Heme/Onc
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86 terms
Terms | Definitions |
|---|---|
 How long do RBCs live? | 120 days |
| What does Poikilocytosis mean? | Varying shapes of RBCs |
| What is contained in the dense granule of a platelet? | ADP calcium |
| What is contained in the alpha-granule of a platelet? | vWF, fibrinogen |
| What is the receptor for fibrinogen on a platelet? | GpIIb/IIIa |
| What is the receptor for vWF? | Gp1b |
| Petechiae are a sign for? | Thrombocytopenia |
| How long do platelets last? | 8-10days |
| In infection, an increase in WBCs with bands (left shift) suggests what TYPE of infection? | extracellular bacteria |
| In infection, a granulocytosis suggests what TYPE of infection? | Fungus |
| In infection, a monocytosis suggests what TYPE of infection? | intracellular bacteria |
| In infection, an eosinophilia suggests what TYPE of infection? | Parasite |
| Monocytosis AND granulocytosis suggest what etiology of infection? | Viral |
| In what conditions do you see hypersegmetented polys? | vitamin B12 and folate deficiency |
| What enzyme is responsible for the green pus of neutrophils? | Myeloperoxidase |
| What is the role of lactoferrin? | binds iron and inhibits growth of phagocytosed bacteria and some fungi |
| Which blood cell has a kidney bean shaped nucleus? | monocyte, only in the blood, in the tissue they're macrophages |
| What cytokine is responsible for differentiation of monocytes? | gamma-interferon |
| What is the cell surface marker for macrophages? | CD14, CD40, also has MHC II and B7, Fc and C3b receptors which when activated enhance phagocytosis |
| What cell surface receptor of macrophages binds LPS? | CD14 |
| What are the contents of an eosinophilic granule? | major basic protein, histaminase and arylsulfatase to limit the reaction |
| What are the contents of a basophilic granule? | histamine, heparin, vasoactive amines, leukotrienes (LTD4) |
| What are the contents of a mast cell granule? | histamine, heparin, eosinophil chemotactic factors. |
| Cell with metachromasia on giemsa stain and degranulation via cross linking of IgE? | Mast cell |
| Uncontrolled proliferation of mast cells causing itching, flushing, abdominal cramps, and PUD? | Systemic mastocytosis: PUD is from increased gastric acid from excessive histamine release |
| MOA of cromolyn sodium? | Prevents degranulation (mast cell membrane stabilizer) |
| What is the cell marker for suppressor T cells? | CD28 |
| Can IgM cross the placenta? | No |
| Can IgG cross the placenta? | Yes |
| How do you preventerythroblastosis fetalis? | Rh antigen on RBC surface of fetus of mother who is Rh-, mother is exposed, makes anti-Rh IgG. THE NEXT PREGNANCY IS AT RISK. We treat using Rho(D) immune globulin (Rhogam) during first pregnancy to bind any spare Rh that might sensitize mom. |
| A deficiency of factor VIII gives you? | Hemophilia A |
| A deficiency of factor IX gives you? | Hemophilia B |
| Vitamin K is responsible for the gamma carboxylation of what factors? | 2, 7, 9, 10, C, S |
| For acute blood loss and severe anemia, what type of blood transfusion do we use? | Packed RBCs, these increase Hb and O2 carrying capacity |
| To stop significant bleeding (thrombocytopenia, qualitative platelet defects) what type of blood transfusion do we use? | Platelets, these will increase the platelet count, usually given in a 6 pack of units or more for therapeutic effect |
| How do we treat DIC, cirrhosis, and warfarin-over-anticoagulation? | Fresh frozen plasma, which will increase coagulation factor levels by about 20% |
| How do we treat coagulation factor deficiencies involving fibrinogen and factor VIII? | Cryoprecipitate which contains fibrinogen, factor VIII, and factor XIII |
| Patient who was recently transfused has tingling in his fingers. What went wrong? | Citrate pretreatment of his transfusion binds calcium, hypocalemia |
| Lysis of RBCs in an old blood unit causes what electrolyte discrepancy? | Hyperkalemia: presents with peaked T waves, wide QRS and arrhythmias |
| Aplastic anemia is associated with what antibiotic? | Chloramphenicol |
| What is the MOA of Chloramphenicol? | Inhibits ribosomyl peptidyl transferase and blocks peptide bond formation at 50S |
| What do we use Chloramphenicol for? | Menigitis (H.flu, N. menigidis, S. pneumo) but not really because of it's side effects. Just keep in mind if patient with penicillin allergy comes in with aplastic anemia |
| Is Chloramphenicol induced aplastic anemia dose independent or dependent? | dose independent |
| Why do babies get grey baby syndrome? | Administration of chloramphenicol, they don't have UDP-glucuronyl transferase |
| Patient with fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae after exposure to benzene? | Aplastic anemia: failure of myeloid stem cells |
| Causes of aplastic anemia? | radiation and drugs (benzene, chloramphenicol, alkylating agents, antimetabolites) Viral agents (parvoB19, EBV, HIV, HCV) Fanconi's anemia (DNA repair defect) Idiopathic (immune mediated, primary stem cell defect) can follow hepatitis |
| How do you treat aplastic anemia? | Remove any offending exposure, allogenic bone marrow transplantation, RBC and platelet transfusions, G-CSF or GM-CSF:: complete recovery in less than 10% of all cases |
