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anemia in aps

Ozan Ünlü, Stephane Zuily, Doruk Erkan
Antiphospholipid syndrome (APS) is the association of thrombosis and/or pregnancy morbidity with antiphospholipid antibodies (aPL). Thirty to forty percent of systemic lupus erythematosus (SLE) patients are tested positive for aPL, which may have an impact on the SLE presentation, management, and prognosis. Compared with SLE patients without aPL, those with aPL have a higher prevalence of thrombosis, pregnancy morbidity, valve disease, pulmonary hypertension, livedo reticularis, thrombocytopenia, hemolytic anemia, acute/chronic renal vascular lesions, and moderate/severe cognitive impairment; worse quality of life; and higher risk of organ damage...
June 2016: Eur J Rheumatol
Øyvind Bruserud, Bergithe E Oftedal, Anette B Wolff, Eystein S Husebye
The gene causing the severe organ-specific autoimmune disease autoimmune polyendocrine syndrome type-1 (APS-1) was identified in 1997 and named autoimmune regulator (AIRE). AIRE plays a key role in shaping central immunological tolerance by facilitating negative selection of T cells in the thymus, building the thymic microarchitecture, and inducing a specific subset of regulatory T cells. So far, about 100 mutations have been identified. Recent advances suggest that certain mutations located in the SAND and PHD1 domains exert a dominant negative effect on wild type AIRE resulting in milder seemingly common forms of autoimmune diseases, including pernicious anemia, vitiligo and autoimmune thyroid disease...
August 6, 2016: Current Opinion in Immunology
Ming Ku, Shuiming Guo, Weifeng Shang, Qing Li, Rui Zeng, Min Han, Shuwang Ge, Gang Xu
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that primarily affects women during their reproductive years. The interaction between SLE and pregnancy remains debated. The objective of this study was to analyze the fetal and maternal outcomes of Chinese women with SLE. A total of 109 pregnancies in 83 SLE patients from June 2004 to June 2014 at a tertiary university hospital were reviewed retrospectively. Patients' characteristics, clinical and laboratory data during pregnancy were obtained from electronic medical records...
2016: PloS One
Yusuke Sakamaki, Konosuke Konishi, Akinori Hashiguchi, Shigeki Tomita, Eiji Kubota, Hiroshi Itoh, Koichi Hayashi
The patient was a 48-year-old Japanese woman diagnosed as having systemic lupus erythematosus at the age of 21 years when she presented with fever and an erythematous skin rash on her face and extremities. Prednisolone was initiated at that time. Thirteen days before admission to our hospital, she was referred to us by her family physician. Upon admission, blood tests showed pancytopenia, hypocomplementemia, and renal dysfunction, as well as the presence of lupus anticoagulant. Urinalysis showed abundant proteinuria and heavy microscopic hematuria...
2016: Nihon Jinzo Gakkai Shi
C Castoro, R Le Moli, M L Arpi, M Tavarelli, G Sapuppo, L Frittitta, S Squatrito, G Pellegriti
PURPOSE: Autoimmune polyendocrine syndromes (APS) type III are characterized by the association of autoimmune thyroid disease (ATD) with other autoimmune diseases such as diabetes, alopecia, pernicious anemia, vitiligo and chronic atrophic gastritis. A strong association between ATD and atrophic gastritis (AG) has been demonstrated. Moreover 10 % of patients affected by AG have a predisposition to develop gastric carcinoid and adenocarcinoma as a result of chronic hypergastrinemia caused by achlorhydria and subsequent ELC cells neoplastic transformation...
July 2016: Journal of Endocrinological Investigation
Ch Lopez-Pedrera, M A Aguirre, P Ruiz-Limon, C Pérez-Sánchez, Y Jimenez-Gomez, N Barbarroja, M J Cuadrado
Antiphospholipid syndrome (APS) is a disorder characterized by the association of arterial or venous thrombosis and/or pregnancy morbidity with the presence of antiphospholipid antibodies (anticardiolipin antibodies, lupus anticoagulant antibodies, and/or anti-β2-glycoprotein I antibodies). Thrombosis is the major manifestation in patients with aPLs, but the spectrum of symptoms and signs associated with aPLs has broadened considerably, and other manifestations, such as thrombocytopenia, non-thrombotic neurological syndromes, psychiatric manifestations, livedo reticularis, skin ulcers, hemolytic anemia, pulmonary hypertension, cardiac valve abnormality, and atherosclerosis, have also been related to the presence of those antibodies...
