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Guillermo Montalban-Bravo, Guillermo Garcia-Manero
DISEASE OVERVIEW: The myelodysplastic syndromes (MDS) are a very heterogeneous group of myeloid disorders characterized by peripheral blood cytopenias and increased risk of transformation to acute myelogenous leukemia (AML). MDS occurs more frequently in older males and in individuals with prior exposure to cytotoxic therapy. DIAGNOSIS: Diagnosis of MDS is based on morphological evidence of dysplasia upon visual examination of a bone marrow aspirate and biopsy. Information obtained from additional studies such as karyotype, flow cytometry or molecular genetics is usually complementary and may help refine diagnosis...
January 2018: American Journal of Hematology
Antonio Girolami, Martina Treleani, Emanuela Bonamigo, Valentina Tasinato, Bruno Girolami
Venous thrombosis usually involves the veins of the limbs, most frequently the leg veins. All other venous districts may sometimes be affected by the thrombotic process. Sometimes, the thrombotic occlusion of the veins of a given region show typical signs and symptoms. In other cases, the picture may not be clear and a high degree of clinical suspicion is needed for a correct approach to patient diagnosis and management. Thrombosis of retinal and jugular veins, right heart thrombosis including thrombosis of coronary sinus and thrombosis of the azygos system may be included in this group...
February 2014: Seminars in Thrombosis and Hemostasis
Walter Ageno
Venous thromboembolism that occurs in unusual sites is challenging because of the potential severity of presentation, the presence of some major provoking risk factors, the high prevalence of potential contraindications to antithrombotic therapies, the lack of solid evidence to guide therapeutic decisions, and because of the severity of long-term consequences. For example, venous thrombosis in the splanchnic veins frequently occurs in patients with liver cirrhosis. Not uncommonly, these patients present with concomitant active gastrointestinal bleeding, and/or low platelet count or oesophageal varices...
April 2015: Journal of Thrombosis and Thrombolysis
Walter Ageno, Jan Beyer-Westendorf, David A Garcia, Alejandro Lazo-Langner, Robert D McBane, Maurizio Paciaroni
Venous thromboembolism (VTE) is a serious and often fatal medical condition with an increasing incidence. The treatment of VTE is undergoing tremendous changes with the introduction of the new direct oral anticoagulants and clinicians need to understand new treatment paradigms. This manuscript, initiated by the Anticoagulation Forum, provides clinical guidance based on existing guidelines and consensus expert opinion where guidelines are lacking. In this chapter, we address the management of patients presenting with venous thrombosis in unusual sites, such as cerebral vein thrombosis, splanchnic vein thrombosis, and retinal vein occlusion...
January 2016: Journal of Thrombosis and Thrombolysis
Kim Ma, Phillip Wells, Charlotte Guzman, David Anderson, Mark Blostein, Andrew Hirsch, Alejandro Lazo-Langner, Michael J Kovacs, Marc Rodger, Vicky Tagalakis, Susan R Kahn
Unusual site deep vein thrombosis (USDVT) is an uncommon form of venous thromboembolism (VTE) with heterogeneity in pathophysiology and clinical features. While the need for anticoagulation treatment is generally accepted, there is little data on optimal USDVT treatment. The TRUST study aimed to characterize the epidemiology, treatment and outcomes of USDVT. From 2008 to 2012, 152 patients were prospectively enrolled at 4 Canadian centers. After baseline, patients were followed at 6, 12 and 24months. There were 97 (64%) cases of splanchnic, 33 (22%) cerebral, 14 (9%) jugular, 6 (4%) ovarian and 2 (1%) renal vein thrombosis...
August 2016: Thrombosis Research
Atisa Koonarat, Ekarat Rattarittamrong, Adisak Tantiworawit, Thanawat Rattanathammethee, Sasinee Hantrakool, Chatree Chai-Adisaksopha, Lalita Norasetthada
: The primary objective was to demonstrate the different risk factors among patients presenting with usual and unusual site venous thromboembolism (VTE). The secondary objectives were to compare clinical characteristics and outcomes in each group. This was a retrospective observational cohort study. Patients admitted in Chiang Mai University Hospital who were diagnosed with VTE during January 2010 through December 2012 were included and classified into two groups: the usual and unusual site VTE. The usual sites of VTE included pulmonary embolism and lower extremity deep vein thrombosis...
January 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
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
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
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
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
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
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
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
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
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
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
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
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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