Penyakit Sistem hematologi 1.

anemiaabnormal blood constituents resulting from various etiologies; anemia is a symptom and is often a result of the decrement in blood constituents, although some forms of elevated blood components that are nonfunctional may be referred to as an anemia 2. angiogenesisthe formation of new blood vessels and expanded systems for nutrient delivery and waste removal; a result of chemokines and hormonal messages that up-regulate the formation of these processes; cancer cells can produce various messengers that trigger this up-regulation, thus allowing the rapidly dividing abnormal cells to acquire materials for growth and spread 3. antidiuresisinhibition of water losses through the kidneys reaction to hormones and abnormal cell signals, which reduce tubular losses 4. apheresisremoval of harmful blood components or substitution of more desirable constituents in blood 5. aplastic anemiaidiopathic anemia from abnormal, defi cient, or absent red cell production due to bone marrow disorders 6. atrophic gastritisatrophy of the lining of the stomach, which contains the parietal cells that produce intrinsic factor, proteases, and hydrochloric acid; this form of infl ammation is often accompanied by bacterial overgrowth due to elevation of the gastric pH 7. bilirubinthe metabolite of heme breakdown excreted with bile; build-up of bilirubin and related compounds causes accumulation in extra-hepatic tissues such as the skin and sclera of the eye 8. chronic myeloproliferative diseaselong-term hyperplasia of hematological tissues, with concomitant overproduction of abnormal cells, growth factors, chemokines, cytokines, and hormones involved in hematopoiesis 9. cicatrixscar tissue formation with calcifi cation or hardening of the connective tissue used in

repair of tissue damage 10. cytokinesglycoproteins that act as local messengers or regional hormones; hundreds of different cytokines have been discovered and categorized, all with very distinct functions in the homeostatic and genetic controls over cellular function 11. dextranshydrophilic branched product of carbohydrate metabolism [(C6H10O5)11] used to expand plasma volume and chelate iron in solution 12. DMT-1transmembrane protein divalent metal transporter that traffi cs iron 13. eryptosissuicidal death of erythrocytes characterized by cell shrinkage, membrane loss, cell disintegration, and engulfi ng by macrophages 14. erythroblastosis fetalisan antigen-induced hemolytic anemia of the newborn or premature infant, as a result of incompatibility of maternal Rh factors with the neonate 15. erythropoiesisproduction of erythrocytes or red blood cells 16. erythropoietinthe hormone produced in the kidney that regulates marrow production of red blood cells 17. ferritinthe storage protein for iron 18. ferroportintransmembrane iron export regulating protein 19. hematocritpacked red blood cell volume expressed as a percentage of whole blood upon centrifugation 20. hematopoiesisproduction of blood cells 21. hemeiron-containing, nonprotein portion of the hemoglobin molecule that contains iron in the ferrous (_3) state 22. hemochromatosisiron overload; elevated levels of iron that can cause tissue damage, especially in the liver 23. hemoconcentrationthe decrease in free water circulating in the blood supply, causing increased levels of proteins, electrolytes, wastes, and nutrients per deciliter of blood; elevations of

several laboratory values are present, and dehydration signs and symptoms may be present 24. hemoglobinthe four-pyrrole ring compound in red blood cells that contains iron centers and is responsible for the transport of oxygen 25. hemoglobinemiaexcess free hemoglobin build-up in circulation 26. hemoglobinuriaexcessive free hemoglobin spillage into the urine 27. hemojuvelinprotein sensor for iron levels in tissues 28. hemolytic anemiaan anemia brought on by the rapid, premature destruction of red blood cells in circulation, which may be precipitated by vitamin E defi ciency 29. hemophiliaan inherited disorder of blood clotting, with pronounced bleeding upon tissue injury 30. hemostasisnormal blood fl ow and blood clotting 31. hepcidinhormone regulating iron homeostasis 32. hephestinmembrane-bound, copper-containing oxidizing protein that incorporates iron into transferrin 33. histoneclass of simple proteins found in the cells nucleus 34. HLA antigensantigens specifi c to the individual that cause a rejection reaction in a host receiving transplantation or foreign cells 35. holotranscobalaminthe fraction of metabolically active B12 that is composed of cobalamin linked to transcobalamin in circulation 36. hypochromicabnormally pale in color upon visual inspection under a microscope 37. iatrogenic, iatrogenicityharm caused by treatment or procedures performed by health care personnel or sustained through hospitalization, medical intervention, or prescription 38. intrinsic factor (IF)the protein produced by the parietal cells in the stomach lining that is responsible for the pick-up of cyanocobalamin from protein in foods 39. kernicterusinfi ltration of excessive amounts of bilirubin into the neurons of the spinal cord

