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HY Heme Part I
Day 15.3
| Question | Answer |
|---|---|
| What allows RBCs to change shape as they pass through vessels? | Spectrin (biconcavity and flexibility) |
| What are some of the different causes of polycythemia? | Polycythemia vera, renal cell CA, pheochromocytoma, hemangioblastoma, chronic hypoxia (COPD, Obstructive Sleep Apnea/OSA), Down syndrome (at birth) |
| What coagulation factor is deficient in hemophilia A? | Factor 8 (Ate) |
| What coagulation factor is deficient in hemophilia B? | Factor 9 (B9) |
| What clotting factors require vitamin K for synthesis? | Protein C and S, and factors 2, 7, 9, and 10 |
| What are the treatment for overdose of heparin? | Protamine sulfate |
| What is the treatment for overdose of warfarin? | Vit K and fresh frozen plasma |
| What lab value is used to monitor the following medications: heparin? | PTT |
| What lab value is used to monitor the following medications: warfarin? | PT/INR (INR should be around 2-3) |
| What lab value is used to monitor the following medications: enoxaparin? | Factor Xa activity |
| What is the treatment for heparin induced thrombocytopenia (HIT)? | Switch to a hirudin derivative like lepirudin or bivalirudin, or argatroban. These drugs directly inhibit thrombin and are a good alternative to heparin for anticoagulating patients with HIT. |
| What are the 3 steps of platelet stimulation? | 1. Adhesion 2. Activation 3. Aggregation of platelets via GpIIb/IIIa |
| Which factor complexes with and stabilizes factor VIII and is responsible for platelet adhesion to the vessel wall and to other platelets? | Von Willebrand's Factor |
| What pathologic form of RBC would you see in the following diseases?: lead poisoning | basophilic stippling |
| What pathologic form of RBC would you see in the following diseases?: G6PD deficiency | bite cells and Heinz bodies (Heinze ketchup is yummy so macrophages want to take a big bite out of it!) |
| What pathologic form of RBC would you see in the following diseases?: DIC | schistocytes/helmet cells |
| What pathologic form of RBC would you see in the following diseases?: abetalipoproteinemia | spur cells/acanthocytes |
| What pathologic form of RBC would you see in the following diseases?: asplenia | Howell-Jolly bodies |
| Iron-protein complex that acts as a cellular storage protein for iron and is an acute phase reactant | Ferritin |
| Protein that binds ferric molecules and transports them through the plasma. | Transferrin |
| Where is transferrin synthesized and what happens to it in iron deficiency? | It is synthesized in the liver. Levels increase with iron deficiency. |
| What are the 3 actions of thromboxane A2 (Goljan)? | Potent vasoconstriction (keeps clot from washing away), platelet aggregation (par excellence!), and bronchoconstriction (can cause problems in asthmatics) |
| What converts fibrinogen to fibrin? Is this pro-coagulation or anti-coagulation? | Thrombin; pro-coagulation; coagulase can also convert fibrinogen to fibrin |
| What amino acid change is responsible for sickle cell disease? | Glu--> Val in beta chain |
| What is the amino acid change responsible for hemoglobin C disease? | Glu-->Lys in beta chain |
| What is the difference between normal adult hemoglobin and normal fetal hemoglobin? | Adult has 2 alpha and 2 beta chains. Fetal replaces the 2 beta chains with 2 gamma chains (2 alpha, 2 gamma) |
| What hemoglobin modification is seen with poorly controlled diabetes (be specific!)? | Normal Hbg gets non-enzymatically glycosylated (glucose gets attached to it), creating Hb A1c (Goljan said this was important to know!) |
| What diseases occur when there are no alpha chains in hemoglobin? | Severe alpha thalassemia: Hb Bart (4 gamma chains)--> hydrops fetalis, Hb H (4 beta chains) |
| What is the cause of anemia given the following statement?: microcytic anemia + dysphagia + glossitis | Plummer Vinson syndrome (Fe deficiency anemia) |
| What is the cause of anemia given the following statement?: microcytic anemia + >3.5% HbA2 | Beta thalassemia minor |
| What is the cause of anemia given the following statement?: megaloblastic anemia not correctable by B12 or folate | orotic aciduria |
| What is the cause of anemia given the following statement?: megaloblastic anemia along with peripheral neuropathy | B12 deficiency |
| What is the cause of anemia given the following statement?: microcytic anemia + basophilic stippling | lead poisoning |
| What is the cause of anemia given the following statement?: microcytic anemia reversible with B6 | sideroblastic anemia |
| What is the cause of anemia given the following statement?: HIV positive patient with macrocytic anemia | zidovudine |
