Hematology 2 Flashcards
(33 cards)
How andexanet works?
Cleave Factor Xa = no thrombin
PT measures?
PTT measures?
Ability to clot
Blood thinning drug ability
What plays role in initiation of coagulation cascade?
TF + F7a
what plays role in amplification of coagulation cascade?
Thrombin - self amplify
Thrombin activates what factors?
F5+8+9 = so produce more thrombin
What criteria needed for secondary hemostasis?
-ve charge phospholipid surface
Calcium + temperature
enough coagulation factors and fibrinogen
Initiation phase occurs in what pathway?
Amplification phase occurs in what pathway?
Intrinsic - initiation
Extrinsic - amplification
HBF consists of what chain?
2 alpha and 2 gamma globin chains
-before birth High HbF + gamma globin
-After birth - Hb rapidly increases
Erythropoiesis process takes place where before and after birth?
Before birth - liver
After birth + delivery = bone marrow
Hemoglobin constant spring (HBH) - what is it?
Non-deletional form of Alpha chain point mutation
-more severe
Leucocyte Alkaline Phosphatase = LAP
high in what?
Low in what?
High - physiological response/ PV/ET (mature WBC)
Low - CML/AML ( immature WBC)
Transfusion dependent Thalassemia are?
non-deletional HBH
Alpha thalassemia major
Beta thalassemia major
Beta thalassemia major/ HBE
Proteins in hereditary spherocytosis?
Proteins in hereditary eliptocytosis?
Affected vertical
Affected horizontal
Fanconi anemia clinical features?
Progressive pancytopenia - BM failure
AML
Head, neck, oropharyngeal carcinoma
Gardner syndrome?
Subtype of FAP (colon + extra colonic tumors)
-osteomas of skull/ Thyroid cancer/Fibromas/Desmoid tumors
Paraneoplastic pemphigus (PNP) are strongly associated with what condition?
What antibodies strongly associated with PNP?
Non-Hodgkin lymphoma/ CLL
Periplakin/Envoplakin
Test for vWF disease?
1)vWF antigen - check vWF protein level
2)vwF RCo (gold standard) - check vWF quality
: Usage of Ristocetin (abx) to promote platelet aggregation + vWF release
3)F8 levels
CAR T-cell therapy function?
Modify T cells receptors to enable them to recognize cancer cells and attack via perforin + grannzymes
Receptors becomes chimeric
- Antigen-binding
- T-cell activating function
BiTE therapy function?
Form link between T cell and tumor cells
-T cell perform cytotoxic stuff INDEPENDENT of MHC I/co-stimulatory
Hepatic sinusoidal obsturction syndrome usually a/w what?
HSCT - post 1 month
Repitilase does what?
Purified reptilian acts as thrombin
Cleaves fibrinopeptide A - generates fibrin
Thrombin does what?
Cleaves both fibrinopeptide A and B to generate fibrin clot
NADPH source in RBC relies on?
Pentose monophosphate shunt ( the only way)
In Polycythaemic Rubae Vera - which mutation has best prognosis?
CARL+ve
2nd is isolated JAK+ve