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Step 1 11.29.12
Hematology IX
| Question | Answer |
|---|---|
| What is the mechanism of leukemias? | unregulated growth of leukocytes in BM--> incr or decr circulating leukocytes and marrow failure-->anemia, infections, hemmorhage. can see infiltrates in liver, spleen, LN |
| What are 5 types of lymphoid neoplasms? | acute lympohblastic leukemia, AL lymphoma, small lymphocytic lymphoma (SLL), chronic lymphocytic leukemia (CLL), hairy cell leukemia |
| What is the onse and and presentation of Acute lymphoblastic leukemia/lymphoma (ALL)? | < 15 y/o. may present with bone marrow invovlement in childhood and mediastinal mass in adolescent males |
| What happens to the BM in ALL and what biomarkers are useful? | BM replaced by highly increased lymphoblasts. TdT+ (marker of pre-T and pre-B), CALLA+ |
| What is the prognosis for ALL and what mutation gives a better prognosis? | most respons. t(12;21) = better prognosis |
| What is the danger spread of Acute lymphoblastic leukemia/lymphoma (ALL)? | CNS and testes |
| When and how does Small lymphocytic lymphoma (ALL)/chronic lymphocytic leukemia (CLL) present? | age > 60 y/o. often Asx. warm Ab autoimmune hemolytic anemia |
| What is seen in PBS of Small lymphocytic lymphoma (ALL)/chronic lymphocytic leukemia (CLL) ? | smudge cells |
| What is the difference between Small lymphocytic lymphoma (ALL) and chronic lymphocytic leukemia (CLL) ? | CLL has peripheral blood plymphocytosis |
| When and how does hairy cell leukemia present? | Adults. mature B cell tumor in the elderly |
| What is seen on PBS in hairy cell leukemia? | filametous, hairlike projections |
| What is a key staining in hairy cell leukemia? | stains TRAP (tartrate-resistant acid phosphatase) positive |
| What are 2 myeloid neoplasms?. | acute myelogenous leukemia (AML), and Chronic myelongenous leukemia (CML) |
| What is the age and onset and what is seen in PBS in acute myelogenous leukemia (AML)? | media onset at 60 y/o. auer rods and incr circulating myeloblasts on peripheral smear |
| What is significant about the t(15;17)---> M3 subtype of acute myelogenous leukemia (AML)? | responds to all-trans retinoic acid (vit A) inducing differentiation of myeloblasts |
| What is a common presentation of acute myelogenous leukemia (AML)? | disseminated intravenous coagulation (DIC) |
| What is the age of onset and what is a defining characteristic of Chronic myelongenous leukemia (CML)? | Age 30-60 years. Defined by Philadelphia Chromosome (t 9;22, bcr-abl). myeloid stem cell proliferation |
| What is the philadelphia chromosome and what is it associated with? | t9;22, bcr-abl. associated with Chronic myelongenous leukemia (CML) |
| How does chronic myelongenous leukemia (CML) present and what can it progress to? | incr neutrophils, metamyelocytes, basophils; splenomegaly. can accelerate and tranform to AML or ALL |
| What is an importnant enzymatic characterisitc ofr chronic myelongenous leukemia (CML)? | low leukocyte alkaline phosphatase due to immature granulocytes |
| What is the tx for chronic myelongenous leukemia (CML) and what is the mech of the drug? | give imantinib ( small molecule inhibitor of bcr-abl tyrosine kinase) |
| What are Auer rods, when are they seen? | peroxidase positive cytoplasmic inclusion in granulocytes and myeloblasts. commonly seen in promyelocytic leukenia M3 (AML) |
| What is a danger of treating AML M3? | can cause release of auer rods which leads to disseminated intrvascular coagulation (DIC) |
| What is t 8;14 and what is it associated with? | c myc activation in Burkitt's lymphoma |
| What is t (14;18) and what is it associated with? | bcl-2 activation in Follicular lymphomas |
| What is t(15;17) associated with and does it mean? | M3 type of AML mean its responsive to all-trans retinoic acid |
| What is t(11;22) associated with? | Ewing's sarcoma |
| What is t (11;14) associated with? | Mantle cell lymphoma |
| What is the pathogenesis of Langerhans cell histiocytosis (LCH)? | proliferative disorder of dendritic cells from monocyte lineage. cell are functionally immature and cant stimulate T lymphocytes via Ag presentation |
| What are some key biomarkers and characterisitcs in Langerhans cell histiocytosis (LCH)? | S-100 and CD1a expressed. see birbeck granules (tennis rackets) on EM |