| What is the cause of anemia given the following statement?: normocytic anemia + red urine in the morning | paroxysmal noctural hemoglobinuria (PNH) |
| What is the cause of anemia given the following statement?: normocytic anemia and elevated creatinine | chronic kidney disease (low erythropoietin levels) |
| What test am I? Prepared antibodies are added to a pt's washed RBC to detect the presence of immunoglobulins already present on the RBC (using an Ab to detect an Ab) | Direct Coomb's Test (DAT) |
| Name 3 situations in which a direct Coomb's test might be +. | 1. Hemolytic disease of the newborn 2. Drug induced autoimmune hemolytic anemia 3. Hemolytic transfusion rxns |
| What test am I? Pt's serum is incubated with normal RBCs to detect for the presence of Abs. | Indirect Coomb's test |
| Name 2 situations in which an indirect Coomb's test might be used. | 1. Used to test blood prior to an infusion. 2. Screening for maternal antibodies to a fetus' blood |
| Antibodies against RBCs that interact more strongly at low temps (4C) than at body temp | Cold agglutinins |
| Which immunoglobulin class is usually seen with cold agglutinins? | IgM |
| Name 3 conditions you would see cold agglutinins in. | 1. Mycoplasma pneumoniae 2. EBV 3. Some malignancies |
| Antibodies that react against RBC protein antigens at body temperature. | Warm agglutinins |
| Warm agglutinins are usually which immunoglobulin class? | IgG |
| Name 4 conditions you would see warm agglutinins in. | 1. Viruses (EBV, also HIV) 2. SLE 3. Malignancies (CLL, non-Hod lymphoma) 4. Congenital immune abnormalities |
| What is the RLS in heme synthesis? | Delta-aminolevulonic acid synthase |
| What is the cause of ITP (idiopathic thrombocytopenic purpura)? | Anti-GpIIb/IIIa antibodies that cause autoimmune destruction of peripheral platelets |
| What is the defect in Bernard Soulier disease? | Can't form platelet plug due to decreased GpIb (defect in platelet-to-collagen adhesion) |
| What is the MC inheritied bleeding disorder? | von Willebrand's disease |
| What are some of the hereditary syndromes of thrombosis (4)? | 1. Factor V Leiden mutation (MCC) 2. Prothrombin gene mutation 3. Antithrombin 3 (ATIII) deficiency 4. Protein C or S deficiency |
| What disease am I used to test for?: Ham's test | PNH diagnosis |
| What disease am I used to test for?: DEB test | Fanconi's anemia diagnosis |
| What disease am I used to test for?: Heinz bodies | G6PD deficiency |
| What disease am I used to test for?: D dimers | DIC (D dimer= fibrin degradation products) |
| What disease am I used to test for?: basophilic stippling | Thalassemia, anemia of chronic disease, Fe deficiency anemia, and lead poisoning (key one to know) |
| What disease am I used to test for?: osmotic fragility test | Hereditary spherocytosis |
| What disease am I used to test for?: + risocetin test | von Willebrand's disease |
| What rapid onset, rapidly progressive disease presents with over 50% myeloblasts in the bone marrow, numerous immature blast cells, and is associated with pancytopenia (anemia, bleeding tendency, and infection)? | AML |
| What rapid onset, rapidly progressive disease presents with over 50% lymphoblasts in the bone marrow, numerous immature blast cells, and is associated with pancytopenia (anemia, bleeding tendency, and infection)? | ALL |
| Which acute leukemia is MC in children and young adults? | ALL |
| Which acute leukemia has a median age of onset of 50yo? | AML |
| Which acute leukemia commonly has bone pain? | ALL |
| Which acute leukemia is associated with the Tdt enzyme? | ALL |
| Which acute leukemia is PAS+? | ALL |
| What is the prognosis of ALL? | Very good |
| Which acute leukemia is CD13, CD33 +? | AML |
| Which acute leukemia has Auer rods? | AML |
| Which acute leukemia is PAS -? | AML |
| Name 3 medical interventions a/w AML. | 1. Radiation 2. Benzene 3. Alkylating agents (e.g., Hodgkin's tx) |
| Name 3 syndromes that are risk factors for AML. | 1. Down syndrome 2. Fanconi syndrome 3. Bloom syndrome |
| Myeloproliferative disease, myelodysplastic syndrome, and aplastic anemia have what cancer risk factor in common? | AML |
| Which cancer can be treated with all-trans retinoic acid? What is the assoc'd translocation? | M3 type of AML; t(15;17) |
| What cancer am I?: t(9;22) | CML (bcr-abl) |
| What cancer am I?: t(8;14) | Burkitt lymphoma (c-myc) |
| What cancer am I?: t(11;22) | Ewing sarcoma |
| What cancer am I?: t(11;14) | Manle cell lymphoma |
| What cancer am I?: t(14;18) | Follicular lymphoma (bcl-2 activation) |
| Proliferative disorder of dendritic cells. Birbeck granules on EM are characteristic; S100 and CD1a +. | Langerhans cell histiocytosis |
Created by:
sarah3148
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