ying Liang, Jian-liu Wang
OBJECTIVE: To investigate the etiology and clinical characteristics of pregnancy-emerged thrombocytopenia. METHODS: A retrospective analysis was conducted on clinical data of 159 pregnancies with thrombocytopenia, who were admitted to Peking University People's Hospital from January 2000 to January 2010. All the patients recruited in this study had no history of blood or immune system disease before pregnancy, and thrombocytopenia was the predominate clinical manifestation during pregnancy, with platelet counts less than 100 × 10(9)/L at least twice during pregnancy...
November 2011: Zhonghua Fu Chan Ke za Zhi
Dongyan Shi, Dandan Wang, Xia Li, Huayong Zhang, Nan Che, Zhimin Lu, Lingyun Sun
Umbilical cord-derived mesenchymal stem cell transplantation (UC-MSCT) has been proved to be effective in the treatment of systemic lupus erythematosus (SLE), based on animal experiments and clinical trials. Diffuse alveolar hemorrhage (DAH) is a rare complication of SLE with a high mortality usually over 50%. This study aimed to assess the efficacy of UC-MSCT in the treatment of SLE-associated DAH. Four SLE patients complicated with DAH, who underwent UC-MSCT, were included. Clinical changes before and after transplantation were assessed by measurements of hemoglobin, platelet level, oxygen saturation, and serological factors...
May 2012: Clinical Rheumatology
R K Gupta, A N Ezeonyeji, A S Thomas, M A Scully, M R Ehrenstein, D A Isenberg
Pure red cell aplasia (PRCA) is a recognized but rare complication of systemic lupus erythematosus (SLE) and is characterized by the near absence of red blood cell precursors in the bone marrow but with normal megakaryocyte and granulocytes. We report a novel case of acquired PRCA occurring simultaneously with immune thrombocytopenia in the context of active SLE. Both syndromes were refractory to conventional treatment but responded to rituximab and cyclophosphamide.
December 2011: Lupus
Hua Chen, Wenjie Zheng, Jinmei Su, Dong Xu, Qian Wang, Xiaomei Leng, Wen Zhang, Mengtao Li, Fulin Tang, Xuan Zhang, Xiaofeng Zeng, Yan Zhao, Fengchun Zhang
OBJECTIVES: To evaluate the safety and efficacy of low-dose rituximab therapy for refractory thrombocytopenia in patients with SLE. METHODS: Ten adult SLE patients with severe refractory thrombocytopenia (mean platelet count 10.4 × 10(9)/l) were enrolled in this prospective pilot study. All patients had failed traditional high-dose CSs and immunosuppressants including methylprednisolone pulse therapy. Patients were scheduled to receive i.v. rituximab at a dose of 100 mg once weekly for 4 weeks...
September 2011: Rheumatology
Ling Li, Guang-fu Dong, Feng-zhen Han, Yang Cui, Yun-zhen Shi, Xiao Zhang
OBJECTIVE: To acquire more knowledge about neonatal lupus erythematosus (NLE). METHOD: Seven cases with neonatal lupus erythematosus who were seen in this hospital from 1990 to 2009 are reported in this paper and 87 cases reported previously from 1980 to now in China were reviewed. The clinical manifestations, serum autoantibodies, treatment and results of long-term follow-up are analyzed and summarized. RESULT: Totally 94 cases were summarized...
February 2011: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
A N Dang Do, R A Umoren, S E Tarvin, B R Heilbrunn, A Mahajerin, S L Bowyer
We report a case of a 3-month old male infant, born to a mother with a known history of systemic lupus erythematosus (SLE). The infant initially presented with petechiae, anemia, and thrombocytopenia. His evaluation revealed antinuclear antibody (ANA) titer of 1 : 160, negative anti-SS-A/SS-B antibody, positive anti-Smith antibody, elevated anti-dsDNA titer, and a slightly low C4 level. His subsequent development of hematuria with nephrotic grade proteinuria fulfilled criteria for a diagnosis of SLE. His condition improved with corticosteroids, mycophenolate mofetil and low-dose aspirin...
