Hem Onc Flashcards
Aplastic crises in sickle cell child
parvovirus b19
massive transfusions can cause hypocalcemia why?
whole blood and packed cells contain citrate anticoagulant which can chelate if given in large doses
hereditary index most specific for spherocytosis
elevation in mean corpuscular hemoglobin concentration
which region of the lymph node is populated by T lymphocytes and dendritic cells
paracortex of lymph node
region of lymph node poorly developed in digeorge
paracortex -
acute phase reactant synthesized by the liver that acts as a central regulator of iron homeostasis - influences body iron through interaction with ferroportin - transmembrane protein responsible for transferring intracellular iron into circulation
hepcidin - upon binding hepcidin, ferroportin is internalized and degraded, decreasing intestinal absorption and inhibiting the release of iron by macrophages (high iron levels thus would increase synthesis)
Transmigration of neutrophils out of the vasculature - i.e. squeezing between endothelial cells - is mediated by integrin attachments and adherence to
platelet endothelial cell adhesion molecule (PECAM-1) - this protein is found primarily at the peripheral intercellular junctions of endothelial cells
can lead to increased intracranial pressure in babies from not receiving appropiriate peri-natal care?
intracranial hemorrhage from vitamin K deficiency and thus ineffective clotting carboxylation
enzyme affected in acute intermittent porphyria
porphobiliongen deaminase (PBG deaminase)
enzyme targeted in in acute intermittent porphyria treatment?
ALA synthase - we want to suppress it! Give dextrose (inhibits PPAR-gamma, a TF for ALAsyn) and heme
avoid alcohol, smoking and p450 inducers
porphyria cutanea tarda - enzyme targeted
later in pathway of heme synthesis so don’s see toxic metabolite buildup causing neurologic symptoms -
uroporphyrinogen decarboxylase
porphyria cutanea tarda - signs
blistering cutaneous photosensitivity
The 5 Ps of Acute intermittent porphyria
pain tummy port wine pee polyneuropathy psych precipitated by p450 inducers (and alcohol and starving)
What should you think of if you see increased 5-HIAA in 24 hour urine
carcinoid syndrome - this is a seratonin metabolite
surface marker of monocyte macrophage lineage
CD14 - seen in TB
EBV - which is associated with mono - which you see lymphocytes with abundant pale blue cytoplasm in a sea of red cells - also is known to cause (give me 4)
hodgkin
non hodgkin
burkitt
nasopharyngeal carcinoma
ondansetron
granisetron
dolasetron
highly effective in blocking chemotherapy induced vomiting via blocking central serotonin (5HT3) receptors in the area postrema (chemoreceptor trigger zone in 4th ventricle) and Nucleus tracuts solitarius (vomiting coordination center in medulla) - also located in the presynaptic nerve terminal of the vagus nerve in the GI tract
nucleus tractus solitarius
vomiting coordiation center in medulla
ruxolitnib
JAK2 inhibitor - rx for primary myelofibrosis (tear cell - massive splenomegaly)
what is bortezomib
a boronic acid containing dipeptide
proteosome inhibitor used for rx of multiple myeloma because ineffective proteosome fx leads to apoptosis and we want to kill the plasma cell that has gone bonkers
In addition to acting as an ADH (vasopressin) analogue, what other cool thing does desmopressin do?
it increases the release of vWF from endothelial cells - which augments platelet binding and also increases factor VIII stability - (note DDAVP also raises factor VIII)
What does hydroxyurea do / what is it used for
treatment of sickle cell disease - increases hemoglobin F synthesis
5Ps of AIP
Painful abdomen Port wine pee Polyneuropathy Pysch Precipitated by drugs (cytochrome p450 inducers - phenobarbital, griseofulvin, phenytoin) alcohol, smoking, starvation
Rx iron poisoning
IV deferoxamine
