Hemoglobinopathy - Hemorrhage

hemoglobinopathy

(hē″mō-glō′bĭ-nŏp′ă-thē) Any one of a group of genetic diseases caused by or associated with the presence of one of several forms of abnormal hemoglobin in the blood. SEE: hemoglobin.

hemoglobin SC disease

(hē′mŏ-glō″bĭn) A hemolytic anemia in people who have inherited two abnormal forms of hemoglobin, S and C.

SYMPTOMS: Those who inherit this hemoglobinopathy may have vaso-occlusive crises similar to those seen in sickle cell anemia, including interruptions in blood supply (infarcts) in bones and internal organs.

hemoglobin S disease

(hē′mŏ-glō″bĭn) A genetic variant of hemoglobin that causes sickle cell trait when it is inherited from one parent (and therefore heterozygous) and sickle cell anemia when it is inherited from both parents (and therefore homozygous).

INCIDENCE: It is esp. common in people of African ancestry, in whom the sickle cell trait is found in 8% to 10% of the population. SEE: sickle cell anemia.

hemoglobinuria

(hē″mŏ-glō-bĭn-ūr′ē-ă) [hemoglobin + -uria] The presence in urine of hemoglobin free from red blood cells. It occurs when the amount of hemoglobin from disintegrating red blood cells or from rapid hemolysis of red cells exceeds the ability of the blood proteins to combine with the hemoglobin. hemoglobinuric (-bĭn-ur′ik), adj.

ETIOLOGY: Causes include hemolytic anemia, scurvy, purpura, exposure to or ingestion of certain chemicals, such as arsenic and phosphorus, typhoid fever, and septicemia.

 cold h. Hemoglobinuria following local or general exposure to cold. SYN: paroxysmal h.

 epidemic h. Hemoglobinuria of the newborn characterized by jaundice, cyanosis, and fatty degeneration of heart and liver. SYN: Winckel disease.

 intermittent h. Paroxysmal nocturnal h.

 malarial h. Blackwater fever.

 march h. Urinary bleeding that occurs following strenuous exercise, e.g., running a marathon.

 paroxysmal h. Intermittent, recurring attacks of bloody urine following exposure to cold (cold hemoglobinuria) or strenuous exercise (march hemoglobinuria). It results from increased fragility of red blood cells or presence of a thermolabile autohemolysin.

 paroxysmal nocturnal h. ABBR: PNH. A rare form of an acquired hemolytic anemia that results from a defect in membrane-anchored proteins of red blood cells.

 SYMPTOMS: The syndrome is characterized by acute onset of fevers and chills, back and extremity pain, and abdominal cramps. Hemoglobinuria occurs if enough red blood cells have been destroyed.

 TREATMENT: Erythropoietin may be used to treat the anemia of PNH. SYN: intermittent h.

 toxic h. Hemoglobinuria resulting from toxic substances such as muscarine or snake venom; toxic products of infectious diseases such as yellow fever, typhoid fever, syphilis, and certain forms of hemolytic ...