topics inn ped hematology Flashcards
what is the difference in affinity to oxygen between HBA and HbF ?
HbF has a higher affinity for oxygen than HbA
generally what are the causes of anemia ?
increased destruction : hemolysis
decreased production
iron deficiency
what diseases are associated with reduced production of RBC ?
diamond-blackfan anemia
TEC transient erythroblastopenia of children
what is diamond blackfan anemia ?
isolated red cell aplasia
what are the differential diagnosis of diamond blackfan anemia ?
congenital parvovirus B19
TEC
what is TEC ?
benign self limiting condition
immune mediated
what is the hemolysis profile or workup ?
retics
LDH
haptoglobin
unconjugated bilirubin
what type of organisms does the spleen help in the protection against ?
encapsulated bacteria
what is the pattern of inheritance of SCD ?
autosomal recessive
what are the hallmarks of SCD ?
pain due to obstruction
anemia due to hemolysis
susceptibility to infections due to auto infarction of the spleen
what are the different crises in SCD ?
acute chest syndrome aplastic crisis hyper-hemolytic crisis splenic sequestrian overt stroke
what are the characteristic features of the blood smear in SCD ?
howell jolly bodies
heinz bodies
what are howell jolly bodies an indication of ?
splenectomy
or functional asplenia
what are the symptoms in SCD ?
painful erection in males
extreme tiredness
shortness of breath or chest pain
jaundice
what is the prophylactic treatment of SCD in neonates ?
start penicillin at 3 months
when do symptoms of SCD begin to appear ?
at 5-6 months when HbF levels start to decrease
what is the long term monitoring for children with SCD ?
TCD to check for CVA
what are the disease modifying options in SCD ?
chronic blood transfusion
hydroxyurea
bone marrow transplantation
what is the problem with chronic blood transfusion ?
iron overload so chelating agents are used like deferoxamine and exjade
how does hydroxyurea work in SCD ?
increases levels of HbF so decreases sickling
what is the problem with bone marrow transplantation ?
infertility
what is the differential diagnosis for microcytic anemia ?
iron deficiency anemia sideroblastic anemia chronic illness thalassemia trait lead toxicity
how is a definitive diagnosis of thalassemia achieved ?
electrophoresis
beta thalassemia major would show low levels of HbA , elevated HbF and HbA2
minor - high levels of HbA2
what are the bone deformities associated with thalassemia ?
skull bossing
flat nasal bridge
long bone deformities