hematologic disease Flashcards
What is the normal size of RBCS?
80-100
what is anemia.?
Disorder which results in decreased numbers of RBC
what is pernicious anemia?
Decrease in Intrinsic factor that results in decrease of vitamin b12 which causes macrocytic anemia.
aplastic anemia definition?
bone marrow disorder that affects myeloid stem cells. Pancytopenia.
What is sickle cell anemia?
Autosomall recessive disorder that causes hemolysis of sickled cells. Valine to glutamic acid mutation.
What are the big concerns with Sickle cell anemia. ?
vaso-occlusive crisis, (acute chest syndrome is specifically of pulmonary capillaries.
Severe hemolytic anemia.
what is the treatment of a vaso-occlusive crisis?
hydration, oxygen, maintain temperature. steroids.
Can use hydroxyurea that creates HbF
what is CML?
chronic myelogenous Leukemia. has Philadelphia chromosome. tyrosine kinase inhibitors to treat. occurs mostly in adults
how to treat ALL?
tyrosine kinase inhibitors. can be cured
what is Hem A and Hemophilia B
disorder of factor 8
factor 9 disorder.
when do you give factor for hemophilia patients?
prior to surgery and within 20 minutes post surgery due to short half life.
What does desmopressin do for bleeding disorders?
causes release of Vw and factor 8.
what is cryoprecipitate and when do you use it?
contains factor 8,9, vwf, fibrinogen.
can use it to treat all types of vwf or hemophilia.
other option is humate P cause it cause vwf and 8
what is the relationship of vwf and factor8
vwf prevents degradation of factor 8.
what is factor 5 leiden?
Gene mutation resulting in resistance to protein c. causes thrombosis.
