QUANTITATIVE Flashcards
decreased hemoglobin production?
thalessemia
B thalassemia is affected how genetically?
B-chain genes, one on each chromosome 11
affect of B allele?
normal, non mutated, full synthesis
affect of B+ alleles?
produce B globin in reduced quantity
affect of B not allele?
absence of B globin synthesis
severe microcytic hypochromic anemia
B-thalassemia major
falls between major and minor in terms of clinical severity
patients only require occasional transfusions
B not/B or B+/B+
B-thalassemia intermedia
what type of thalassemia is this?
4 alpha chain genes, one pair on each chromosome 16
a-thalassemia
for alpha-thalassemia, what predisposes someone to this?
number of deleted genes (severity)
locations of deleted genes (inheritance)
name this type of a-thalassemia trait?
microcytic, hypo chromic anemia
polychromasia and mild anisocytosis
target cells
more common in African descent, -alpha
”–” more common in asians
a-thalassemia trait
name this type of a-thalassemia?
B globin tetramers (B4) form due to markedly decreased levels of alpha chains
HbH molecules form insoluble aggregates, both chronic hemolytic anemia and splenomegaly
HbH disease (–/-alpha)
name this type of a-thalassemia?
total absence of alpha chains
HbH and Hb Barts produced but ineffective at oxygen transport
incompatible with life in utero
frequent cause of still birth in SE Asia
hydros fetalis (–/–)
varying quantities of HbH and Hb Barts seen, depending on severity of the disease and patient age
affect on HbA
HbA2/HbF
electrophoresis, most present at birth
decreased
normal
ineffective erythropoiesis resulting in low RBC mass
iron deficiency
ineffective globin production
marrow compensates by increasing RBC mass
thalassemia
