HemOnc Flashcards
Describe presentation, lab findings, pathphis of Paroxysmal Noc Hemaglobinuria….
Sudden onset abdominal pain, ascites, signs of hemolytic anemia, hypercoagulation. Labs show pancytopenia. Flow cytometry shows CD55 and CD59 deficiency.
Inflammatory triggers, more concentrated appearing urine in morning.
Mutation in PIGA gene, defect in GPI anchor, a gylcolipid involved in surface proteins CD55 and CD59, which INACTIVATE complement. Hence, RBCs more susceptible to hemolyis via compliment. Pts also more likely to develop thrombotic complications (ie Budd Chiari Formation)
Steps involved in Leukocyte Accumulation…
Margination, Rolling (selectins), Activation, Adhesion/Crawling (integrins, ICAM), Transmigration (PECAM1)
Defects in these lead to leukocyte adhesion disorders
What nerve passes thru the POSTERIOR TRIANGLE of neck, what does it innervate, what deficits would you see?
CN XI, Innervates SCM and Trapezius(elevates scapula, stabilizes shoulder), Deficit would show DROPPING of SHOULDER, and difficulty ABDUCTING above horizontal.
Fluctuations in parkinsons drug therapy (ie LEVO), caused by…?
possible nigrostriatal degeneration, as disease progresses, causes UNPREDICTABLE FLUCTUATION IN DRUG EFFICACY.
human MDR1 gene encodes for what?
multidrug resistant gene, encodes for a p-glycoprotein, ATP-dependent efflux pump
Other systemic effects of Inhaled Aenesthetics…?
- Decrease cardiac activity, decrease CO, increase artial/ventric pressures, HYPOtension
- Respratory Depression (excpet Nitrous oxide), decrease TV and Minute Vent
- Decrease vascular resistance in BRAIN, INCREASE blood flow to brain, INCREASE intracranial pressure
- Deacrease GFR, Increase renal vasc resistance
Presentation, Pathphys of TTP…..
Thrombotic Thrombocytopenic Purpura = New onset neuro symptoms, Schistocytes, Thrombocytopenia, Acuet Kidney Injury.
Defect in ADAMTS13 which is a VWF cleaving protease. L:eads to microvascular thrombosis
How does Anti-Rh(D) immunoglobulin work? When/why is given to Rh (-) pregnant women?
It is IgG to Rh + erythrocytes. Given to Rh (-) preg woman at 28 weeks gestation and immediately postpartum.
It binds the Rh+ erythrocytes from baby, and blocks exposure to moms immune sys, so that she does not form her own Abs. Prevents hemolytic disease of newborn in subsequent pregs.
Which hypothalamic nucleus mediates saitiety? Huger?
Ventromedial (satiety)
Lateral (hunger)
Rasburicase?
Drug given in conjunciton with therpaies that cause massive cell lysis (ie cancer drugs).
Converts uric acid to more soluble compound (Allantoin)
Drugs for Malaria..?
CHLOROQUINE.
If resistant to Chloroquine (African species), use MEFLOQUINE.
Which nerve root traverse the L5-S1 foramen?
L5 -
Nerve roots in lumbosacral region exit BELOW the corresponding vertebral bodies.
Disc herniation at level of L5-S1 would effect which nerve?
S1
Describe Acute intermittent porphyria ..?
AD defect in Porphobilinigen Deaminase, elevated PBG and ALA, ABD PAIN/NEUROPATHY/PYSCHOTIC FEATURES, NO photosensitivity
Splenectomy in a pt with hereditary spherocytosis will likely PREVENT what in the future?
Gallstones
