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Hematologia

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12 papers 0 to 25 followers
https://www.readbyqxmd.com/read/28189175/autoimmune-hemolytic-anemia
#1
REVIEW
Howard A Liebman, Ilene C Weitz
Autoimmune hemolytic anemia is an acquired autoimmune disorder resulting in the production of antibodies directed against red blood cell antigens causing shortened erythrocyte survival. The disorders can present as a primary disorder (idiopathic) or secondary to other autoimmune disorders, malignancies, or infections. Treatment involves immune modulation with corticosteroids and other agents.
March 2017: Medical Clinics of North America
https://www.readbyqxmd.com/read/26043387/paroxysmal-nocturnal-hemoglobinuria-a-complement-mediated-hemolytic-anemia
#2
REVIEW
Amy E DeZern, Robert A Brodsky
Paroxysmal nocturnal hemoglobinuria is manifests with a chronic hemolytic anemia from uncontrolled complement activation, a propensity for thrombosis and marrow failure. The hemolysis is largely mediated by the alternative pathway of complement. Clinical manifestations result from the lack of specific cell surface proteins, CD55 and CD59, on PNH cells. Complement inhibition by eculizumab leads to dramatic clinical improvement. While this therapeutic approach is effective, there is residual complement activity resulting from specific clinical scenarios as well as from upstream complement components that can account for suboptimal responses in some patients...
June 2015: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/26043385/cold-agglutinin-mediated-autoimmune-hemolytic-anemia
#3
REVIEW
Sigbjørn Berentsen, Ulla Randen, Geir E Tjønnfjord
Cold antibody types account for about 25% of autoimmune hemolytic anemias. Primary chronic cold agglutinin disease (CAD) is characterized by a clonal lymphoproliferative disorder. Secondary cold agglutinin syndrome (CAS) complicates specific infections and malignancies. Hemolysis in CAD and CAS is mediated by the classical complement pathway and is predominantly extravascular. Not all patients require treatment. Successful CAD therapy targets the pathogenic B-cell clone. Complement modulation seems promising in both CAD and CAS...
June 2015: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/26043384/warm-autoimmune-hemolytic-anemia
#4
REVIEW
Rakhi Naik
Warm autoimmune hemolytic anemia (AIHA) is defined as the destruction of circulating red blood cells (RBCs) in the setting of anti-RBC autoantibodies that optimally react at 37°C. The pathophysiology of disease involves phagocytosis of autoantibody-coated RBCs in the spleen and complement-mediated hemolysis. Thus far, treatment is aimed at decreasing autoantibody production with immunosuppression or reducing phagocytosis of affected cells in the spleen. The role of complement inhibitors in warm AIHA has not been explored...
June 2015: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/24418298/diagnosis-and-classification-of-autoimmune-hemolytic-anemia
#5
REVIEW
Garrett F Bass, Emily T Tuscano, Joseph M Tuscano
Uncompensated autoantibody-mediated red blood cell (RBC) consumption is the hallmark of autoimmune hemolytic anemia (AIHA). Classification of AIHA is pathophysiologically based and divides AIHA into warm, mixed or cold-reactive subtypes. This thermal-based classification is based on the optimal autoantibody-RBC reactivity temperatures. AIHA is further subcategorized into idiopathic and secondary with the later being associated with a number of underlying infectious, neoplastic and autoimmune disorders. In most cases AIHA is confirmed by a positive direct antiglobulin test (DAT)...
April 2014: Autoimmunity Reviews
https://www.readbyqxmd.com/read/22330255/diagnosis-and-treatment-of-cold-agglutinin-mediated-autoimmune-hemolytic-anemia
#6
REVIEW
Sigbjørn Berentsen, Geir E Tjønnfjord
Exact diagnosis of the subtype has essential therapeutic consequences in autoimmune hemolytic anemia. Cold-antibody types include primary chronic cold agglutinin disease (CAD) and rare cases of cold agglutinin syndrome (CAS) secondary to cancer or acute infection. Primary CAD is a clonal lymphoproliferative disorder. Not all patients require pharmacological therapy, but treatment seems indicated more often than previously thought. Corticosteroids should not be used to treat primary CAD. Half of the patients respond to rituximab monotherapy; median response duration is 11 months...
