Heme/Onc Flashcards
Dense granules vs alpha granules if platelets ?
dense = ADP, calcium; alpha = vWF, fibrinogen
Decreased FC receptor in immature neutrophils?
Decreased CD16
Eosinophils produce?
Histaminase and arylsulfatase (helps limit reaction following mast cell degranulation).
Kallikrein stimulates ?
HMWK to Bradykinin
activated protein C (w/ S) cleaves and inactivates ?
favctors Va, Vllla
antithrombin inhibits activated forms of factors ?
12, 11, 7, 9, 10, 2
“activation” phase of platelet plug formation?
ADP binding to R induces Gpllb/llla expression at platelet surface
dec ESR?
Microcytosis, hypofibrinogenemia
Acanthocytes (spur cells) seen in ?
Liver disease, abetalipoproteinemia (states of cholesterol dysregulation)
Howell-Jolly bodies seen in ?
pts mothball ingestion (naphthalene)
ferroportin ?
Basolateral iron transporter of enterocyte to blood
alpha thalassemia - Hb Barts vs HbH ?
gamma4 (4 gene deletion) //
beta3 (3 gene deletion)
beta thalassema - mild anemia mech ?
Severe anemia mech ?
DNA splicing defect //
stop codon
dx of beta-thalassemia minor confirmed by?
inc HbA2 (> 3.5%) ; dec HbA
tx of Beta-thalassemia major?
long term blood transfusion
Sideroblastic anemia causes changes in lvls ?
inc iron, normal TIBC, inc ferritin
hemosiderin ?
iron carried by transferrin in circulation –> binds to apoferritin in cells
tx of orotic aciduria?
Uridine monophosphate
nonmegaloblastic macrocytic anemia?
liver disease; alcoholism; reticulocytosis; drugs (5-FU, AZT, hydroxyurea) //
macrocytes are round not oval and hypersegmental neutros not present
Intravascular hemolysis causes changes in ?
dec haptoglobin, inc LDH
Extravascular hemolysis causes changes in ?
inc UCB, inc LDH
inc calcium bilirubinate gallstones (black) in which intrinsic hemolytic normocytic anema (extravasc) ?
hereditary spherocytosis
paroxysmal nocturnal hemoglobinuria triad, lab findings, tx ?
Hemolytic anemia, pancytopenia, and venous thrombosis //
CD55/59 neg RBCs //
tx eculizumab.
Most common cause of death in sickle cell anemia?
Strep pneumo sepsis
Sickle cell anemia affects kidney how ?
Renal papillary necrosis (due to low O2 in papilla)
transferrin production affected by pregnancy and oral contraceptives?
inc in pregnancy and by oral contraceptives
tx for acute intermittent porphyria?
Glucose and heme, which inhibit ALA synthase
Thrombotic thrombocytopenic purpura (TTP) ?
def of ADAMTS 13 (vWF cleaving metalloprotease)// inc LDH// pentad of neuro and renal sx, fever, thrombocytopenia, and micrangiopathic hemolytic anemia
Acute idiopathic thrombocytopenic purpura (ITP)?
Most common cause of thrombocytopenia in children; Abs made in spleen
vWF acts to carry/protect?
factor VIII
Ristocetin cofactor assays?
evaluate vWF fxn (abnormal in Vw disease and BS disease)
Serum is missing?
factors 5,8; prothrombin 2; fibrinogen
Prothrombin gene mutation?
Mutation in 3’ untranslated region
Protein C or S def leads to inc risk of?
Thrombotic skin necrosis w/ hemorrhage following administration of warfarin
use of cryoprecipitate?
Treat coagulation factor def involving fibrinogen and factor VIII
Blood transfusion risks?
Hypocalcemia (citrate is a calcium chelator) and hyperkalemia
Leukemoid rxn ?
Perforated appendicitis (neutros); pertussis (lymphos); and inc leukocyte alkaline phosphatase
Origin of Reed Sternberg cells?; secrete ?
CD30+ and CD15+ B-cell origin; RS cells secrete cytokines
Translocation of what in mantle cell lymphoma?; how is the prognosis?
translocation of cyclin D1 (11); poor prognosis (CD5+)
Most common adult NLH?
Diffuse large B-cell lymphoma
mycosis fungoides/ Sezary syndrome?
Cells spread to invade blood with sezary cells (lymphocytes w/ cerebriform nuclei - lobular!) //
present w/ Cutaneous patches/nodules; CD4+
tx of hairy cell leukemia?
Cladribine (an adenosine deaminase inhibitor)
CML ?
inc basophils; may accelerate and transform to AML or ALL (“blast crisis”); very low leukocyte alkaline phosphatase
Langerhans cell histiocytosis?
Cells express S-100 (neural crest origin) and CD1a. // Birbeck granules
Myelofibrosis?
Ineffective erythropoeisis, EMH
LMW heparin examples?; what do they act on?
enoxaparin and dalteparin; they act more on factor Xa
Heparin-induced thrombocytopenia (HIT)?
dvlpmnt of IgG Abs against heparin bound to platelet factor 4 (PF4)//
complex activates platelets
hep vs warfarin ? (structure, inhibits coag in vitro?)
Heparin: Large anionic, acidic polymer ; yes inhibits coag in vitro
Warfarin: Small lipid-soluble molecule
Toxicity of thrombolytics?
Causes bleeding
ADP R inhibitors ? toxicity ?
Clopidogrel, ticlopidine, prasugrel, ticagrelor // neutropenia, fever, and mouth ulcers
Cilostazol and dipyridamole?
Phosphodiesterase III inhibitor//
inc cAMP in platelets, thus inhibiting platelet aggregaton; also vasodilators
Mechanism of Cytarabine (arabinofuranosyl cytidine) ?
Acts as a pyrimidine analog
Toxicity of methotrexate (an antimetabolite)?
Macrovesicular fatty change in liver
Dexrazoxane ?
Dexrazoxane used to prevent cardiotoxicity from doxorubicin
Cyclophosphamide, ifosamide mech ?
Covalently X-link DNA at guanine N-7. Toxicity from acrolein is prevented with mesna.
nitrosoureas mech and use ?
Require bioactivation; alkylating agent
Clinical use: Brain tumors
Procarbazine ?
alkylating agent; MAOI
amifostine (free radical scavenger) and chloride diuresis to prevent nephrotox from ?
cisplatin, carboplatin
Vemurafenib mech ?
Small molecule inhibitor of forms of B-Raf kinase w/ V600E mutation
Bevacizumab mech ?
Monoclonal antibody against VEGF
Abciximab, eptifibatide, tirofiban?
GP2b/3a inhibs