| What are the levels of RBCs, WBCs, platelets seen in polycythemia vera? | incr RBCs, incr WBCs, incr platelets |
| Does polycythemia vera have Philadelphia chromosomes or JAK2 mutations? | YES to JAK2, not to philadelphia |
| What are the RBCs, WBCs and platelet levels seen in essential thrombocytosis? | normal RBCs, normal WBCs, incr platelets |
| Does essential thrombocytosis have philadelphia chromosome or JAK2 mutations? | 30-50% hav JAK2, no philadelphia |
| What RBC, WBC, and platelet leves are seen in myelofibrosis? | decr RBCs, variable WBCs, variable platelets |
| Does myelofibrosis have JAK2 mutation of philadelphia chromisome? | 30-50% have JAK2, none have philadelphia chromosome |
| What are the RBC, WBC, and platelet levels seen in CML? | decr RBC, incr WBC, incr platelets |
| Does CML have a JAK2 mutation or philadelphia chrmosome? | no JAK2, yes to philadelphia chromosome |
| What are the classic findings in polycythemia vera? | abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors |
| What are the classic findings in essential thrombocytosis? | similar to polycythemia vera, but specfic for megakaryocytes |
| What are the classic findings in myelofibrosis? | fibrotic obliteration of bone marrow. tear drop cell. "Bone marrow is crying" |
| What are the classic findings in CML? | bcr-abl tr4ansformation leads to incr cell division and apoptosis. DO NOT see JAK2 mutations |
| What plasma volume, RBC mass and O2 sat is seen in relative polycythemia? | decr plasma volume, no change in RBCs, no O2 sat |
| What plasma volume, RBC mass and O2 sat is seen in appropriate absolute polycythemia? | no change in plasma volume, incr in RBC mass, decr O2 sat |
| What disease is appropriate absolute polycythemia seen in? | lung disease, CHD, high altidtude |
| What plasma volume, RBC mass and O2 sat is seen in inappropriate absolute polycythemia? | no change in plasma volume, incr RBC mass, no change in O2 sat |
| What diseases is inappropriate absolute polycythemia? What is it due to? | RCC, Wilm's tumor, cyst, HCC, hydronephrosis, due to ectopic eryhtropoetin |
| What plasma volume, RBC mass and O2 sat is seen in polycythemia vera? | incr plasma volume, very incr RBCs, no change in O 2 sat |
| What is the mechanism of heparin? | cofactor for activation of antithrombin. decr thrombin and Xa. short half life |
| What is the clinical use of heparin? Can it be used during pregnancy | immediate anticoagulation for PE, stroke, acute coronary sndrome, MI, DVT. can used during pregnancy |
| What lab value should be followed on a pt on heparin? | PTT |
| What are the major SE of heparin? | thrombocytopenia (HIT), osteoporosis |
| What can be used for rapid reversal of heparin SE? How does it work? | use protamine sulfate (positively charged that binds negatively charged heparin) |
| What is the advantages and disadvantages of low molecular weight heparins? | act more on Xa, better bioavailability. can be given subQ. dont lead to monitor labs buts its less reversible |
| How does heparin induce thrombocytopenia? | heparin binds platelet factor IV, causing Ab production that binds to and activates platelets leading to their clearance |
| What are lepirudin, bivalirudin? | hirudin derivatives, directly inhibit thrombin. used as alternative to heparin for anticoagulating patients with HIT |
| What is the mechanism of warfarin (coumadin)? | intereferes with normal synth and carboxylation of K dependent clotting factors II,VII,IX,X, protein C and S |
| What metabolizes warfarin (coumadin) and what lab values should be followeD? | metabolized by cyt p450. affects EXtrinsic pathay, and incr PT. Follow PT/INR |
| What is used for warfarin overdose? | vitamin K for slow reversal. for rapid reversal in a sever OD give fresh frozen plasma |
| What is the clinical use of warfarin? | chronic anticoagulation (post STEMI, venous thrombosis prophylaxis) |
| IS warfarin used in pregnancy? | NO |
| What are the major SE of warfarin (coumadin)? | bleeding, teratogenic, skin/tissue necrosis, |
| What is the structure of heparin vs warfarin? | heparin: large anionic, acidic polymer warfarinL small lipid soluble |
| What is the route of administration of heaprin vs warfarin? | heparin: parenteral (IV,SC). warfarin: oral |