| MOA of methotrexate? | folic acid analog that inhibits dihydrofolate reductase |
| MOA of 5-FU? | Pyrimidine analog -> complexes w/ folic acid --> inhibits thymidylate synthase and decreases dTMP |
| MOA of 6-MP? | Purine analog --> decreases purine synthesis, but must be activated by HGPRTase |
| MOA of 6-TG? | Purine analog, but this one you can give with allopurinol as it is not metabolized by xanthine oxidase (6-MP) |
| MOA of Cytarabine? | Pyrimidine analog --> inhibits DNA polymerase |
| Which anticoagulant is associated with neutropenia? | Ticlopidine |
| What do you give for severe warfarin toxicity? | Fresh frozen plasma (if less severe, vitamin K supplement will suffice) |
| What drug do you use for long term prophylaxis against pulmonary embolism? | Warfarin |
| How do you treat von Willebrand's disease? | DDAVP (desmopressin) releases vWF stored in endothelium |
| PT monitors which pathway? | Extrinsic |
| PTT monitors which pathway? | Intrinsic |
| What conditions are caused by EBV? | Monospot positive mono, Hodgkin's lymphoma, Burkitts lymphoma, nasopharyngeal carcinoma, hairy leukoplakia, Lymphoepithelioma |
| What monoclonal antibody binds CD20? | Rituxumab |
| Most common cause of sepsis in sickle cell patients? | S. pnumoniae (followed by H. flu) |
| What is the triad of wiscott aldrich syndrome? | ezcema, recurrent infections and thrombocytopenia |
| What is the pathogenesis of wiscott aldrich syndrome? | XR- progressive deletion of B and T cells resulting in increased IgE, IgA, and decreased IgM (defective response to polysaccaride antigens) |
| What is the translocation in Burkitt's lymphoma? | t(8:14) moves the c-myc gene next to the heavy chain |
| What is the translocation in mantle cell lymphoma? | t(14:18) moves the bcl-2 gene closer the the heavy chain. bcl-2 inhibits apoptosis --> superduper dividing machine! |
| Describe the framework for Burkitt's lymphoma? | P: Adolescents or young adults, starry sky appearance, sheets of lymhocytes with interspersed macrophages, Jaw lesion in africa, abdomen/pelvic mass in sporadic form. M: t(14:18) of the c-myc gene moves to the heavy Ig chain, associated with EBV C: Leukemic phase rare, prognosis is rare, abdominal lesions can cause bowel obstruction, jaw lesions: loosening of teeth Dx: PCR/Fish for c-myc, bone marrow biopsy, B markers (IgM) Tx:CDOX regimen, rituxumab (monclonal against CD20) |
| Describe the framework for Diffuse large B-cell lymphoma? | P: Older males |
| Describe the framework for Follicular lymphoma? | P: Adults with painless adenopathy, waxing and waning in size (otherwise asymptomatic), associated with immunodeficiency, scanty cytoplasm with twisted nuclei that resemble germinal centers M: t(14:18) bcl-2 translocation which is an anti-apoptotic gene, which when translocated next to the heavy chain, causes overexpression in B-cells and immortal cells C: Difficult to cure, indolent course, 8-10 year survival waxing and waning Dx: Lymph node biopsy, usually express Bcl-2 Tx:Radiation |
| Infant born with extensive extramedullary hematopoeisis, splenomegally, severe anemia, kernicterus. Dx? | Erythroblastosis fetalis from maternal type II sensitization to fetal Rh +, IgG can cross the placenta and destroy fetal RBCs |
| Ganciclovir and zidovudine increase the risk of what side effect? | Neutropenia |
| What do you administer for a DVT? | Warfarin is first line |
| What inhibits the Pt and the PTT but not the TT? | Direct factor Xa inhibitors: idraparinux, rivaroxaban, apixaban |
| Mutation to what receptor renders an individual resistant to HIV infection? | Homozygous mutation of CCR5 will make an individual resistant, heterzygotes have a slower course |
| Explain the binding of HIV to cells. | gp120 identifies the host T cell and binds CD4 and CCR5, gp 41 mediates fusion and entry. |
| How do you test for HIV? | Begin with ELISA (sensitive but has a high false positive rate), then confirm with western blot (specific, but has a high falses negative rate). We use PCR to monitor viral load. |
| Why might an HIV negative infant born to an HIV positive mother present with a positive ELISA? | gp120 can cross the placenta |
| What do you give an HIV positive mother to prevent transmission? | Zidovudine (Nucleotide reverse transcriptase inhibitor, causes megaloblastic anemia |
| Thymomas are associated with what conditions? | Myasthenia Gravis, pure red cell aplasia, Lambert-Eaton Syndrome |
| What conditions have schistocytes? | DIS, TTP-HUS, traumatic hemolysis |
| What conditions have spur cells (acanthocytes)? | Liver disease, abetalipoproteinemia |
| Which conditions have basophilic stippling? | Thalassemias, anemia of chronic disease, iron deficiency, lead poisoning |
| What condition has bite cells? | G6PD |
| What two conditions have spherocytes? | Spherocytosis and autoimmune hemolytic disease |
| What conditions have target cells? | HbC disease, Asplenia, liver disease, thalassemia |
| Which coagulation factor has the shortest half-life? | Factor 7, expect to see in alcoholics with liver failure since it's the first to go out. Easy though, since all the other ones don't impact the pT. |
| What do you see on peripheral smear for DIC? | Schistocytes: also seen in TTP.HUS, and traumatic hemolysis (heart valves) |
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