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17 Cards in this Set
- Front
- Back
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generally, what kinds of platelet disorders can there be, and how are those subdivided?
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can be quantative or qualatative.
qualatative can be due to acquired or inherited. inherited - glycoprotein dysfunctino, problems with granules, or problems with signal transduction/secretion. |
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what's bernard soulier syndrome?
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BIG PLATELETS - due to problem with GP 1b-IX, platelets can't bind to busted endothelium. thromboCYTOPENIA.
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if that has big platelets, what has small platets?
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Wiscott-Aldrich has small platelets and ecsema. Sialophorin mutation.
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Acquired platelet qualatative disorders - most common! what are the big ones?
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Uremia causes stippling. Uremic platelets are completely fucked. but THINK ANTIBODIES in any patient whose platelets are above 50,000 but STILL BLEEDING.
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what do myeloproliferative disorders due to platelet number and function?
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ET, CML, P. Vera, etc can all up or drop the platelet counts - but they don't work very well! Maybe you bleed, maybe you get thrombosis.
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so, what can antibodies do to platelets?
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either mess with function (bind IIb/IIIa) or mess with number (ITP).
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Talk about ITP and TTP
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ITP patients will have LOW platelet counts due to antibody mediated destruction. Secondary to other disorders, more common in MEN, SLE, etc. Maybe remove spleens to help.
TTP - NON IMMUNE destruction of platelets! Platelets get activated, set up fibrin strands everywhere, get MAHA. symptoms always include CNS and RENAL and FEVER and THROMBOCYTOPENIA. Schistocytes!. |
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Decreased platelet production makes one think of...
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drugs - alcoholics and chemo patients get random thrombocytopenia. Also think of asplastic anemia in someone who has a preexisting blood disorder, etc.
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what % of the bone marrow has to be blasts to diagnose someone with acute leukemia (either ALL or AML?)
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20%
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howell-jolly bodies are found in what?
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splenectomy.
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what's hemoglobin H disease?
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3 mutations in the alpha gene - so SIGNIFICANT anemias. 2 mutations = trait, 1 = silent carrier.
remember that in beta thalasemia, there is INCREASED A2 that you CAN SEE on electrophoresis. |
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talk about paroxysmal nocturnal hemoglobinuria
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PIGA gene mutated, get concentration of red cells in the bladder at night.
Red cells become more sensitive to complement OVER TIME, so this is ACQUIRED and INTRINSIC hemolytic anemia. PIGA makes GPI linked proteins: CD55 = DAF CD59 makes MIRL. INTRAVASCULAR (like GP6d) |
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Peutz Jegher's - what mutation, what's wrong?
what about basal cell nevus syndrome? |
colon polyps (jegger gives you the shits). also STK11?
basal cell nevus = JAW cysts, PTCH gene. get a pitch in the face. |
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Von Hippel Lindau -
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think of hemnagioblastomas, sac tumors, kidney tummors, pheochromocytoma.
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Bone growth in kids - what's most common?
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osteoid osteoma - super common, long bones, benign. relieved by aspirin.
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where is osteosarcoma likely to occur? what about a situation makes you think about chondrosarcoma?
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distal femur (knee) = osteosarcoma.
Chondrosarcoma = someone in their 30's/40's with big ugly bone tumor. |
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high homocystine makes you think of...?
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ARTERIAL thrombosis, as opposed to vascular.
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