| What is the site of action of heparin vs warfarin? | heparin: blood warfarin: liver |
| What is the onset of heparin vs warfarin? | heparin: seconds warfarin: slow, limited by half lives of normal clotting factors |
| What is the duration of action of heparin vs warfarin? | heparin: hours warfarin: days |
| Does heparin or warfarin inhibit coagulation in vitro? | heparin, not warfarin |
| What is the class of streptokinase, urokinase, tPA (alteplase), APSAC (antistreplase)? | thrombolytics |
| What is the mech of streptokinase, urokinase, tPA (alteplase), APSAC (antistreplase)? | directly or indirectly aid in conversion of plasminogen to plasmin which cleaves thrombin and fibrin clots |
| What is the effect of streptokinase, urokinase, tPA (alteplase), APSAC (antistreplase) on lab vaules? | incr PT, PTT |
| what is the clinical use of streptokinase, urokinase, tPA (alteplase), APSAC (antistreplase)? | early MI, early ischemic stroke |
| What are the major SE of streptokinase, urokinase, tPA (alteplase), APSAC (antistreplase)? | bleeding. contraI in pt with active bleeding, hx of intracranial bleed, surgery, sever HTN |
| How do you tx the SE of streptokinase, urokinase, tPA (alteplase), APSAC (antistreplase)? | aminocaprioic acid, an inhibitor of fibrinolyisis |
| What is the mech of aspirin? | acetylates and irreversibly inhibts COX1 and COX2 to prevent conversion of arachidonic acid to TXA2 |
| What is the effect of aspirin on BT and PT, PTT? | incr bleeding time no effect on PT and PTT |
| What is the clinical sue of aspirin? | antipyretic, analgesic, anti-inflammatory, antiplatelet drug |
| What are the major SE of aspirin? | gastric ulceration, bleeding, hyperventialtion, Reye's syndrome, tinnitus (CNVIII) |
| What is the mech of clopidogrel, ticlopidine? | inhibit platelet aggregation by irreversibly blocking ADP receptors. inhibits fibrinogen binding by preventing glycoprotein IIb/IIIa expression |
| What is the clinical use of clopidogrel, ticlopidine? | acute coronary sndrome, coronary stenting, decr incidence of recurrence of thrombotic stroke |
| What are the major SE of clopidogrel, ticlopidine? | netropenia (esp ticlopidine) |
| What is the mech of cilostazol, dipyridamole? | phosphodiesterase III inhibitor, incr cAMP in platelets thus inhibiting platelet aggregation; vasodilators |
| What is the clinical use of cilostazol, dipyridamole? | intermittent claudication, coronary vasodilation, prevention of stroke or TIAs (combined w aspirin), angina prophylaxis |
| What are the major SE of cilostazol, dipyridamole? | nausea, HA, facial flushing, hypotension, abdominal pain |
| What is the mech of abciximab? | monoclonal ab that binds to IIb/IIIa on platelets, preventing aggregations |
| What is the clinical use of abciximab? | acute coronary syndrome, percutaneous translumina coronary angioplasty |
| What are the major toxicities of abciximab? | bleeding, thrombocytopenia |
| What part of the cell cycle do antimetabolites interfere with? | S. DNA synthesis |
| What part of the cell cycle does etopodside interefere with? | S DNA synth, and G2 synthesis of mitosis components |
| What part of the cell cycle does bleomycin interfere with? | G2, synthesis of components for mitosis |
| What part of the cell cycle do vinca alkaloids and taxols interfere with? | M |
| What 3 antineoplastics inhibit nucleotide synth? | methotrexate, 5-FU (thymidine) and 6-MP (purines) |
| How do alkylating agents and cisplatin interfere with DNA? | cross link the DNA |
| How do dactinomycin, doxorubicin interfere with DNA? | DNA intercalators |
| How does etoposide interfere with DNA? | inhibits topoisomerase II |
| How do Vinca alkaloids inhibit cellular division? | inhibit microtubule assembly |
| How does Paclitaxel interfere with cellular division? | inhibits microtubule disassembly |
| What is the class of methotrexate, 5-FU, 6-MP, 6-TG, and ara-c? | antimetabolite neoplastics |
| What is the mechanism of methotrexate? | folic acid analog that inhibits DHF reductase= decr dTMP= decr DNA and protein synth |
| What is the clinical use of methotrexate for cancers? | leukemias, lymphomas, choriocarcinomas, sarcomas |
| What are the non neoplastic uses of methotrexate? | abortion, ectopic pregnancy, rheumatoid arthritis, psoriasis |