May 2012: Blood Reviews
https://www.readbyqxmd.com/read/20650555/immune-hemolytic-anemia-associated-with-drug-therapy
#7
REVIEW
George Garratty
Drug-induced immune hemolytic anemia (DIIHA) is rare; it can be mild or associated with acute severe hemolytic anemia (HA) and death. About 125 drugs have been implicated as the cause. The HA can be caused by drug-independent antibodies that are indistinguishable, in vitro and in vivo, from autoantibodies causing idiopathic warm type autoimmune hemolytic anemia (AIHA). More commonly, the antibodies are drug-dependent (i.e., will only react in vitro in the presence of the drug). The most common drugs to cause DIIHA are anti-microbials (e...
July 2010: Blood Reviews
https://www.readbyqxmd.com/read/20548093/how-i-treat-autoimmune-hemolytic-anemias-in-adults
#8
REVIEW
Klaus Lechner, Ulrich Jäger
Autoimmune hemolytic anemia is a heterogeneous disease with respect to the type of the antibody involved and the absence or presence of an underlying condition. Treatment decisions should be based on careful diagnostic evaluation. Primary warm antibody autoimmune hemolytic anemias respond well to steroids, but most patients remain steroid-dependent, and many require second-line treatment. Currently, splenectomy can be regarded as the most effective and best-evaluated second-line therapy, but there are still only limited data on long-term efficacy and adverse effects...
September 16, 2010: Blood
https://www.readbyqxmd.com/read/25808506/western-immunoblotting-as-a-new-tool-for-investigating-direct-antiglobulin-test-negative-autoimmune-hemolytic-anemias
#9
COMPARATIVE STUDY
Evgenia M Bloch, Darinka Sakac, Haley A Branch, Christine Cserti-Gazdewich, Jacob Pendergrast, Katerina Pavenski, Donald R Branch
BACKGROUND: Direct antiglobulin test-negative (DAT(-)) autoimmune hemolytic anemia, characterized by hemolysis without detectable immunoglobulin or complement on patient red blood cells (RBCs), poses a diagnostic challenge. To select therapy, classification of the hemolysis as immune- or non-immune-mediated is important. We developed a method using Western immunoblot (WB) to classify DAT(-) patients by measuring and comparing levels of RBC immunoglobulin (Ig)G to normal donors. STUDY DESIGN AND METHODS: RBC samples from 42 normal donors were made into ghosts and analyzed by WB and densitometry to establish a normal mean relative quantity of IgG (RQIgG) on the RBCs...
June 2015: Transfusion
https://www.readbyqxmd.com/read/26582375/complement-in-hemolytic-anemia
#10
REVIEW
Robert A Brodsky
Complement is increasingly being recognized as an important driver of human disease, including many hemolytic anemias. Paroxysmal nocturnal hemoglobinuria (PNH) cells are susceptible to hemolysis because of a loss of the complement regulatory proteins CD59 and CD55. Patients with atypical hemolytic uremic syndrome (aHUS) develop a thrombotic microangiopathy (TMA) that in most cases is attributable to mutations that lead to activation of the alternative pathway of complement. For optimal therapy, it is critical, but often difficult, to distinguish aHUS from other TMAs, such as thrombotic thrombocytopenic purpura; however, novel bioassays are being developed...
November 26, 2015: Blood
https://www.readbyqxmd.com/read/25271314/treatment-of-autoimmune-hemolytic-anemias
#11
REVIEW
Alberto Zanella, Wilma Barcellini
Autoimmune hemolytic anemia (AIHA) is a relatively uncommon disorder caused by autoantibodies directed against self red blood cells. It can be idiopathic or secondary, and classified as warm, cold (cold hemagglutinin disease (CAD) and paroxysmal cold hemoglobinuria) or mixed, according to the thermal range of the autoantibody. AIHA may develop gradually, or have a fulminant onset with life-threatening anemia. The treatment of AIHA is still not evidence-based. The first-line therapy for warm AIHA are corticosteroids, which are effective in 70-85% of patients and should be slowly tapered over a time period of 6-12 months...
October 2014: Haematologica
https://www.readbyqxmd.com/read/26494915/so-you-know-how-to-treat-iron-deficiency-anemia
#12
COMMENT
Stanley L Schrier
In this issue of Blood, Moretti et al provide data that challenge the entrenched oral treatment of iron deficiency anemia. The paper shows how the newer understanding of hepcidin and iron metabolism in general can lead to very practical improvements in the management of iron deficiency anemia, a disorder that may affect as many as 1 billion people.
October 22, 2015: Blood
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