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70 Cards in this Set

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what is hemopexin
heme binding protein (has the highest heme binding affinity) that transports heme to the liver
when are hemopexin and haptoglobin depleted?
in the face of massive hemolysis, they bind to heme faster than they can be produced (and are therefore not detectable in the blood)
How does the adminsitration of methylene blue distiguish cause of methemoglobinemia
methemoglobinemia will not correct when the cause if hemoglobin M disease
symptoms of cytochrome b5 reductase deficiency
MR, FTT, +/- seizures, neurologic problems, most die infancy
treatment for methemoglobinemia
methylene blue
ascorbic acid
what is the mutation in hemoglobin M?
tyrosine substitute for histadine
what are dohle bodies
blue staining reminent RER aggreagates found in the cytoplasm of granulocytes

seen in May Hegglin anomaly
inheritance of may hegglin
AD
pathophys of may hegglin
mutation of the MYH9 gene present on chromosome 22q12-13,
results in disordered production of nonmuscle myosin heavy chains type IIA.
half life of random donor platelets
3 days
mode of inheritence of congenital dyserythropoietic anemia
I - AR
II- AR
III - AD
radiographic finding in CDA III
hair on end appearance on skull radiograph
which CDA has in increased incidence of lymphoproliferative disorders
III
red cell size in CDA
macrocytic - can be normal in II
Ham test in CDA
I - negative
II - positive
III - negative
anti- I agglutinability in CDA
I- normal
II - strong
III - normal
what is the HAM test
lysis of RBCs in acidified samples

(looks for fragility of RBCs in mild acid environment)
what differentiates PNH from CDA when the HAM test is positive?
clinical picture

sugar water hemolysis test: positive in PNH (cells lyse in a low salt sucrose solution),
CDA: negative sucrose
what is the effect of polycythemia on PTT?
can falsely elevate PTT b/c there is an insufficient amount of citrate in the test tube.
what is prolonged bleeding from the umbilical stump usually due to?
factor 13 deficiency
test for factor 13 deficiency?
euglobulin lysis test (ELT)
tests for PLASMIN activity (measure of fibrinolysis)

without factor 13 you can make aclot but it is an unstable clot.
will PT/PTT be abnormal in factor 13 deficiency?
no
subacute side effect of mediastinal radiation
radiation pneumonitis
presents with cough and dyspnea
bilateral parahilar infiltrates
treatment of radiation pneumonitis
steroids
timeframe of radiation pneumonitis
few weeks to months
result of ATIII deficiency
hypercoaguable
why hypercoaguable in nephrotic syndrome
preferential loss of ATIII
mechanism of action for tranexamic acid
inhibitor of plasminogen activation
diagnosis with nearly absent IgG
SCID
what is the clinical manifestation of Hemoglobin Chesapeake?
mild POLYCYTHEMIA
what does hemoglobin lepore act like
beta thalessemia
what is the pathophysiology of hgb lepore?
it is a beta/delta fusion gene b/c it is under the regulation of delta globin regulation it is produced at low levels
what is the binding affinity of hemoglobin zurich for carbon monxide?
very high, the hemoglobin is stabilized by carbon monoxide
hereditary pyropokilocytosis
congenital hemolytic anemia
spectrin chain defect
increased ratio of cholesterol to membrane protein
what is the effect on engraftment of t cell depleted BMT
decreased engraftment
when is amicar contraindicated
hematuria
liver disease
DIC
how will factor 8 levels differ in severe DIC vs. advanced hepatocellular carcinoma
factor 8 will be low in DIC not in advanced hepatocellular carcinoma as factor 8 is made in sites other than the liver
imaging study for follow-up of synovial cell sarcoma
MRI
what lab abnormality does IIbIIIa deficiency cause
prolonged bleeding time
pernicious anemia
absence of intrinsic factor
can be due to gastric atrophy
can be due to autoimmunity against intrinsic factor and can be associated with hypothyroidism
risk of RB to offspring of mom with bilateral RB and normal dad
40%
what underlying diagnosis makes a bili of 24 in a neonate more concerning for kernicterus
hereditary spherocytosis
what is the MCV in diamond blackfan
increased
what does activated protein c do ?
inactivates factor 5a
is candidiasis seen in SCID orCGD
SCID
catalase positive bacterial infections seen in CGD
6 day old with DIC, purpura fulminans could have...
homozygous protein S deficiency
which renal tumor has the worst prognosis
rhabdoid
at what inhibitor level do you switch from giving factor 8 to giving something else
> 10 bethesda units
treatment for CGD with aspergillosis
ampho B plus gamma interferon
liver tumor with best prognosis
fetal hepatoblastoma
3 day old male with eccymosis bleeding umbilical cord normal platelets, prolonged PT, PTT
afibrinogenemia
what type of testing is used for evaluating DR in HLA typing
oligonucleatide
12 yo with blurred right vision, partially detached retina, hgb 12, TARGET cells
hgb sc
what morphologic finding differentiates hgb beta thal/hgb E from homozygous HGB E
Homozygous HGb E has target cells
what does isopropanol precipitation test test for
unstable hemoglobin
hemoglobin zurich and isopropanol test
postive test
what type of hemolytic anemia is associated with hemoglobin zurich
oxidant induced
how is protein c activated
endothelial surface thrombin - thrombomodulin interaction
what is the cause of death in hermansky pudlak syndrome
pulmonary fibrosis
what is the quebec platelet disorder? (factor V Quebec)
AD inheritance
mild thrombocytopenia
increased megas
defective EPINEPHRINE induced platelet aggregation
decreased concentration of granule proteins
what does the MYH9 gene code for
non-muscle myosin heavy chain IIA
gene mutation in wilms aniridia
11p13
difference in location for embryonal vs alveolar RMS
embryonal: head and neck, orbit, GU
Alveolar: extremities, trunk
cell variant of paratesticular RMS
spindle cell
location of botyroid variant of RMS
bladder, vagina, nasopharynx, bile duct
what is the mutation in constant spring

what type of hemoglobin is elevated
point mutation in alpha globin - doesn't get made

Hgb H (beta tetramer)
where does thrombin to thrombomodulin take place
endothelial cell
"infectious" complication of IVIG
aseptic meningitis
what is Chuvash polycythemia
AR inheritance mutation of von-Hippel Lindau gene

causing increased production of erythropoietin
what are the oncologic complications ofvon Hippel Landau syndrome
hemangioblastomas
pheochromocytoma
renal cell carcinoma