| What are the major SE of methotrexate? What can be done to reverse some of them? | 1. myelosurpression (reversible with leucovorin (folinic acid) 2. macrovesicular fatty change in liver 3. mucositis 4. teratogenic |
| What is the mechanism of 5-fluorouracil (5-FU)? | pyrimadine analog which covalently complexes folic acid. this complex inhibits thymidylate synthase= decr TMP, decr DNa and protein synth |
| What is the clinical use of 5-fluorouracil (5-FU)? What other anti neoplastic is it syngergisitc with? | colon cancer and other solid tumors, basal cell carcinoma. synergisitic with MTX |
| What are the major SE of 5-fluorouracil (5-FU) and how can an OD be rescued? | 1, myelosurpression that is reversible with thymidine 2. photosensitivity |
| What is the mechanism of 6-mercaptopurine (6-MP)? What activates it? | purine (thiol) analog which decr de novo purine synth, activated by HGPRTase |
| What is the clinical use of 6-mercaptopurine (6-MP)? | leukemias, lymphomas (not CLL or Hodgkin's) |
| What are the major SE of 6-mercaptopurine (6-MP)? What other drug can increase these and why? | bone marrow, GI, liver. metabolized by xanthine oxidase so toxicity will increase with allopurinol |
| What is the mech of 6-thioguanine (6-TG)? What activates it? | purine (thiol) analog which decr de novo purine synth, activated by HGPRTase |
| What is the clinical use of 6-thioguanine (6-TG)? | acute lymphoid leukemia |
| What are the major SE of 6-thioguanine (6-TG)? | bone marrow depression, liver toxicity. can be given with allopurinol unlike 6-MP |
| What is the mechanism of Cytarabine (ara-c)? | pyrimidine analog--> inhibits DNApol |
| What is the clinical use of Cytarabine (ara-c)? | AML,ALL, high grade non hodgkin's lymphoma |
| What are the major SE of Cytarabine (ara-c)? | leukopenia, thrombocytopenia, megaloblastic anemia |
| 5-FU would cause a build up of what substrate? | dUMP |
| MTX would cause a build up of what substrate? | DHF |
| What is the class of dactinomycin, doxorubicin, daunorubicin, bleomycin, etoposide, teniposide? | anti tumor abtx |
| What is the mechanism of dactinomycin, (actinomycin D)? | intercalates in DNA |
| What is the clinical use of dactinomycin, (actinomycin D)? | Wilm's tumor, Ewing's sarcoma, rhabdomyosarcoma. used for child hood tumors |
| What are the major SE of dactinomycin, (actinomycin D)? | myelosurpression |
| What is the mech of Doxorubicin (adriamycin), daunorubicin? | generate free radicals which noncovalently intercalate in DNa causing breaks and decr replication |
| What is the clinical use of Doxorubicin (adriamycin), daunorubicin? | Hodgkin;s lymphomas, also myelomas, sarcomas, solid tumors of breast ovary lung |
| What are the major SE of Doxorubicin (adriamycin), daunorubicin? What can be given to help prevent some of them? | cardiotoxicity (DCM), myelosurpression, alopecia. toxic to tissues with extravasation. Dexrazoxane (iron chelating agent) used to prevent cardiotoxicity |
| What is the mechanism of bleomycin? | induces free radical formation, which causes breaks in DNA strands |
| What is the clinical use of bleomycin? | testicular cancer, Hodkin's lymphoma |
| What are the major SE of bleomycin? | pulmonary fibrosis, skin changes. minimal myelosupression |
| What is the mechanism of Etoposide (VP-16), teniposide? | inhibits topoisomerase II--> incr DNA degradation |
| What is the clinical use of Etoposide (VP-16), teniposide? | small cell carcinoma of the lung and prostate, testicular carcinoma |
| What is the major SE of Etoposide (VP-16), teniposide? | myelosurpression, GI irritation, alopecia |
| What is the class of cyclophosphamide, ifofamine, mitrosureas ( carmustine, lomustine, semustine, streptozocin), busulfan? | Alkylating agent anti neoplastics |
| What is the mech of Cyclophosphamide, ifosfamide? What activates them? | covalently X link (interstrand) DNA at guanine N-7. Requires bioactivation by liver |
| What is the clinical use of Cyclophosphamide, ifosfamide? | Non hodgkin's lymphoma, breast and ovarian carcinoma. also immunosurpressants. |
| What are the major SE of Cyclophosphamide, ifosfamide? how can some of them be prevented? | myelosupression; hemorragic cystitis can be partially prevented with mesna |
| What is the mech of Nitrosureas (carmustine, lomustine, semustine, streptozocin)? | require bioactivation, cross blood-brain barrier-->CNS |
