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antithrombin III deficiency, factor V Leiden, protein C,S deficiency, abnormal plasminogen, dysfibrogenemia
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malignancy, pregnancy, OCPs, nephrotic, immob, UC and Crohn's, myeloproliferative, DIC, TTP
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pance hematology
| Question | Answer |
|---|---|
| antithrombin III deficiency, factor V Leiden, protein C,S deficiency, abnormal plasminogen, dysfibrogenemia | congenital hypercoaguable states |
| malignancy, pregnancy, OCPs, nephrotic, immob, UC and Crohn's, myeloproliferative, DIC, TTP | acquired hypercoaguable states |
| congenital d/o's assoc'd w/thrombotic states typically are genetically | autosomal dominant |
| little spontaneous bleeding, but may hemorrhage w/surgical procedures; tx w/FFP; autosomal recessive | Factor XI def (hemophilia C) |
| x-linked recessive d/o (affects males) similar to hemophilia A but occurs less frequently | Factor IX def (christmas disease) |
| excessive bleeding following trauma or surgery; infusion of heat-treated or recombinant factor VIII concentrates; desmopressin may help; avoid ASA; PTT prolonged, red'd factor VIII; C levels (vWF NL) x-linked recessive | hemophilia A (Factor VIII deficiency) |
| mucous membrane bleeding, spontaneous hemarthrosis; prolonged bleeding time; vWF low; desmopressin acetate; ASA exacerbates; pregnancy/estrogen decreases; 6 types; autosomal dominant | vWF |
| prolonged bleeding time, skin/mucosal bleeding; nl # of platelets, but platelet fxn abnl | ASA and NSAIDs |
| generalized hemorrhage w/severe underlying systemic illness (sepsis, tissue injury) large volume plasmapheresis; prednisone and antiplatelet agents; splenectomy req'd | TTP |
| disorder similar to TTP but found primarily in children and does not include neurological sx's | hemolytic uremic syndrome |
| estrogen use, pregnancy, drugs, (quinine and ticlopidine) | may precipitate TTP |
| causes of secondary thrombocytopenia | SLE and CLL |
| what drug most commonly causes an ITP-like reaction in hospitalized pts | heparin (also sulfonamides, thiazides, cimetidine, gold) |
| d/o of bleeding that may occur at any age and is more common in women; often coexists w/other autoimmune diseases | chronic ITP |
| self-limited autoimmune (IgG) disorder found most commonly in children of both sexes and is assoc'd w/a preceding viral URI | ITP |
| abnl decr in # of platelets in blood; most common cause of abnl bleeding; impaired production, inc'd destruction, splenic sequestration or dilution | thrombocytopenia |
| GI tract, skin, bone, bone marrow (Burkitt's presents w/abd'l fullness) | extralymphatic sites for nonHodgkin's lymphoma |
| diffuse of isolated, painless, persistent lymphadenopathy; bone marrow involvement is frequent; malignancy that arise from lymphocytes (90% B); peak 20-40 | nonhodgkin's lymphoma |
| initial tx choice for hodgkin's lymphoma | radiation therapy |
| Reed-Sternberg Cells confirm diagnosis | Hodgkin and nonHodgkins |
| painless cervical, supraclavicular, and mediastinal LA; pain in affected node after ingestion of alcohol may occur; 15-45 yo; ?EBV | Hodgkin's |
| only therapy that is curative for CML | allogeneic bone marrow transplant |
| leukocytosis (>150K); philadelphia chromosome | CML |
| leukocytosis (>20K), smudge cells, Richter's syndrome; survival time 6 years; clonal malig of B lymphs; most prevalent | CLL |
| isolated node transforms into aggressive large-cell lymphoma | Richter's syndrome |
| mediastinal mass on CXR, terminal deoxynucleotidyl transferase, presenceof Philadelphia chromosome unfavorable; pancytopenia with 20% blasts | ALL |
| auer rods; pancytopenia with 20%blasts | AML |
| avoid oxidative drugs; hemolytic episodes are self-limited as RBC's are replaced; Heinz bodies, retics, indirect bili increase; episodic hemolysis usually healthy and w/o splenomegaly; x-linked recessive | G6PD deficiency |
| dapsone, quinidine, nitrofurantoin, sulfonamides | drugs that cause G6PD deficiency |
| Howell-Jolly bodies, elev retic, nucleated RBC's; cholelithiasis, splenomegaly, poorly healing ulcers, infxn w/encapsulated organisms, strokes, priopism; AVN of hip; aplastic crisis; autosomal recessive | sickle cell |
| dehydration, acidosis, hypoxema causes | sickling to increase |
| glossitis, stocking-glove paresthesias,, loss of position, fine touch and vibratory sensation, clumsiness, dementia and ataxia | B12 deficiency |
| macrocytic anemia with Howell-Jolly bodies; sore tongue, vague GI, no neuro sx | folate deficiency |
| acqured d/o w/red'd Hgb synthesis causing iron accumulation, especially in mitochondria; myelodysplasias, chronic alcoholism, lead poisoning are causes of | sideroblastic anemia |
| transufion to keep Hgb conc'ns at least 12; poss bone marrow transplant and iron chelation or splenectomy (avoid Fe); basophilic stippling | B-thalassemia |
| folic acid supplements and AVOID Fe and oxidative drugs (dapsone, quinidine, sulfonamides) | A-thalassemia |
| brittle nails, cheliosis, smooth tongue, esophageal webs (Plummer-Vinson syndrome); pica | Fe def |
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