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
endocrine abnormality in thalassemia
hypothyroidism
hypogonadotropic gonadism
hypoparathyrodism
diabetes mellitus
iron chelators
deferoxamine
deferasirox
alpha thalassemia trait
deletion of 2 alpha globin
silent alpha thalassemia
deletion of 1 alpha globin
episodic hemolytic anemia
g6pd deficiency
heinz body
hemolysis
like g6pd deficiency
most common cause of drug-immune hemolytic anemia.
Cephalosporin
concomitant immune thrombocytopenic purpura with autoimmune hemolytic anemia
Evans syndrome
Warm antibody
IgG
Cold antibody
IgM
When to stop Prednisone in autoimmune hemolytic anemia
once the direct Coombs test result becomes negative.
Tx in cold autoimmune hemolytic anemia
Rituximab
hyperpigmentation cafe au lait vitilogo panyctopenia absent thumbs horseshoe or absent kidneys short hypogonadal
fanconi anemia
cbc monitoring in fanconi anemia every
1-3 months
tx for fanconi anemia
stem cell
pancytopenia low igG fialure to thrivve pancreatic insufficiency - failure of acinar development short stature bifid thumb
shwachman diamond syndrome
refractory sideroblastic anemia
cytoplasmic vacuolization
met acid
exocrine pancreastic insufficiency
pearson syndrome
reticulate skin pigmentation
mucosal leukoplakia
nail dystrophy
bone marrow failure
Zinsser-Cole-Engman syndrome
or
Dyskeratosis congenita
dx for paroxysmal nocturnal hemoglobinuria
CD55
CD 59
loose joints
lax skin
easy bruising
ehlers danlos
surface activation of clotting
intrinsic
tisse facter mediation activation of clotting
extrinsic
mixing studies result
normal plasma added to test plasma 1:1
if normalized PT/PTT after then deficiency in factor
if still abnormal PT/PTT then inhibitor is present
hallmark of hemophilic bleeding is
hemarthrosis
hemostatic level factor VIII
> 30 - 40%
hemostatic level factor IX
> 25 - 30%
formula to compute for dose factor VIII
%desired (rise in factor VIII) x wt x 0.5
formula to compute for dose factor IX
%desired (rise in factor IX) x wt x 1.4
desmopressin DDAVP for
hemophilia A factor VIII
most common hereditary bleeding disorder
von willebrand disease
von willebrand disease if VWF levels
<30 IU
__ can increase VWF and factor VIII level
desmopressin DDAVP
rapidly spreading purpuric lesions from thromboses of small dermal vessels followed by bleeding into skin
purpura fulminans
management purpura fulminans
FFP
venous and arterial thromboses
deficiency in cystathione b synthase
homocystinuria
tx homocystinuria
vit B6
factor VIII is also considered as
acute phase reactant
most common thrombotic event in neonates
renal artery thrombosis
t or f
heparin crossed the placenta
false
heparin contraindicated in
bacterial endocarditis cns bleed bleed in the body recent eye, brain, spine surgery lumbar puncture
heparin can be neutralized with
protamine sulfate
warfarin affects
factor 10, 9, 7, 2
protein c
protein s
warfarin effect enhanced by
antibiotics anabolic steroids chloral hydrate laxatives allopurinol methylphenidate hydrochloride vitamin e
warfarin effect diminished by
oral contraceptive
barbiturates
vit k
phenytoin
t or f
warfarin crosses placenta
true
what clotting factor is NOT produced in the liver
factor VIII 8
most common acquired anticoagulat inhibitor
lupus anticoagulant
thrombocytopenia with small platelets eczema recurrent infection x linked WAS protein
wiskott aldrich
tx of ITP
IVIG 0.8g to 1g/kg/day for 1-2 days
drugs that cause thrombocytopenia
valproin acid phenytoin carbamazepine sulfonamide cotrimoxazole vancomycin
fever microangiopathic hemolytic anemia thrombocytopenia abnormal renal fxn CNS changes
thrombotic thrombocytopenic purpura
ttp cause
acquired deficiency of metalloproteinase (ADAMTS 13)
giant hemangioma
localized intravascular coagulation
thrombocytopenia
hypofibrinogenemia
kasabach meritt syndrome
thrombocytopenia (absent/hypoplasia megarkaryocytes)
absent radius
cows milk allergy (diarrhea, eosinophilia, leukemoid rxn)
TAR
thrombocytopenia absent radius syndrome
torch infection that causes thrombocytopenia
rubella
cmv
syphilis
toxoplasmosis
MOA of aspirin
irreversibly acetylates cyclooxygenase (impt in thromboxane)
absence of VWF/glycoprotein Ib receptor
bernard soulier syndrome
defect in platelet aggregation (deficiency in platelet fibrinogen receptor)
glanzmann thrombastenia
tx for platelet dysfxn
desmopressin
platelet transfusion