| What is the clinical use of Nitrosureas (carmustine, lomustine, semustine, streptozocin)? | brain tumors (including glioblastoma multiforme) |
| What are the major SE of Nitrosureas (carmustine, lomustine, semustine, streptozocin)? | CNS toxicity ( dizziness, ataxia) |
| What is the mechanism of busulfan? | alkylates DNA |
| What is the clinical use of busulfan? | CML, also used to ablate patients bone marrow before BMT |
| What are the major SE of Busulfan? | pulmonary fibrosis, hyperpigmentation |
| What is the class of vincristine, vinblastine, paclitaxel and the taxols? | microtubule inhibitors |
| What is the mechanism of vincristine, vinblastine? | alkaloids bind to tubulin in M phase and block polymerization of microtubules so that mitotis spindle cant form. microtubules are the VINES of the cell |
| What is the clinical use of vincristine, vinblastine? | Hodgkin's lymphoma, Wilm's tumor, choriocarcinoma, ALL |
| What is the major SE of vincristine? | neurotoxic (areflexia, peripheral neuritis), parylactic ileus |
| What is the major SE of vinblastine? | Bone marroe surpression |
| What is the mech of paclitaxel and the taxols? | hyperstabilize polymerized microtubules in M phase so the mitotic spindle cant break down (no anaphase) |
| What is the clinical use of paclitaxel and the taxols? | ovarian and breast carcinomas |
| What is the major SE of paclitaxel and the taxols? | myelosurpression and hypersensitivity |
| What is the mech of cisplatin, carboplatin? | cross link DNa |
| What is the clinical use of cisplatin, carboplatin? | testicular, bladder, ovarian, lung carcinomas |
| What is the major SE of cisplatin, carboplatin? how can these be prevented? | nephrotoxicity and acoustic nerve damage. prevent nephrotoxicity with amifostine (free radical scavenger), chloride diuresis |
| What is the mechanism of hydroxyurea? | inhbits ribonucelotide reductase--> decr DNA synthesis (S phase) |
| What is the clinical use of hydroxyureas? | melanoma, CML, Sickle cell disease (incr HbF) |
| What is the mech of prednisone? | may trigger apoptosis, might work on nondividing cells |
| What is the clinical use of prednisone? | used in CLL, Hodgkin's lymphoma (part of MOPP). also immunosurpressant used in AID |
| What are the major SE of prednisone? | Cushing-like syndrome, immunosupression, cataracts, acne, osteoprosis, HTN, peptic ulcers, hyperglycemia, psychosis |
| What is the mech of tamoxifen, raloxifene? | SERMs- receptor antagonists in breast and agonists in bones. block binding of estrogen to estrogen receptor + cells |
| What is the clinical use of tamoxifen, raloxifene? | breast cancer, also useful to prevent osteoporosis |
| What is the major SE of tamoxifen? | incr risk of endometrial carcninamo via partial agonist effects, hot flashes |
| what is the major SE of raloxifene compared to tamoxifen? | no incr in endometrial carcinoma because it is an endometrial antagonist |
| What is the mech of trastuzumab (herceptin)? | monoclonal ab against HER-2 (erbB2), tyrosine kinase. heps kill breast cancer cells which overexpress HER-2 |
| What is the clinical use of trastuzumab (herceptin)? | metastatic breast cancer |
| What is the major SE of trastuzumab? | cardiotoxicity |
| What is the mechanism of imantinib (gleevec)? | philadelphia chromosome bcr-abl tyrosine kinase inhibitor |
| What is the clinical use of imantinib (gleevec)? | CML, GI stromal tumors |
| What is the major SE of imantinib (gleevec)? | fluid retention |
| What is the mechanism of rituximab? | monoclonal Ab against CD 20 which is found on most B cell neoplasms |
| What is the clinical use of rituximab? | non-Hodgkin's lymphoma, rheumatoid arthritis (with methotrexate) |
| What antineoplastics commonly cause nephrotoxicity and acoustic nerve damage? | cisplatin/carboplatin |
| What antineoplastic commonly causes peripheral neuropathy? | vincristine |
| What antineoplastic commonly causes pulmonary fibrosis? | bleomycin |
| What antineoplastic commonly causes cardiotoxicity? | doxorubicin |
| What antineoplastic commonly causes hemorrhagic cystitis? | cyclophosphamide |
| What antineoplastics commonly causes myelosurpression? | 5-FU, 6-MP, methotrexate |
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tjs2123
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