Chapter 11 Part II (QUIZ) Flashcards
Sickle cell anemia is autosomal ______ with an mutation/instability of the _____ globin
recessive
B-globin
8% of African Americans are ______ or _____
heterozygous carriers
sickling –> _______ obstruction
microvascular obstruction
locations of sickle cell anemia occurs in areas of _____; bone marrow, spleen; usually has symptoms of _____ and _____/inflammation
stasis
dehydration
infection
thrombosis, chronic low-level pain, joint pain, priapism, fever, malaise, splenomegaly/infarction, gallstones are symptoms of
sickle cell anemia
heterozygous carriers for sickle cell anemia are RARELY _____ ; homozygous: ___% survive beyond 5th decade
symptomatic
50%
MC cause of death in someone with SCA (2)
acute chest syndrome
stroke
MC location of congestion/infarction for SCA
bone marrow
vertebral involvement in SCA is called _______; microvascular endplate infarctions with central endplate depression
Lincoln log vertebra (H-shaped vertebra)
thalassemia is autosomal ________ with abnormal ______ PRODUCTION
recessive
hemoglobin
hemoglobin shape in thalassemia (2)
microcytic (small)
hypochromic (pale)
areas of endemic malaria (2)
africa
SE asia
B-thalassemia is on chromosome ____
11
A-thalassemia is on chromosome ___
16
mutation in thalassemia is excess of the ________ globin chain
opposite
in thalassemia, there is damage to _____ called HEMOLYSIS and damage to ____
RBCs
erythroblasts
in ______ thalassemia, there is decreased B-globin chain synthesis and excess of unpaired A chains
B-thalassemia
aka B-thalassemia trait; features are ASYMPTOMATIC or MILD; normal life span of RBC; treatment is often not necessary
B-thalassemia minor
type of B-thalassemia that has severe hemolysis/anemia with SKELETAL DEFORMITIES; leads to eventual IRON OVERLOAD
b-thalassemia major
the eventual iron OVERLOAD in b-thalassemia is called _______ and leads to lethal ______
hemochromatosis
cardiomyopathy
a-thalassemia involves ____ a-globin gene, and _____ B-globin
mutated
excess
a-thalassemia is ____ severe than b-thalassemia
less
__-thalassemia is HIGHLY variable, with __ total globin chains
a-thalassemia
4 (higher # = more severe)
function is to neutralize oxidants that may cause hemolysis
glutathione (GSH)
enzyme needed for GSH synthesis
G6PD
a type of hemolytic anemia in which a deficiency –> DECREASE GSH –> HEMOLYSIS
glucose 6-phosphate dehydrogenase deficiency (G6PD)
MC method of decrease GSH production is
deficiency of G6PD
who is most affected with G6PD?
males (x-linked)
MC environmental stimulus that creates oxidative stress with G6PD (asymptomatic until environmental stimulus)
infection
others are ADRs and fava beans
oxidation in G6PD is associated with _____ PHAGOCYTOSIS
splenic
splenic macrophages that are due to loss of deformability
bite cells (G6PD)
oxidized Hb = clumps; damages RBC membrane –> hemolysis
Heinz bodies (G6PD)
acute onset, fatigue, pale skin, splenomegaly, back of abdominal pain, hemosideruria; hemolysis 2-3 days after exposure
G6PD
treatment of G6PD depends on ______; identify/______ provocative stimulus, possible _____ or partial splenectomy
severity
terminate
blood transfusion
rare type of hemolytic anemia that is ACQUIRED in MYELOID stem cells
paroxysmal nocturnal hemoglobinuria (PNH)
gene involved in PNH
PIGA
in paroxysomal noctural hemoglobinuria, there is _____ complement fixation onto RBC’s, also ____ urine UPON WAKING because of ______ while sleeping
increase
dark
decrease pH
increase of thrombosis and ___ anemia is MC with PNH
mild
treatment for PNH
antibodies that inhibit the MAC
type of hemolytic anemia in which antibodies bind RBCs; rare
*chronic/mild anemia and Raynaud phenomenon
immunohemolytic anemias
in immunohemolytic anemias, there is ______ of RBCs and complement fixation onto RBCs
opsonization
more immunohemolytic anemias are _____ (IDIOPATHIC and SPONTANEOUS) than ______ (ADRs, toxic exposure)
endogenous
exogenous
_____ antibody immunohemolytic anemias are at 98.6 F and antibody is ____
warm
IgG
____ antibody immunohemolytic anemias are at
cold
IgM
hemolytic anemia with sign of repetitive physical stress to RBCs
traumatic hemolysis
traumatic hemolysis can occur incidentally during any activity, but more common with ________ and ______
prosthetic heart valves
narrowing of vasculature
hemolytic anemia that is observed in pathologic states in which small vessels become partially obstructed or narrowed by lesion that predispose passing red cells to mechanical damage
microangiopathic hemolytic anemia
morphologic alterations in injured RBCs are called
schistocytes
(2) alterations in schistocytes
burr cells
helmet cells