August 2015: International Immunopharmacology
Dharmendra Bhadauria, Praveen Etta, Anupma Kaul, Narayan Prasad
BACKGROUND: Concurrent thrombotic and hemorrhagic manifestations are uncommon in patients with Antiphospholipid Syndrome. CASE CHARACTERISTICS: A 10-year-old girl with fever, edema, rash and joint pains, who later developed deep venous thrombosis (DVT), stroke, thrombotic microangiopathic hemolytic anemia and pulmonary hemorrhage. Investigations confirmed Antiphospholipid syndrome associated with systemic lupus erythematosus. OUTCOME: She went into complete remission with intravenous immunoglobulins, plasmapheresis, immunosuppression and anticoagulation...
April 2015: Indian Pediatrics
Gonzalo Carreno-Tarragona, Francisco Trapiello-Valbuena, Judit Aranda-Salom, Joaquín Martínez-López, Carlos Lumbreras, Encarnación Oliveira-Ramírez, Carmen Díaz-Pedroche
No abstract text is available yet for this article.
August 2015: Annals of Hematology
Thelma Skare, Renata Damin, Renata Hofius
OBJECTIVE: To study systemic lupus erythematosus in a Brazilian population using the American College of Rheumatology hematological classification criteria and report associations of the disease with serological and clinical profiles. METHODS: This is a retrospective study of 460 systemic lupus erythematosus patients followed in a single rheumatologic center during the last 10 years. Hematological manifestations considered for this study were hemolysis, leukopenia, lymphocytopenia and thrombocytopenia...
March 2015: Revista Brasileira de Hematologia e Hemoterapia
Sergio Umberto De Marchi, Emanuela Cecchin, Sergio De Marchi
INTRODUCTION: Autoimmune polyglandular syndromes (APS) are a group of disorders characterized by the presence of autoimmune processes in several endocrine and non-endocrine organs that are classified into 4 types. METHODS: We describe the case of a woman affected with APS type 3 who presented initially with Hashimoto thyroiditis and pernicious anemia and subsequently developed autoimmune chronic urticaria, myasthenia gravis, and type 1 diabetes mellitus. RESULTS: This patient had a combination of components of APS types 3b, 3a, and 3c...
September 2015: Muscle & Nerve
Erica L Berry, Sara N Iqbal
HELLP syndrome is a collection of symptoms described as hemolysis, elevated liver enzymes and low platelets. HELLP syndrome complicates 0.01-0.6% of pregnancies and can be considered a severe variant of preeclampsia. The occurrence of HELLP syndrome diagnosed before the 20th week of gestation has been most commonly reported in association with antiphospholipid antibody syndrome (APS) or triploid chromosomal anomalies. A 41-year-old primigravida was admitted at 17 weeks and 6 days gestation with hypertension, proteinuria, hemolytic anemia and acute renal injury...
December 2014: Journal of Clinical Gynecology and Obstetrics
Amy E Lin, Nadine Shehata, Heather Reich, David Barth, Carl Laskin, Stephen Lapinsky, Shital Gandhi
OBJECTIVES: We outline a case of a woman with lupus (SLE) and antiphospholipid syndrome (APLA) at 26 weeks' gestation who develops a complex multiorgan condition with a broad differential. METHODS: Case report. RESULTS: A 34 year old woman, G1P0, presented at 26 weeks gestation with a 5 day history of epigastric pain and emesis. She has a history of lupus anticoagulant (LAC), anticardiolipin (ACL) and anti-dsDNA (ADD) positive SLE and APLA syndrome diagnosed at age 13 with multiple small cerebral infarcts...
January 2015: Pregnancy Hypertension
M C Abdulla, J Alungal, S Hashim, M M Ali, M Musambil
A 24 year old female with hereditary spastic paraplegia presented with intermittent headache for one year. She also had lower abdominal pain and vomiting for two months. She was pale, had icterus and mild splenomegaly. On diagnostic evaluation she was found to have hemolytic anemia, thrombocytopenia and bilateral adrenal, subdural, soft tissue (scalp and orbit) hemorrhages due to systemic lupus erythematosus (SLE). However, antiphospholipid syndrome (APS) antibodies were negative. Bilateral adrenal hemorrhage without associated APS is a rare phenomenon in SLE...
September 2015: Lupus
Ignasi Rodríguez-Pintó, Gerard Espinosa, Ricard Cervera
The catastrophic antiphospholipid syndrome (CAPS) is a rare disease that affects 1 % of cases with antiphospholipid syndrome (APS). CAPS can mimic or overlap with different thrombotic diseases; many patients present with a microthrombotic storm or thrombotic microangiopathic hemolytic anemia (TMHA). Thus, the differential diagnosis of CAPS includes thrombotic thrombocytopenic purpura (TTP), typical and atypical hemolytic uremic syndrome (HUS), systemic infections, malignancies, pregnancy-related disorders, malignant hypertension, heparin-induced thrombocytopenia, and drug-induced thrombotic microangiopathies...
