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Blood Disorders
Hematologic diseases are disorders which primarily affect the blood and Blood formation, blood-forming organs. Hematologic diseases include rare genetic disorders, anemia, HIV, sickle cell disease and complications from chemotherapy or transfusions. Myeloid * Hemoglobinopathy, Hemoglobinopathies (congenital abnormality of the hemoglobin molecule or of the rate of hemoglobin synthesis) ** Sickle cell disease ** Thalassemia ** Methemoglobinemia * Anemias (lack of red blood cells or hemoglobin) ** Iron-deficiency anemia ** Megaloblastic anemia *** Vitamin B12, Vitamin B12 deficiency **** Pernicious anemia *** Folate deficiency ** Hemolytic anemias (destruction of red blood cells) *** Genetic disorders of RBC membrane **** Hereditary spherocytosis **** Hereditary elliptocytosis **** Congenital dyserythropoietic anemia *** Genetic disorders of RBC metabolism **** Glucose-6-phosphate dehydrogenase deficiency (G6PD) **** Pyruvate kinase deficiency *** Immune mediated hemolytic anemia (Co ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Blood
Blood is a body fluid in the circulatory system of humans and other vertebrates that delivers necessary substances such as nutrients and oxygen to the cells, and transports metabolic waste products away from those same cells. Blood is composed of blood cells suspended in blood plasma. Plasma, which constitutes 55% of blood fluid, is mostly water (92% by volume), and contains proteins, glucose, mineral ions, and hormones. The blood cells are mainly red blood cells (erythrocytes), white blood cells (leukocytes), and (in mammals) platelets (thrombocytes). The most abundant cells are red blood cells. These contain hemoglobin, which facilitates oxygen transport by reversibly binding to it, increasing its solubility. Jawed vertebrates have an adaptive immune system, based largely on white blood cells. White blood cells help to resist infections and parasites. Platelets are important in the clotting of blood. Blood is circulated around the body through blood vessels by the ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hereditary Spherocytosis
Hereditary spherocytosis (HS) is a congenital hemolytic disorder wherein a genetic genetic mutation, mutation coding for a structural membrane protein phenotype causes the red blood cells to be sphere-shaped (spherocytosis), rather than the normal biconcave disk shape. This abnormal shape interferes with the cells' ability to flex during blood circulation, and also makes them more prone to hemolysis, rupture under osmotic stress, mechanical stress, or both. Cells with the dysfunctional proteins are degraded in the spleen, which leads to a shortage of erythrocytes and results in hemolytic anemia. HS was first described in 1871, and is the most common cause of inherited hemolysis in populations of northern European descent, with an incidence of 1 in 5000 births. The clinical severity of HS varies from mild (symptom-free carrier), to moderate (anemic, jaundiced, and with splenomegaly), to severe (hemolytic crisis, in-utero hydrops fetalis), because HS is caused by genetic mutations i ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Alloimmune
Alloimmunity (sometimes called isoimmunity) is an immune response to nonself antigens from members of the same species, which are called alloantigens or isoantigens. Two major types of alloantigens are blood group antigens and histocompatibility antigens. In alloimmunity, the body creates antibodies (called alloantibodies) against the alloantigens, attacking transfused blood, allotransplanted tissue, and even the fetus in some cases. Alloimmune (isoimmune) response results in graft rejection, which is manifested as deterioration or complete loss of graft function. In contrast, autoimmunity is an immune response to the self's own antigens. (The ''allo-'' prefix means "other", whereas the ''auto-'' prefix means "self".) Alloimmunization (isoimmunization) is the process of becoming alloimmune, that is, developing the relevant antibodies for the first time. Alloimmunity is caused by the difference between products of highly polymorphic genes, primarily genes of the major histocompa ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Infectious Mononucleosis
Infectious mononucleosis (IM, mono), also known as glandular fever, is an infection usually caused by the Epstein–Barr virus (EBV). Most people are infected by the virus as children, when the disease produces few or no symptoms. In young adults, the disease often results in fever, sore throat, lymphadenopathy, enlarged lymph nodes in the neck, and fatigue (medical), fatigue. Most people recover in two to four weeks; however, feeling tired may last for months. The liver or spleen may also become swollen, and in less than one percent of cases splenic rupture may occur. While usually caused by the Epstein–Barr virus, also known as human herpesvirus 4, which is a member of the herpesviridae, herpesvirus family, a few other viruses and the protozoon ''Toxoplasma gondii'' may also cause the disease. It is primarily spread through saliva but can rarely be spread through semen or blood. Spread may occur by objects such as drinking glasses or toothbrushes or through a cough or sneez ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Paroxysmal Cold Hemoglobinuria
Paroxysmal cold hemoglobinuria (PCH) or Donath–Landsteiner hemolytic anemia (DLHA) is an autoimmune hemolytic anemia featured by complement-mediated intravascular hemolysis after cold exposure. It can present as an acute non-recurrent postinfectious event in children, or chronic relapsing episodes in adults with hematological malignancies or tertiary syphilis. Described by Julius Donath (1870–1950) and Karl Landsteiner (1868–1943) in 1904, PCH is one of the first clinical entities recognized as an autoimmune disorder. Paroxysmal cold hemoglobinuria is a result of cold-reacting antibody immunoglobulin (Ig) induced hemolytic response inside vessels leading to anemia and, thus, a cold antibody autoimmune hemolytic anemias (CAAHA). In most patients with DLHA, the antibody selectively targets against the red blood cells on-surface antigen called the antigen P or antigen I, respectively. Most cases were found to be owing to polyclonal IgG. Nonetheless, IgM-induced DLHA ha ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Cold Agglutinin Disease
