amboss 6/25 Flashcards
what are some hereditary causes of hyper coagulability
factor V leiden, protein C def, protein S def, antithrombin III def, hyperhomocysteinemia, plasminogen def, sickle cell anemia
what are some acquired causes of hypercoagulability
pregnancy, age, smoking, obesity, surgery, immobilization, trauma, malignancy, nephrotic syndrome oral contraceptive use, SLE, heparin-induced thrombocytopenia
what is the result of heparin treatment
increases the PTT; X inhibitor
what is the differential in someone that is treated with heparin, but their PTT doesnt change
this means that they are heparin resistant. Usually antithrombin III, high levels of VIII or fibrinogen
what does the PTT measure; which factors
VII, XI, IX, VIII, X V, II, I
what would you expect to find on peripheral smear from someone with sickle cell anemia
holly-jolly bodies
sickled cells
what are people with sickle cell trait at risk of developing
renal papillary necrosis
what GI disorder are people with sickle cell disease at risk for
cholelithiasis
what are the most common presentation of people with sickle cell trait
hyposthenuria (lots of urine with low osmolarity due to kidney dysfunction). painless hematuria as well. UTIs, chronic kidney disease, renal medullary carcinoma.
what is the best treatment for sickle cell
hydroxurea becuase it reduces the incidence of vasoocclusive crises
what are the mechanisms of hydroxyurea treatment for sickle cell
increases reticulocyte and fetal hemoglobin
what is the presentation of paroxysmal nocturnal hemaglobinuria
intermittent abdominal pain, jaundice, hemaglobin in the urine, intravascular hemolysis with normal blood smear and combs test because this is complement mediated, not antibody. they will have dark urine in the morning
what does paroxysmal nocturnal hemaglobinuria put the patient at highest risk for
thrombosis; this is the most common cause of death
what is the presentation of intravascular hemolysis
increased LDH, reticulocytes, hemoglobinuria, and decreased haptoglobin
What is the cause of anemia in an elderly person with a murmur in the right upper field
aortic stenosis causing shearing forces on the RBC
what is the cause of thrombocytopenia in cirrhosis
The main cause is cirrhosis causes portal hypertension and decreased venous drainage from the spleen. This causes hypersplenism and sequestration of upwards of 90% of platelets in the spleen.
also, due to liver dysfunction there is a paucity of hormones (thrombopoeitin) for the production of platelets
what is the presentation of chronic myelogenous leukemia
fatigue, splenomegaly and SEVERE leukocytosis (250,000)
what is the presentation of acute myelogenous leukemia
fatigue and splenomegaly with leukocytosis (80,000), with Auer rods (cytoplasmic inclusion needles)
what is the significance of 9:22 translocation
this is the Philadelphia chromosome BCR:ABL acute lymphocytic leukemia.
what is the first line therapy for acute myelogenous leukemia
imatinib
what is the definitive diagnostic test for leukemia
bone marrow biopsy; a blood smear is not definitive
what are Down syndrome patients at risk of developing for blood cancers
acute lymphoblastic leukemia, acute myeloid leukemia
what is the best way to prevent tumor lysis syndrome
IV hydration
presentation of pediatric HUS
This child presents with thrombocytopenia, signs of hemolysis (normocytic anemia, ↑ LDH, indirect hyperbilirubinemia, schistocytes), and renal dysfunction (hematuria, proteinuria, ↑ BUN, and ↑ creatinine), which is a triad indicative of hemolytic uremic syndrome (HUS).