January 2015: Current Rheumatology Reports
Iñigo Rúa-Figueroa Fernández de Larrinoa
Systemic lupus erythematosus is a heterogeneous rheumatic systemic disease with extremely varied clinical manifestations and a diverse pathogenesis, as illustrated in this review on the most relevant new knowledge related to the disease. Topics such as anemia, pathogenesis, cardiovascular risk assessment, antiphospholipid syndrome, prediction of damage and recent advances in treatment, including tolerogenic and biological agents, are discussed. Relevant contributions regarding classical therapies such as corticosteroid and antimalarials and their optimal use, as well as the roll of vitamin D, are also referred...
January 2015: Reumatología Clinica
Aurélie Revaux, Arsène Mekinian, Pascale Nicaise, Martine Bucourt, Françoise Cornelis, Eric Lachassinne, Sylvie Chollet-Martin, Olivier Fain, Lionel Carbillon
OBJECTIVES: Chronic intervillositis of unknown etiology (CIUE) is characterized by an intervillous infiltrate of mononuclear cells and a high recurrence rate of adverse obstetrical outcomes. The aim was to describe obstetrical history in patients with at least one event characterized by CIUE, and the possible impact of systematic investigation of an underlying autoimmune disease on the obstetrical outcome of subsequent pregnancies. METHODS: We retrospectively reviewed all pregnancies in patients having experienced at least one adverse obstetric outcome associated with chronic intervillositis of unknown etiology diagnosed by placental histological analysis between 2004 and 2011 in our university hospital...
June 2015: Archives of Gynecology and Obstetrics
Marianna Strakhan, Mariana Hurtado-Sbordoni, Nahun Galeas, Kamila Bakirhan, Karenza Alexis, Tarek Elrafei
Catastrophic antiphospholipid syndrome (CAPS) is a rare but potentially life-threatening condition characterized by diffuse vascular thrombosis, leading to multiple organ failure developing over a short period of time in the presence of positive antiphospholipid antibodies (aPL). CAPS is a severe form of antiphospholipid syndrome, developing in about 1% of cases of classic antiphospholipid syndrome, manifesting as microangiopathy, affecting small vessels of multiple organs. It is acute in onset, with majority of cases developing thrombocytopenia and less frequently hemolytic anemia and disseminated intravascular coagulation...
2014: Case Reports in Hematology
Lishan Guo, Xinhua Qian, Danhua Zhao, Zhuoming Lu, Jianfeng Chen, Qianqian Wu, Meijia Xu
β‑thalassemia is a common genetic disease, characterized by the insufficient production of the hemoglobin (Hb) β‑chain. Upregulation of the expression of fetal hemoglobin (HbF) alleviates the severity of β‑thalassemia. Radix astragali (RA) is a traditional Chinese medicine used in the treatment of anemia. The present study aimed to determine the effectiveness and safety of long‑term RA treatment in non‑transfusion‑dependent (NTDT) β‑thalassemic patients, to evaluate whether responses to RA can be predicted in vitro and to examine the mechanisms underlying RA‑induced HbF synthesis...
November 4, 2014: Molecular Medicine Reports
Subodh Banzal, Abhishek Singhai
Autoimmune polyendocrine syndrome Type II (APS II), also known as polyglandular autoimmune syndrome Type II or Schmidt syndrome, is constellations of multiple endocrine gland insufficiencies. It is a rare, but most common of the immunoendocrinopathy syndrome. It is characterized by the obligatory occurrence of autoimmune Addison's disease in combination with thyroid autoimmune diseases and/or Type I diabetes, hypogonadism, hypophysitis, myasthenia gravis, vitiligo, alopecia, pernicious anemia, and celiac disease...
May 2014: Indian Journal of Critical Care Medicine
Bahar Artim-Esen, Erhan Çene, Yasemin Şahinkaya, Semra Ertan, Özlem Pehlivan, Sevil Kamali, Ahmet Gül, Lale Öcal, Orhan Aral, Murat Inanç
OBJECTIVE: Associations between autoantibodies and clinical features have been described in systemic lupus erythematosus (SLE). Herein, we aimed to define autoantibody clusters and their clinical correlations in a large cohort of patients with SLE. METHODS: We analyzed 852 patients with SLE who attended our clinic. Seven autoantibodies were selected for cluster analysis: anti-DNA, anti-Sm, anti-RNP, anticardiolipin (aCL) immunoglobulin (Ig)G or IgM, lupus anticoagulant (LAC), anti-Ro, and anti-La...
July 2014: Journal of Rheumatology
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