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Definition
| diff factor to megakaryocyte, eventually become platelets |
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Definition
| amelioration of luekocyte associated anticancer chemotherapy. Priming patietns for autologous transplantation collection. Also for alloegeniec donor priming. |
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Definition
| megakaryocte to platelets |
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Definition
| rbc cant bee madewithout it . Used for anemia caused by renal failure. Then used for chronic infalmamtory diseases and cancer. chemotherapy . Cardiovascualr issue as well as incresed tumor progression/ thromboembolism |
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Definition
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Definition
| myeloid and monocyte markerss |
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Definition
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Definition
| hemtopoietc progenitor marekr |
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Term
| HSC morphology and immunophenotype |
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Definition
| mononcuelar, agranular cytoplasm, single nucleuous. Immunophenotype: Thy+ Rhodamine 123dull c-kitbright CD34+. |
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Term
| hemotpoeitn grwof factor classes |
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Definition
| 1. bind to hemopoeitin receptor superfamily 2. kinase receptor superfamily. |
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Definition
| very similair to GCSf- however causes fever |
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Definition
| stimulates t cells against renal cancer and melanoma. 9 t cel lgrowth factor) . V ery toxic. |
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Definition
| amelioration of thrombocytopenia. |
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Definition
| stimuatled produciton fo paletelts, whoever also a ctivated them , causin clotting. So abandoned |
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Definition
| potential synergist with other cyotkiens. However is also mast cel lgrwoth factor, which creates hypersensitivyt oxicity. Abandoned. |
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Definition
| immunoglobulin type molecule , TPO receptor agonist, make platelet count go up |
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Definition
| Small molecule taken orally, TPO receptor agonist |
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Definition
| synthed by activated t cells feeds back to HSC, broad acting , potential synergist with other cyotkiens, however didn’t work |
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Definition
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Definition
| enhances life and activity of eosonophils. |
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Term
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Definition
| overpoduction of granulocyte and megakaryocyte, however also lymphoid levels are increased, a true disease of hematopoeticstem cell.- normal differentation and funcitoning , just too many. (unless blast phase, where a lot of immature blasts seen). HSC myeloprolifertiave. Disease of adults. |
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Term
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Definition
| CML, t (9:22) ,bcr-abl, results in failure of apotpotis |
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Term
| essentialthromboytosis diagnosis/syptoms/ treatment |
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Definition
| sustained platelet count of more than 450 k without any other cause . idnetifiable. Patients presenting with: Thrombois or diathesis if paltelets dysfunctional. |
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Term
| plycythemia vera syptoms defintiion treatment : o |
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Definition
| verpdocution of rbcs, granulocytes, platlets, and splenomegaly. Symptoms: dyspnea , high whole blood viscoity, cns, thrombosis and erythromelalgiea (itching form warm water) , can also bleed, plethora (redness fo complexion). LOW SERUM EPO . Treatmetn: venesection/removal to maintain hematocrit. low doseaspirin. Manage thrombotic risk factors. cytoredution if top methods dotn work . |
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Term
| disease in which jak/stat mutation. Also may be seen in ET. |
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Definition
| polycythemia vera- constituve activation of jak/stat instead of EPO. SO EPO not required. |
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Term
| myelofibrosis and myelodi metaplasia definition syptoms: treatmetn/charactteristic . prognosis criteria: treatment: transplant. |
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Definition
| : fibrosis of amrrow cavity ,creating ihospoital env for hemtopoeis. Hematopoeitc progenits leave bone marrow reside in spleen and liver. Pancytopenia. Syptoms: fatigue enlarged spleen/liver easy bleeidng , fevers, bone pain, shortness of breath, easy brusiigng , pale skin. Dacrocyte: tear drop cell . prognosis: wbC /hb (wbc may be high or low). - graded based on three levels. treatment: transplatn |
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Term
| CELHES : myeloproliverative variant |
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Definition
| FIP/IL1 fusion genetic fusion. Eosophillic clonaility. Abnormalities in PDGF 9constituve activation.. Eosonophilic overgrowth. Signs/syptoms: icnreased tryptase levels, increased b12 levels , incresed mast cells- also resutls in increased endomycardium fibrosis due to accumualtion of eosoionphil basic protein . |
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Term
| CEL/HES: lypholiferative variatn |
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Definition
| abbertat t cl lpoulation. More indolent than Myeloproliferative. Clonat t cell rearragnement,. Increased T cell production of eospophillopoietics. |
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Term