April 2011: Lupus
Thari Alanazi, Mohammad Alqahtani, Huda Al Duraihim, Khalid Al Khathlan, Bader Al Ahmari, Dorothy Makanjuola, Mohammad Afzal
Clinical and radiological liver diseases are uncommon in patients with systemic lupus erythematosus (SLE). We report a 29-year-old female with SLE who presented with right upper quadrant abdominal pain, thrombocytopenia, elevated liver enzymes and multiple hypodense lesions in the liver on a computed tomography (CT) study that mimicked multiple liver abscesses. A liver biopsy showed mild chronic inflammation. Culture results were negative. With steroid therapy the patient improved clinically, the platelet count returned to the normal range and the multiple liver lesions disappeared radiologicaly...
November 2009: Annals of Saudi Medicine
A Mardjuadi, M Soedirman, Buddy Utoyo, J J Rasker
A 31-year-old Chinese lady presented with severe SLE with nephrotic syndrome, anemia, leucopenia and thrombocytopenia, skin lesions, and joint inflammation after failing previous standard therapy. After treatment with three infusions of rituximab she showed immediate improvements regarding clinical and laboratory parameters. She received no cytotoxic drugs and remained well for at least 7 months, despite stopping prednisolone.
June 2009: Clinical Rheumatology
Géza Vass, Zsuzsanna Antal, Mária Katona, Sarolta Kárpáti, Lídia Balogh
Neonatal lupus erythematosus (NLE) is a disease of the first few months of infancy. It is caused by anti-SSA and anti-SSB antibodies, which are products of maternal autoimmune disorders (SLE, Sjögren, rheumatoid arthritis) and can be passively transported across the placenta. The prevalence of NLE is low. The major clinical findings are cutaneous (typical annular erythematous plaques), cardiac, hepatic and hematologic alterations. Its most severe consequence is third-degree heart block, which is irreversible, requires pacemaker-implantation and responsible for the 20-30% mortality rate...
October 28, 2007: Orvosi Hetilap
Lai-kei Yuen, Wai-ming Lai, Pak-chiu Tong, Wing-tat Poon, Kei-chiu Tse, Man-chun Chiu
Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially fatal disease in childhood. The association of microangiopathic hemolytic anemia, schistocytes, and thrombocytopenia without fever, neurologic, and renal involvement is sufficient to suspect TTP at an early stage for prompt plasma infusion or exchange therapy. TTP has been increasingly described especially in association with systemic lupus erythematosus (SLE). We report the youngest Chinese boy who presented his SLE with TTP and subsequently experienced 9 relapses of TTP in a 2-year period...
August 2007: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
Francisco José García Hernández, Celia Ocaña Medina, Rocío González León, Rocío Garrido Rasco, Regina Colorado Bonilla, María Jesús Castillo Palma, Julio Sánchez Román
BACKGROUND AND OBJECTIVE: To assess the value of rituximab in systemic autoimmune diseases which are refractory to others treatments. PATIENTS AND METHOD: Prospective study on 12 patients -7 with systemic lupus erythematosus (SLE), 4 with Wegener's granulomatosis (WG), and 1 with overlapping connective disease and autoimmune thrombocytopenia-, controlled in a specialized unit of a tertiary hospital. Four weekly doses of rituximab, 2 biweekly doses of cyclophosphamide, and glucocorticoids were administered to all patients, and other immunosuppressants were also administered as considered necessary in each case...
March 31, 2007: Medicina Clínica
Megan E B Clowse, Laurence Magder, Frank Witter, Michelle Petri
OBJECTIVE: Hydroxychloroquine (HCQ) is often needed to manage disease activity in systemic lupus erythematosus (SLE) during pregnancy. The purpose of this study was to examine lupus activity and pregnancy outcomes in women with SLE treated or not treated with HCQ during pregnancy. METHODS: This was a prospective study of pregnancies in women with SLE who were evaluated between 1987 and 2002. The pregnancies were divided into 3 groups: no HCQ exposure during pregnancy (163 pregnancies), continuous use of HCQ during pregnancy (56 pregnancies), or cessation of HCQ treatment either in the 3 months prior to or during the first trimester of pregnancy (38 pregnancies)...
November 2006: Arthritis and Rheumatism
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