and brain 40. leukocyteswhite blood cells (WBC); a generic term for several types of WBC that arise from the same parent cell in the bone marrow 41. leukocytopoiesisthe production of all categories of white blood cells from the pluripotent(able to differentiate into multiple cell types) stem cells found in the bone marrow 42. macrocyticrefers to abnormally large cell size megaloblasticrefers to an immature, large red blood cell that is oval in shape and abnormal 43. microcyticrefers to abnormally small cell size 44. osteopetrosisdeath of bone cells through excessive calcifi cation 45. pancytopeniaa reduction in the numbers of all the blood elementswhite, red, other cells, and proteins 46. pernicious anemiathe anemia associated with B12 defi ciency that is slow, aggressive, and potentially life threatening; it is specifi c to gastrointestinal dysfunction, namely, to gastric enterocytic atrophy, with diminished availability of intrinsic factor, HCl, and enzymes; neuropathy (especially peripheral) results from prolonged defi ciency; the nervous system has a decreased ability to regenerate as well as regain function and feeling in the affected areas 47. phlebotomyblood removal through a venous puncture; blood draw 48. picaeating of abnormal items, or non-nutritive substances, such as laundry starch, clay, ice, dirt, paint chips, etc. 49. plasmathe portion of the blood in which blood constituents are dissolved or suspended; it contains water, proteins, electrolytes, gases, nonproteinaceous compounds, wastes, and nutrients 50. porphyriaa cluster of blood-related disorders characterized by abnormal porphyrin synthesis or metabolism; these disorders are hereditary and vary greatly depending upon which enzyme in the cascade of reactions is affected 51. prohepcidinprecursor to hepcidin; a marker for hemostasis 52. prophylaxispreventative administration of a

compound to avoid consequences of a disease state 53. protoporphyrinthe derivative of hemoglobin containing four pyrrole rings without the iron centers 54. reticulocytesimmature red blood cells; normal ranges for circulating erythrocytes exist, and levels refl ect the ability of the bone marrow to produce precursor cells in normal amounts 55. serumthe fraction of blood containing water after the removal of cellular components 56. sickle cell anemiaa hereditary disease of genetically altered red blood cells that have a sickled shape, carry abnormally formed hemoglobin, and have abnormal transport capabilities for oxygen; the disease is thought to confer protection against malaria 57. sideroblastic anemiaa form of anemia characterized by the appearance of sideroblasts, immature ferritin-containing blast marrow cells in circulation 58. systemic lupus erythematosus (SLE)an autoimmune, chronic infl ammatory disease that affects the connective tissue; affects skin, joints, kidneys, central nervous system, and mucous membranes and eventually spreads to all tissues, invoking a systemic reaction with pain, fever, sensitivity to light, and skin lesions 59. thalassemiaa group of related blood disorders involving abnormal globin subunits in the hemoglobin molecule; these are hereditary and are most common in persons of Mediterranean or southeastern Asian descent 60. thrombocytesplatelets; essential to blood clotting, these pieces of larger immature cells contribute to the formation of a thrombus (clot) by aggregating (coalescing) upon chemical activation after endothelial wall (blood vessel) tissue injury 61. thrombopoietina stimulatory protein in red bone marrow that responds to the need for more platelets post-injury; causes an increase in the production of new platelets and also signals

other systems to speed up the maturation and activation of the new platelets; up-regulates the complex mechanisms in hemostasis under conditions of injury and trauma 62. total iron-binding capacity (TIBC)the capacity for the binding of iron by blood constituents; a surrogate measure for transferrin, since it binds the majority of iron 63. transcobalamin IIIIa group of proteins that are responsible for the transfer of vitamin B12 64. transferrinthe protein responsible for the transport of iron 65. transferrin saturationthe saturation of the carrier protein for iron, which is a sensitive indicator of iron status and stages of anemia 66. von Willebrand factora protein on the platelet membrane surface that is sensitive to the chemical signals of an injured cell and causes the platelet to become sticky and adhere to other platelets and blood constituents