Skel D part II Flashcards
what is abnormal is sickle cell anemia
Hb S
what causes the sickling phenomenon
substitution of valine for glutamic acid on beta globulin chain
what is true SCA
sickle cell anemia
homozygous HbSS
what is the initial sign of SCA? and when do they manifest
hand/foot syndrome:
painful swelling dt infarct or salmonella infection
6 months
common pathology associated with SCA
ACN of femoral and humeral heads
anemia–> spenic enlargement then atrophy
what is also known as sickle cell crisis
mesenteric infarcts
common xray features of SCA
honeycombing of cortex--> hair on end appearance in skull signs of AVN H shaped vertebra splenic calcifications (30%) extra medullary hematopoiesis
aka for vertebral shape in SCA
fish vertebra
h shaped
step down sign
reynold phenomenon
what infection are SCA patients prone to? where? apperance
salmonella (100x)
femur, tibia,fibula, humerus, radius, ulna, spine
bilateral, symmetric and in diaphysis
motheaten destruction and periosteal reaction
thalassemia aka
cooley anemia
Mediterranean anemia
how many types of thalassemia are there
3
Major (die age 3-4)
Minor
Intermediate
what is the cause of thalassemia
imbalance globulin chain production (alpha/beta)
what type of cells are present with thalassemia
hypochomic and microcytic
s/s associated with thalassemia
joint pain scoliosis LBP pallor, lethargy rodent face (maxillary over growth)
xray findings with thalassemia
coarse trabeculae (honey combing)+ thin cortex erlenmeyer flask deformity from lack of normal metaphyseal diaphysela concave constriction