Cold agglutinin disease (CAD) is a rare autoimmune disease characterized by the presence of high concentrations of circulating cold sensitive antibodies, usually IgM and autoantibodies that are also active at temperatures below , directed against red blood cells, causing them to agglutinate and undergo lysis. It is a form of autoimmune hemolytic anemia, specifically one in which antibodies bind red blood cells only at low body temperatures, typically 28–31 °C. When affected people's blood is exposed to cold temperatures ( to ), certain proteins that normally attack bacteria (IgM antibodies) attach themselves to red blood cells and bind them together into clumps (agglutination). This eventually causes red blood cells to be prematurely destroyed (hemolysis) leading to anemia and other associated signs and symptoms. Cold agglutinin disease can be primary (unknown cause) or secondary, due to an underlying condition such as an infection, another autoimmune disease, or certa ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Evans Syndrome
Evans syndrome is an autoimmune disease in which an individual's immune system attacks their own red blood cells and platelets, the syndrome can include immune neutropenia. These immune cytopenias may occur simultaneously or sequentially. Its overall phenotype resembles a combination of autoimmune hemolytic anemia and immune thrombocytopenic purpura. Autoimmune hemolytic anemia is a condition in which the red blood cells that normally carry oxygen are destroyed by an autoimmune process. Immune thrombocytopenic purpura is a condition in which platelets are destroyed by an autoimmune process. Platelets are a component of blood that contribute to the formation of blood clots in the body to prevent bleeding. The syndrome was first described in 1951 by R. S. Evans and colleagues. Signs and symptoms The symptoms of Evans syndrome vary between patients depending on which blood cells are affected. If red blood cells are attacked, symptoms may include weakness and fatigue, paleness or j ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Systemic Lupus Erythematosus
Lupus, formally called systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. Symptoms vary among people and may be mild to severe. Common symptoms include painful and swollen joints, fever, chest pain, hair loss, mouth ulcers, swollen lymph nodes, feeling tired, and a red rash which is most commonly on the face. Often there are periods of illness, called flares, and periods of remission during which there are few symptoms. Children up to 18 years old develop a more severe form of SLE termed childhood-onset systemic lupus erythematosus. The cause of SLE is not clear. It is thought to involve a combination of genetics and environmental factors. Among identical twins, if one is affected there is a 24% chance the other one will also develop the disease. Female sex hormones, sunlight, smoking, vitamin D deficiency, and certain infections are also believed to increa ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Warm Antibody Autoimmune Hemolytic Anemia
Warm antibody autoimmune hemolytic anemia (WAIHA) is the most common form of autoimmune haemolytic anemia. About half of the cases are of unknown cause, with the other half attributable to a predisposing condition or medications being taken. Contrary to cold autoimmune hemolytic anemia (e.g., cold agglutinin disease and paroxysmal cold hemoglobinuria) which happens in cold temperature (28–31 °C), WAIHA happens at body temperature. Causes AIHA may be: * Idiopathic, that is, without any known causeAUTOIMMUNE HEMOLYTIC ANEMIA (AIHA) By J.L. Jenkins. The Regional Cancer Center. 2001 * Secondary to another disease, such as an antecedent upper respiratory tract infection, |
Autoimmune Hemolytic Anemia
Autoimmune hemolytic anemia (AIHA) occurs when a person's immune system produces antibodies directed against their own red blood cells (RBCs). These antibodies attach to red cells, causing them to break down ( lyse), and reducing the number of oxygen-carrying red blood cells in circulation (anemia). The antibodies are usually directed against common red cell antigens, therefore they also bind to allogenic or transfused red cells and cause them to lyse. (ref). Autoimmune haemolytic anaemia can be caused by different types of antibodies with reactivity at different temperatures. The one caused by IgG antibodies is called warm-immune haemolytic anaemia and has an incidence of 5-10 cases per million whereas ‘cold agglutinin disease’ is caused by IgM antibodies with an incidence of 1-1.8 cases per million. The terminology used in this disease is somewhat ambiguous. Although MeSH uses the term "autoimmune hemolytic anemia", some sources prefer the term "immunohemolytic anemia" so dr ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Coombs Test
The direct and indirect Coombs tests, also known as antiglobulin test (AGT), are blood tests used in immunohematology. The direct Coombs test detects antibodies that are stuck to the surface of the red blood cells. Since these antibodies sometimes destroy red blood cells they can cause anemia; this test can help clarify the condition. The indirect Coombs test detects antibodies that are floating freely in the blood. These antibodies could act against certain red blood cells; the test can be carried out to diagnose reactions to a blood transfusion. The direct Coombs test is used to test for autoimmune hemolytic anemia, a condition where the immune system breaks down red blood cells, leading to anemia. The direct Coombs test is used to detect antibodies or complement system, complement proteins attached to the surface of red blood cells. To perform the test, a blood sample is taken and the red blood cells are washed (removing the patient's plasma and unbound antibodies from the red b ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Pyruvate Kinase Deficiency
Pyruvate kinase deficiency is an inherited metabolic disorder of the enzyme pyruvate kinase which affects the survival of red blood cells. Both autosomal dominant and recessive inheritance have been observed with the disorder; classically, and more commonly, the inheritance is autosomal recessive. Pyruvate kinase deficiency is the second most common cause of enzyme-deficient hemolytic anemia, following G6PD deficiency. Signs and symptoms Symptoms can be extremely varied among those suffering from pyruvate kinase deficiency. The majority of those suffering from the disease are detected at birth while some only present symptoms during times of great physiological stress such as pregnancy, or with acute illnesses ( viral disorders). Symptoms are limited to or most severe during childhood. Among the symptoms of pyruvate kinase deficiency are: * Mild to severe hemolytic anemia * Cholecystolithiasis * Tachycardia * Hemochromatosis * Icteric sclera * Splenomegaly * Leg ulcers * Jaundi ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
