ch 11 pt 2 Flashcards
abnormal hemoglobin production, hb is microcytic and hypochromic
thalassemia
B-thalassemia involves chromosome __ while a-thalassemia involves chromosome __
11
16
a mutation in thalassemia with excess of the opposite globin chain damages RBCs, causing:
hemolysis
B-thalassemia ___ involves 1 allele and is asympt or very mild, while B-thalassemia ___ involves 2 alleles and causes splenomegaly, stunted growth, skeletal abnormalities
minor
major
treatment of B-thal major that decreases anemia symp and deform and extends survival into 20s
blood transfusions, iron chelation
repeated blood transfusions to tx B-thal major can cause eventual iron overload, known as _____, which can cause lethal cardiomyopathy
hemochromatosis
a-thalassemia is less severe than B-thal, causes slight decreased O2 capacity, has ineffective ____, and his highly variable due to there being __ total a-globin genes
erythropoiesis
4
in a-thal, severity is determined by the # of a-globin gene deletions:
- ___ deletion/s is asymp, or a silent carrier
- ___ deletion/s causes no O2 capacity, and lethal hydrops fetalis
1
4
aox that neutralizes oxidants that may cause hemolysis; reduced amounts leaves RBCs oxidative stress
glutathione (GSH)
enzyme needed for GSH synth, deficiency of which causes hemolysis
-x linked, MC in males
G6PD (glucose 6 phosphate dehydrogenase)
G6PD deficiency is typically asymptomatic until the pt is exposed to an environmental stim that creates oxidative stress such as (2):
infections (MC)
ADRs
(fava beans)
a pt with fatigue, pale skin, splenomegaly, back/abdom pain, and hemosiderinuria that occured in acute onset is most likely to have:
G6PD deficiency
2 tx for G6PD deficiency, depending on severity
terminate provocative stim blood transfusion (partial splenectomy)
acquired mutation in myeloid stem cells that increases complement fixation onto RBCs and causes hemolysis
-decreases pH while sleeping, dark urine upon waking
PNH (paroxysmal nocturnal hemoglobinuria)
tx for PNH involves antibodies that inhibit the:
MAC
____ anemias are rare, and involve RBCs being attacked by the immune system, causing opsonization and complement fixation of/onto rbc’s
immunohemolytic
two causes of immuno hemolytic anemia:
-____ which is spontaneous and idiopathic, or ____, caused by ADRs, toxic exposure
endogenous
exogenous
WARM antibody immunohemolytic anemias function at ___ and involve ____, and are MC idiopathic (primary)
COLD antibody immunohemolytic anemias function at
98.6* F
IgG
86* F
IgM
immunohemolytic anemias have subtle features such as chronic/mild anemia, ___, or __ ___
jaundice
raynaud phenomenon
sign of repetitive physical stress to RBCs, such as prosthetic heart valves or narrowing of vasc
traumatic hemolysis
hemolysis caused by the narrowing of vasculature is called ____ hemolytic anemia
microangiopathic
traumatic hemolysis damages RBCs, creating fragmented rbcs called ___, which should prompt investigation into causation
schistocytes
more lethal form of malaria is from ___ ___, from the female anopheles mosquito and cause merozoite showers (fever) every ___ hours
plasmodium falciparum
48
a pt with: episodic shaking, chills and fevers , vomiting, sweating, hemoglobinuria, renal failure, jaundice, splenomegaly, HA, joint pain may have ____!
malaria
___ malaria has cns involvement and may be lethal within days due to RENAL failure
cerebral
