Hematology 2 Flashcards
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
CLL is disorder in what?
B cell - hypogammaglobulinemia
DLBCL
- DNA microanalysis that has good prognosis?
- DNA microanalysis that has bad prognosis?
Good prognosis - Germinal B-cell DLBCL
Bad prognosis -Activated B-cell DLBCL (ABC)
Cold AIHA antibody towards?
Warm AIHA antibody towards?
Cold - CD3
Warm - IgG
Mantle Cell lymphoma has what phenotype?
CD19 - its a B cell lymphoma
CD23 NEGATIVE!!
Cyclin D1 expression
Hairy Cell Leukemia does not express what?
CD5
How to distinguish anemia of chronic disease and anemia of iron deficiency?
(Functional status IDA)
sTFR - soluble transferrin receptor INCREASED IN IRON DEFICIENCY
low in chronic disease - hemachromatosis/hypoalbuminemia
Which blood group more susceptible to Cholera?
O group - unclear mechanism
What differentiate CLL from CML?
CD23 POSITIVE
Smudge cells
When Treat CLL?
SALE
Splenomegaly/Symptomatic - B symptoms
AIHA/ITP
Lymphocytosis - doubling >50% in 2 months
Enemia/Thrombocytopenia