topics inn ped hematology

Question 1

what is the difference in affinity to oxygen between HBA and HbF ?

Answer 1

HbF has a higher affinity for oxygen than HbA

Question 2

generally what are the causes of anemia ?

Answer 2

increased destruction : hemolysis
decreased production
iron deficiency

Question 3

what diseases are associated with reduced production of RBC ?

Answer 3

diamond-blackfan anemia

TEC transient erythroblastopenia of children

Question 4

what is diamond blackfan anemia ?

Answer 4

isolated red cell aplasia

Question 5

what are the differential diagnosis of diamond blackfan anemia ?

Answer 5

congenital parvovirus B19

TEC

Question 6

what is TEC ?

Answer 6

benign self limiting condition

immune mediated

Question 7

what is the hemolysis profile or workup ?

Answer 7

retics
LDH
haptoglobin
unconjugated bilirubin

Question 8

what type of organisms does the spleen help in the protection against ?

Answer 8

encapsulated bacteria

Question 9

what is the pattern of inheritance of SCD ?

Answer 9

autosomal recessive

Question 10

what are the hallmarks of SCD ?

Answer 10

pain due to obstruction
anemia due to hemolysis
susceptibility to infections due to auto infarction of the spleen

Question 11

what are the different crises in SCD ?

Answer 11

acute chest syndrome 
aplastic crisis 
hyper-hemolytic crisis
splenic sequestrian 
overt stroke

Question 12

what are the characteristic features of the blood smear in SCD ?

Answer 12

howell jolly bodies

heinz bodies

Question 13

what are howell jolly bodies an indication of ?

Answer 13

splenectomy

or functional asplenia

Question 14

what are the symptoms in SCD ?

Answer 14

painful erection in males
extreme tiredness
shortness of breath or chest pain
jaundice

Question 15

what is the prophylactic treatment of SCD in neonates ?

Answer 15

start penicillin at 3 months

Question 16

when do symptoms of SCD begin to appear ?

Answer 16

at 5-6 months when HbF levels start to decrease

Question 17

what is the long term monitoring for children with SCD ?

Answer 17

TCD to check for CVA

Question 18

what are the disease modifying options in SCD ?

Answer 18

chronic blood transfusion
hydroxyurea
bone marrow transplantation

Question 19

what is the problem with chronic blood transfusion ?

Answer 19

iron overload so chelating agents are used like deferoxamine and exjade

Question 20

how does hydroxyurea work in SCD ?

Answer 20

increases levels of HbF so decreases sickling

Question 21

what is the problem with bone marrow transplantation ?

Answer 21

infertility

Question 22

what is the differential diagnosis for microcytic anemia ?

Answer 22

iron deficiency anemia 
sideroblastic anemia 
chronic illness
thalassemia trait 
lead toxicity

Question 23

how is a definitive diagnosis of thalassemia achieved ?

Answer 23

electrophoresis
beta thalassemia major would show low levels of HbA , elevated HbF and HbA2
minor - high levels of HbA2

Question 24

what are the bone deformities associated with thalassemia ?

Answer 24

skull bossing
flat nasal bridge
long bone deformities

Question 25

what are the red cell membrane diseases defects ?

Answer 25

hereditary spherocytosis

and hereditary elliptocytosis

Question 26

what is pagophagia ?

Answer 26

a type of pica associated with chewing ice and is associated with iron deficiency anemia

Question 27

what are the lab findings in iron deficiency anemia ?

Answer 27

everything is low except TIBC

Question 28

what does anemia of chronic disease look like in comparison to iron deficiency anemia ?

Answer 28

TIBC is low
anemia is normocytic normochromic
serum ferritin is normal

Question 29

what genetic defect is associated with iron deficiency anemia ?

Answer 29

TMPRSS6