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Definition
| multifocal dense ifnitlrates of mast cells inbone marrows or extracutaneous organs. Activation mutation; D816V. Syptoms: derived from release of mast cell mediators (i.e. histamine)- anaphylaxis/rash , osteoperios, lytic lesions, dairrhea ,adn ulcersetc. Treatment: avoid triggersand some antihistamines |
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Term
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Definition
| chronic phase- cells of all different types seen, blast phase, more monotoneous with cells of one immature type. |
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Term
| CML AP Phases and criteria for diagnosis |
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Definition
| AP: 10-19 percent blasts in periph or bone arrow, basophils of at least 20 percent. Persistent thrombocytopenia/thrombocytosis.(chronic phase allwos fo rpoduction of plateletes wheras blast/accelerated phasem ay impair). Icnreasing wbc count unreposnive ot therapy and icnreasing spleen size, cytogenetic evidvence of clonal evolution. |
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Term
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Definition
| 20 percent blasts, extramedullary blast proliferation |
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Term
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Definition
| drug used to treat CML, specifically philadelphia chromosome- operates by going into bcr-able tyrosine kianse active site, inhibiting atp dependant phosphorylatio of substrates . However there are restiances iemergnign to Imatinib. |
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Term
| differential for throbocytosis |
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Definition
| reactive, CML, Polycythemia Vera, Myelodysplatic sydnrom (5qminus presenets with eelvated paltelets). |
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Term
| causes fo secodnary thrombocytopenia |
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Definition
| acute blood loss, recovery from thrombocytopenia (rebound) acute infection/ifnlammation, resposnet oe xcercise. Iron deficiency ,hemolytic anemia, asplenia, cancer, chronic finallamtory or ifnectious diseases, drug reactions. |
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Term
| distinguishing between clonal thrombocytosis and secondary (based on disease, digitial /cerbocascul ischemia, large vessel arterial thrombosis, bleeidng complciations , splenomegaly, peripheral blodo smear, platelet function, bone marrow megakaryotcytes, nubmerand morpholobical features |
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Definition
| no udnerlying systemic disease in clonal but present in reactive , digital/cerebro ischemai characteristic in clonal not in secodary, large vessel thrombosis icnreased inc lonal , nto in secondary, bleedign complciations incerasedi nc loanl, not in secondary. splenomegaly icnreasedi ncloanl not insecondary. peripheral blood smear shwos giant platelets in clona l ,normal i nsecodnary,platelet function ma ybe normal in secondary, but abnormal in cloanl. nubmer of megakaryocytes in both is increased, however clonal thromsosis has giant dysplasmit forms |
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Term
| histology of essential thrombocythemia |
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Definition
| number of platelets is abnormally high aand are dysplastic, boen marrow is hyeprceullaular |
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Term
| managmemtn for essential thrombocythemia |
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Definition
| main problem is to prevent clottign ,not worried about progressiotn o leukemias. So for all patients, important ot manage reversible cardiovascular risk factors, high risk pations (with prior thrrombosis or over 6 0years) can get aspirin blut hydroxyurea. Intermedaite (40-60 with no high risk features) can stay on low dose asporin unelss cardiovascular featurespresent. |
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Term
| polycythema vera histology |
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Definition
| bone marrow: hypercellular, icnreased rbc precursors. |
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Term
| secondary forms of erythrocytosis. |
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Definition
| high epo levels high o2 affinity of rbc resultign in decerased delivery, vhl mutation resulting in defects of 02 sensation , hpoxia resposne /carbon monoxide rsponse. May also be due to ectopic epo production |
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Term
| polycythema vera mutation |
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Definition
| jak2 mutation occurs, causing cytokine receptor to always be on , epo receptor is insenstive to epo and is consituvively active. |
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Term
| clssification of mast cell disease |
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Definition
| cutaneous, idnolent systemic , systemic( other organs) , mast cel leuekmias, and mast cell sarcomas |
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Term
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Definition
| hematopoeitc cellss , microvasculature, extracellular matrix , stromal cells, stromal/ecm cells can feedback and tlell cells what to do. |
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Term
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Definition
| unerlies red cell membrane formign ytoskeleton. Linked to membrae via ankryn. Mutations in any of these proteins cause the rbcs characteristic shape to change and may cause fragmentation. Also band 4.1 |
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Definition
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Definition
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Definition
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Definition
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| Essential thrombocytosis mutation |
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Definition
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