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Hematology
Red Blood Cell Disorders
32
Medical
Undergraduate 1
09/12/2011

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Term
Anemias
Definition
diseases characterized by low oxygen transport capacity of the blood, because of low red cell count or some abnormality of the red blood cells or the hemoglobin.
Term
Aplastic anemia
Definition
is a condition where bone marrow does not produce sufficient new cells to replenish blood cells.
Term
Atransferrinemia
Definition
is an autosomal recessive metabolic disorder in which there is absence of transferrin, a plasma protein that transports iron through the blood.
Term
Autoimmune hemolytic anemia
Definition
is a type of hemolytic anemia where the body's immune system attacks its own red blood cells (RBCs), leading to their destruction (hemolysis)
Term
Cavernous sinus thrombosis
Definition
Cavernous sinus thrombosis (CST) is the formation of a blood clot within the cavernous sinus. One possible cause may be the spread of a dental infection in a tooth of the maxilla (upper jaw).
Term
Evans syndrome
Definition
is an autoimmune disease in which an individual's antibodies attack their own RBCs as well as their platelets
Term
Haemophilia
Definition
is a group of hereditary genetic disorders that impair the body's ability to control blood clotting or coagulation. In its most common form, Hemophilia A, clotting factor VIII is absent.
Term
Haemorrhagic disease of the newborn
Definition
is a coagulation disturbance in newborns due to Vitamin K deficiency. As a consequence of Vitamin K deficiency there is an impaired production of coagulation.
Term
Hemoglobinemia
Definition
is a medical condition in which there is an excess of Hemoglobin in the blood plasma. This is because of intravascular hemolysis, in which hemoglobin separates from red blood cells, a form of anemia.
Term
Hemolytic anemia
Definition
is anemia due to hemolysis, the abnormal breakdown of red blood cells either in the blood vessels (intravascular hemolysis) or elsewhere in the body.
Term
Haemolytic disease of the newborn
Definition
is an alloimmune condition that develops in a fetus, when the IgG antibodies that have been produced by the mother and have have passed through the placenta include ones which attack the red blood cells in the fetal circulation.
Term
Hereditary spherocytosis
Definition
is a genetically-transmitted form of spherocytosis, an auto-hemolytic anemia characterized by the production of red blood cells that are sphere-shaped rather than dount-shaped, and therefore more prone to hemolysis.
Term
Hypochromic anemia
Definition
is a generic term for any type of anemia in which the red blood cells (erythrocytes) are paler than normal.
Term
Idiopathic cold hemaggulatinin syndrome
Definition
IgM binds to red blood cell surface antigens in the cold. This can occur in a susceptible individual as blood passes through cold extremities in cold weather.
Term
Macrocytic anemia
Definition
is an anemia (defined as blood with an insufficient concentration of hemoglobin) in which the erythrocytes ("red blood cells" or RBCs) are larger than their normal volume.
Term
Megaloblastic anemia
Definition
is an anemia (of macrocytic classification) which results from inhibition of DNA synthesis in red blood cell production.
Term
Methemoglobinemia
Definition
is a disorder characterized by the presence of a higher than normal level of methemoglobin in the blood. Methemoglobin is a form of hemoglobin that does not bind oxygen.
Term
Paroxysmal cold hemoglobinuria
Definition
is a disease of humans that is characterized by the sudden presence of hemoglobin in the urine.
Term
Polycythemia
Definition
is a condition in which there is a net increase in the total number of blood cells, primarily red blood cells, in the body
Term
Sickle-cell disease
Definition
is a blood disorder characterized by red blood cells that assume an abnormal, ridid, sickle shape. Sickling decreases the cells' flexibility and results in their restricted movement through blood vessels, depriving downstream tissues of oxygen.
Term
Spherocytosis
Definition
RBCs or erythrocytes, that are sphere-shaped, rather than donut-shaped or, more specifically, bi-concave disc shaped. It is caused by a molecular defect in one or more of the proteins of the red blood cell cytoskeleton.
Term
Thalassemia
Definition
is an inherited autosomal recessive blood disease. In thalassemia, the genetic defect results in reduced rate of synthesis of one of the globin chains that make up hemoglobin.
Term
Warm Antibody Autoimmune Hemolytic Anemia
Definition
is the most common of the autoimmune hemolytic diseases. About half of the cases are idiopathic, with the other halp attributable to a predisposing condition or medications being taken.
Term
Microcytic Anemia
Definition
is a generic term for any type of anemia characterized by small red blood cells. The normal mean corpuscular volume (abbreviated to MCV on full blood count results) is 76-100 fL, with smaller cells )<76 fL) described as microcytic and larger cells (>100 fL) as macrocytic.
Term
Iron deficiency anemia
Definition
is the most common type of anemia, and is also known as sideropenic anemia. It is the most common cause of microcytic anemia.
Term
Sideroblastic anemia
Definition
is caused by the abnormal production of red blood cells, usually as part of myelodysplastic syndrome, (1) which can evolve into hematological malignancies (especially acute myelogenous leukemia). Thus, the body has iron available, but cannot incorporate it into hemoglobin.
Term
Lead poisoning
Definition
is a medical condition caused by increased levels of the metal lead in the blood. Lead may cause irreversible neurological damage as well as renal disease, cardiovascular effects, and reproductive toxicity.
Term
Chronic Obstructive Pulmonary Disease
Definition
is a disease of the lungs where the airways become narrowed. This leads to a limitation of the flow of air to and from the lungs causing shortness of breath.
Term
Vitamin B12 deficiency
Definition
is a reduction in vitamin B12 from inadequate dietary intake or impaired absorption. The condition is commonly asymptomatic, but can also present as anemia characterized by enlarged blood corpuscles, so called megaloblastic anemia.
Term
Perniciious anemia
Definition
is a form of megaloblastic anemia due to vitamin B12 deficiency, caused by impaired absorption of vitamin B12 (1) due to the absence of intrinsic factor.
Term
Alpha (α) thalassemias
Definition
There are four genetic loci for a globin, two of which are maternal in origin and two of which are paternal in origin. The severity of the α thalassemias is correlated with the number of affected a globin loci: the greater the number of affected loci, the more severe will be the manifestations of the disease.
Term
Beta β thalassemias
Definition
Beta thalassemias are due to mutations in the HBB gene on chromosome 11, also inherited in an autosomal-recessive fashion. The severity of the disease depends on the nature of the mutation.
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