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hemonc
hematology oncology hematopoeisis and myeloproliefeartive
51
Medical
Graduate
09/03/2011

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Term
CFU-Meg
Definition
diff factor to megakaryocyte, eventually become platelets
Term
GCF
Definition
amelioration of luekocyte associated anticancer chemotherapy. Priming patietns for autologous transplantation collection. Also for alloegeniec donor priming.
Term
TPO
Definition
megakaryocte to platelets
Term
EPO
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
Term
cd 2-10
Definition
t lymph markers
Term
cd 11-15
Definition
myeloid and monocyte markerss
Term
cd 19-23
Definition
b lymph markers
Term
cd 33
Definition
immature myeloid marekr
Term
cd 34
Definition
hemtopoietc progenitor marekr
Term
HSC morphology and immunophenotype
Definition
mononcuelar, agranular cytoplasm, single nucleuous. Immunophenotype: Thy+ Rhodamine 123dull c-kitbright CD34+.
Term
hemotpoeitn grwof factor classes
Definition
1. bind to hemopoeitin receptor superfamily 2. kinase receptor superfamily.
Term
GMCSF
Definition
very similair to GCSf- however causes fever
Term
IL-2
Definition
stimulates t cells against renal cancer and melanoma. 9 t cel lgrowth factor) . V ery toxic.
Term
IL-11
Definition
amelioration of thrombocytopenia.
Term
TPO
Definition
stimuatled produciton fo paletelts, whoever also a ctivated them , causin clotting. So abandoned
Term
Stem cell factor
Definition
potential synergist with other cyotkiens. However is also mast cel lgrwoth factor, which creates hypersensitivyt oxicity. Abandoned.
Term
Romiplostim
Definition
immunoglobulin type molecule , TPO receptor agonist, make platelet count go up
Term
eltrombopag
Definition
Small molecule taken orally, TPO receptor agonist
Term
il-3
Definition
synthed by activated t cells feeds back to HSC, broad acting , potential synergist with other cyotkiens, however didn’t work
Term
Il-4
Definition
b cell growth factor
Term
IL-5
Definition
enhances life and activity of eosonophils.
Term
CML
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.
Term
Philadelphai chrosome
Definition
CML, t (9:22) ,bcr-abl, results in failure of apotpotis
Term
essentialthromboytosis diagnosis/syptoms/ treatment
Definition
sustained platelet count of more than 450 k without any other cause . idnetifiable. Patients presenting with: Thrombois or diathesis if paltelets dysfunctional.
Term
plycythemia vera syptoms defintiion treatment : o
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 .
Term
disease in which jak/stat mutation. Also may be seen in ET.
Definition
polycythemia vera- constituve activation of jak/stat instead of EPO. SO EPO not required.
Term
myelofibrosis and myelodi metaplasia definition syptoms: treatmetn/charactteristic . prognosis criteria: treatment: transplant.
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
Term
CELHES : myeloproliverative variant
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 .
Term
CEL/HES: lypholiferative variatn
Definition
abbertat t cl lpoulation. More indolent than Myeloproliferative. Clonat t cell rearragnement,. Increased T cell production of eospophillopoietics.
Term
systemic mastocytosis:
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
Term
CML histology
Definition
chronic phase- cells of all different types seen, blast phase, more monotoneous with cells of one immature type.
Term
CML AP Phases and criteria for diagnosis
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.
Term
CML BP
Definition
20 percent blasts, extramedullary blast proliferation
Term
Imatinib
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.
Term
differential for throbocytosis
Definition
reactive, CML, Polycythemia Vera, Myelodysplatic sydnrom (5qminus presenets with eelvated paltelets).
Term
causes fo secodnary thrombocytopenia
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.
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
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
Term
histology of essential thrombocythemia
Definition
number of platelets is abnormally high aand are dysplastic, boen marrow is hyeprceullaular
Term
managmemtn for essential thrombocythemia
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.
Term
polycythema vera histology
Definition
bone marrow: hypercellular, icnreased rbc precursors.
Term
secondary forms of erythrocytosis.
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
Term
polycythema vera mutation
Definition
jak2 mutation occurs, causing cytokine receptor to always be on , epo receptor is insenstive to epo and is consituvively active.
Term
clssification of mast cell disease
Definition
cutaneous, idnolent systemic , systemic( other organs) , mast cel leuekmias, and mast cell sarcomas
Term
bone marrow composition
Definition
hematopoeitc cellss , microvasculature, extracellular matrix , stromal cells, stromal/ecm cells can feedback and tlell cells what to do.
Term
spectrin-ankrin
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
Term
spherocyte slide
Definition
harrison lecture
Term
neutrophil histology
Definition
3 segments
Term
monocyte
Definition
horshoe nucleus
Term
plasma cell
Definition
eccentric nucleus
Term
Essential thrombocytosis mutation
Definition
jak2 mutation
Term
dacrocyte
Definition
myelofiboriss
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