Hema Flashcards
stages of hematopoeisis
1. mesoblastic/yolk sac begins 10-14th day of gestation 2. liver begins 6-8wks AOG up to 20-24wks AOG 3. bone marrow
pallor apparent at
7-8 g/dL
autosomal dominant macrocytic anemia, low retic anemia at 2-6mo snub nose hypertelorism flattening of thenar prominence triphalengeal thumb abscence of radial pulse
diamond blackfan
or
congenital hypoplastic anemia
increase in chromosomal breaks when lymphocytes exposed to alkylating agents
fanconi anemia
tx of diamond blackfan
steroids
macrocytic anemia failure to thrive panceratic fibrosis insulin dependent diabetes muscle and neuro impairment villous atrophy with chronic diarrhea early death
pearson syndrome
most common acquired red cell aplasia in childhood
transient erythroblastopenia of childhood
physiologic anemia of infancy at
8-12 weeks
at 11g/dL
physiologic anemia in prematures
3-6weeks
7-9 g/dL
reticulocytosis in IDA tx occur
48-96 hours
expect hg to increase by __ once iron given over __wks
1-2g/dL
4weeks
defect in spherocytosis
spectrin
ankyrin
Cytoskeletal protein defects
Often involve vertical interactions
of spectrin ankyrin, protein 3
Hereditary spherocytosis
lab results Hereditary spherocytosis
Spherocytes on blood film Negative Coombs test eliminates immune hemolysis Increased incubated osmotic fragility Abnormal cytoskeletal protein analysis
Primary acquired marrow disorder
RBCs unusually sensitive to
complement-mediated lysis
Paroxysmal nocturnal
hemoglobinuria
lab result Paroxysmal nocturnal
hemoglobinuria
Decreased WBC CD55 and CD59 or decreased RBC CD59 by flow cytometry Marrow aspirate and biopsy to assess cellularity Decreased decay-accelerating factor
warm antibody
igG
cold antibody
igM
Absence of apolipoprotein β Acanthocytes on blood film Vitamin E deficiency and heightened sensitivity to oxidative damage
Abetalipoproteinemia
tx Abetalipoproteinemia
vit ADEK
folic acid
dietary restriction triglyerides
hemoglobin S due to
single base pair change
thymine for adenine
encodes VALINE instead of GLUTAMINE
sickle cell prophylaxis
oral pen V K 125mg bid up to 3yrs then
250mg bid up to 5 years old
osteomyelitis pathogen in sickle cell
salmonella
staph
splenic sequestration
engorgement of spleen increase in size hypovolemia drop Hg by > 2 g/dL thrombocytopenia reticulocytosis
tx to reduce pain episodes in sickle cell
hydroxyurea
tx of priapism >4hrs sickle cell
aspiration of blood from corpora cavernosa
injection of dilute epinephrine
PRES or
reversible leukoencephalopathy syndrome associated with
headache, confusion, seizures and visual loss
sickle cell
management for sickle cell kid with acute focal neuro deficit
oxygen to keep sats >96
blood transfusion w/in 1 hr to keep Hg > 10g/dL
exchange transfusion (manual or erythrocytapharesis)
CT scan or MRI
goal in blood transfusion tx in sickle cell
keep Hg S < 30% in the first 2 yrs after new stroke then 50% after
tx in ACS sickle cell
macrolide
third gen cephalosporin
pathogen ACS in sickle cell
strep
mycoplasma pneumoniae
chalmydia
cyanosis visible at methemoglobin level
1.5g/24 hr
or
15%
methemoglobin lethal at
70%
tx methemoglobinemia
methyl blue if toxic
vit C if not toxic looking patient
methyl blue should not be given to
g6pd
barts hemoglobin
4 gamma globin
hemoglobin h
4 beta globin
cooley anemia
b thalassemia major
4 alpha globin
features thalassemia
maxilla hyperplasia
frontal bossing
flat nasal bridge