Question | Answer |
t(15;17) | Acute promyelocytic leukemia /M3
Females w/ sudden onset of bleeding// DIC
Retinoic acid receptor alpha gene
Retinoic acid= complete remission |
t(9;22) | CML
Philadelphia chromosome// flower garden/// decreased LAP score |
t(8;21) | AML/ M2
Adults, dysmeylopoiesis, psuedo pelger huet
Myeloblastic anemia w/o maturation |
11q23 | AML/M5
Schilling type
Monoblastic young adults & children |
t(16;16) | AML myelomonocytic
M4 Eo variation |
CD 10, CD19, CD22, CD24 | ALL B cell precursors |
CD2,CD4, CD5, CD8 | ALL T cell precursors |
CD 19, CD20, CD23, CD5 | CLL |
CD103, CD11c, CD25, TRAP | Hairy cell leukemia |
Waldenstrom's macroglobulinemia | Malignancy of the lymphoplasmacytoid cells which manufacture IgM |
WBC scatter plot:
A: top left, B middle, C right, D bottom left | A:monocytes
B:neutrophils
C: Eosinophils
D: lymphocytes |
Why is the WBC count increased when the PB is analyzed automatically in a sickle cell anemia patient? | Sickled RBC's are resistant to lysis so are counted among the WBC population |
What reagents are used in the PT test? | Thromboplastin and calcium replace the tissue thromboplastin and calcium necessary in vivo to activate factors VII and VIIa. this ultimately generates thrombin from pro thrombin via the coagulation cascade |
Which ratio of coagulant to blood is correct for coagulation procedures? | 1:9 |
The anticoagulant of choice for most routine coagulation studies is... | Sodium citrate |
Which results would be expected for the prothrombin time PT and activated partial thromboplastin time APTT in a patient with polycythemia? | Both prolonged: The blood in a polycythemia patient contain so little plasma that access anticoagulant remains in is available to buy in to reagent calcium thereby resulting in a prolongation of the PT and aPTT |
Which test would be abnormal in a e patient with Stewart power factor (factor X) deficiency? | Stewart power factor (factor X)!is involved in the common pathway of the coagulation cascade therefore it's deficiency prolongs both PT in aPTT |
Normal Hgb | A, F, A2 |
Sickle cell trait hgb | A: 50-70%, S: 20-40%, A2,F |
Sickle cell anemia Hgb | S, F, A2 |
Hgb AC trait | C,A,
A2, F |
Hgb SC disease | C,S, F
A2 |
Beta thalassemia minor Hgb | A2, A in equal parts
F small |
Beta thalassemia majior | Lg F, md A2, small A |
Which clotting factor is not measured by PT and APTT test? | Factor XIII (fibrin stabilizing factor)
It creates covalent bonds between fibrin monomers formed during the coagulation process to produce a stable fibrin clot. |
What happens in the absence of factor 13 | The hydrogen bonded fiber in a polymers are soluble and 5M urea or in 1% monochloroacetic acid |
A modification of which procedure can be used to measure measure fibrinogen? | Thrombin time. Fibrinogen can be quantitatively measured by a modification of thrombin time by diluting the plasma since of thrombin clotting time of diluted plasma is inversely proportional to the concentration of fibrinogen. (th clause method) |
Vitamin K is necessary for activation of which clotting factors | Factors II,VII, IX and X
It is required for Corboxylatiin of glutamate residues of these coagulation factors |
Is vitamin K enhanced by heprin therapy | No |
Distribution 30% spleen, 70% peripheral blood
Reference ranges for platelets are 150,000 to 400,000/ mm3
the lifespan is 9 to 12 days | |
What does the PFA test do | A test to see if platelets properly adhere an aggregate at the injury site. Basically checks if platelets are working correctly
It takes the place of the historical bleeding time test |
What does thrombin do? | Cleves peptides off of the fibrinogen molecules to form fibrin which polymerizes to form insoluble fibrin strands.
thrombin also activates factor 13 which cross-links the fibrin strands at the D region . (birth of the D dimer) |
Other function is of thrombin are | Feeds back to protonate factors V & VIII
recruits and aggregates platelets
turns on endothelial cell thrombomodulin (receptor/ activator for protein C and protein S system) to in activate factors V and VIII. |
INR : | International normalized ratio reference ranges under 13 seconds therapeutic goal is 2.0 to 3.5
INR( patient result/ mean of ref range)^ISI |
APTT | Screen for intrinsic and common pathways
measures all factors except for VII & XIII
monitors heparin therapy.
Reagents: activator (Kaolin, celite, or ellagic acid), PLT phospholipid, cacl2 |
APTT
reference range | 20 to 40 seconds
therapeutic goal is 1.5 to 2.5 times normal or use heparin response curve |
Fibrinogen assay | Quantitative measure of factor I (fibrinogen)
reference ranges : 200 to 400 mg/dl |
PT prothrombin time | Screen for extrinsic and common pathways
Measures factors I, II, V, VII, and X
Monitors oral anticoagulants (warfarin Coumadin)
Reagents: tissue thromboplastin and CACL2
sensitive to vitamin k factors
INR |
PT time reference range | < 13 seconds
therapeutic reference range: 2.0 to 3.5 |
Von Willebrand's disease | Wimpy Willy
normal platelets
decreased factor VIII
decreased vWF |
Bernard-Soulier | Super Bernie giant platelets |
Reptile lease time | Snake venom enzyme.
Test similar to thrombin time but is not inhibited by heparin (good to use on patients on heparin)
reference range: 18 to 22 seconds |
Abnormal ristocetin induced aggregation | Bernard- Soulier & von Willebrand |
Hemophilia A | Inherited disorder
deficiency of factor VIIII, sex linked recessive (men)
spontaneous bleeding into joints, treat with commercial factor VIIII |
Hemophilia B | Inherited disorder deficiency and factor IX,
sex linked recessive ( men)
clinically identical; hemophilia A
treat with factor IX concentrates |
Hemophilia C | Inherited disorder, deficiency in factor XI
Incomplete Autosomal recessive,
wide ranging clinical severity, high incidence in Ashkenazi Jews, only contact factor associated with bleeding |
Von Willebrand disease | Inherited disorder, primary { 1*}defect in the von Willebrand factor, vWF factor Binds platelets via the glycoprotein 1B/V/IX receptor.
2*: deficiency a factor VIII, Autosomal dominant abnormal PFA test treat with cryoprecipitate |
Factor XIII DEFICIENCY | Inherited disorder, Autosomal recessive, not detected by common coagulation test, poor wound healing, detected with the 5M urea test |
Inherited disorders coagulation | Hemophilia A, hemophilia B, hemophilia C, von Willebrand disease, factor XIII deficiency |
| |
Intrinsic pathway | Pk, hk, XII, XI, IV, VIII |
Extrinsic | III, VII, TF |
COMMON patheay | X, V, II, I
II: prothrombin
I: fibrinogen |
What is required to activate Protien C and become fully functional? | Streptokinase |