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Hematology Internship Section 3
Coagulation/Hemostasis Coagulation Disorders. Page 99
43
Biology
Undergraduate 4
10/14/2015

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Term
Given the etiology, name the Coagulation Disorder associated with it:

Sex-linked DECREASED in Factor VIII:C, seen in males from carrier moms.

VIII:vWF NORMAL & Platelet Function NORMAL
Definition
Hemophilia A
Term
Given the etiology, name the Coagulation Disorder associated with it:

Autosomal deficiency of VIII:C & VIII:vWF. Seen in Males & Females.

Type 1 has a variably reduced level of vWF & VIII
Definition
von Willebrands Coagulation Disorder
Term
Given the etiology, name the Coagulation Disorder associated with it:

Sex-linked deficiency of factor IX
Definition
Hemophila B (Christmas Disease)
Term
Given the etiology, name the Coagulation Disorder associated with it:

Inappropriate, uncontrolled fibrin formation throughout the body from damage to endothelial cells
Definition
DIC / Consumption coagulopathy
Term
What is the most common of all of the single factor genetic deficiencies?
Definition
Hemophilia A
Term
What is the most common type of von Willebrands coagulation disorder?
Definition
Type 1
Term
What does the following suggest:
1% factor VIII activity
Definition
Severe Bleeding
Term
What does the following suggest:
5-12% factor VIII activity
Definition
Mild Disease
Term
What does the following suggest:
3-5% factor VIII activity
Definition
Moderate Disease
Term
Hemophilia A Carriers have _____ factor VIII Activity
Definition
50%
Term
What tests would you use to diagnose Hemophilia A?
Definition
Factor VIII Assay
INCREASED PTT
NORMAL PT
Term
True or False:
Hemophilia A will have an INCREASED PTT & a NORMAL PT
Definition
True
Term
True or False:
When considering Hemophilia A, the Factor assay will correct with Absorbed Plasma
Definition
True
Term
True or False:
When considering Hemophilia A, the Factor assay will NOT correct with Aged Serum
Definition
True
Term
True or False:
When considering Hemophilia A, the Factor assay will correct with Aged Serum
Definition
False
Term
True or False:
When considering Hemophilia A, the Factor assay will NOT correct with Absorbed Plasma
Definition
False
Term
Given the clinical representation, name the Coagulation Disorder:

Severe bleeding, especially in joints or after trauma; Carriers have NO symptoms and a NORMAL PTT
Definition
Hemophilia A
Term
What test will distinguish Hemophilia A from vWD?
Definition
NORMAL BT
Term
How should/would you treat Hemophilia A?
Definition
Factor VIII (AHF) concentrate or Cryo
Term
True or False:
AHF is safer than cryoprecipitate because the AHF concentrate can be virus inactivated.
Definition
True
Term
Given the clinical representation, name the Coagulation Disorder:

Mucosal bleeding, bruising, bleeding after hemostatic challenge
Definition
von Willebrands Coagulation Disorder
Term
Given the clinical representation, name the Coagulation Disorder:

Similar to hemophilia A, but not as Severe
Definition
Hemophilia B (Christmas Disease)
Term
Given the clinical representation, name the Coagulation Disorder:

Life-threatening thrombosis and bleeding
Definition
DIC
Term
If bleeding warrants, how should/would you treat von Willebrands Coagulation Disorder?
Definition
Desmopressin (DDVAP) or Cryoprecipitate
Term
Why would you use Cryoprecipitate to reat von Willebrands Coagulation Disorder?
Definition
Cry INCREASES Factor VIII activity higher than would be expected
Term
How would/should you treat Hemophilia B (Christmas Disease)?
Definition
Factor IX Concentrate, Prothrombin Complex Concentrate (PCC), or Fresh Frozen Plasma (FFP)
Term
With Coagulation Disorder is associated with sepsis, obstetrical complications, trauma, and malignancies?
Definition
DIC
Term
How would/should you treat DIC?
Definition
Support therapy, heparin, fibrinolysis inhibitors, and Cryo to restore fibrinogen.
Term
What will the PT and PTT be for von Willebrands Coagulation Disorder?
Definition
Slightly INCREASED PTT
NORMAL PT
Term
What will the BT be for von Willebrands Coagulation Disorder?
Definition
INCREASED BT
Term
What test would you perform, other than PT, PTT, & BT, to diagnose von Willebrands Coagulation Disorder?
Definition
vWF Antigen Immunoassay
Term
True or False:
There will be NO platelet aggregation with ristocetin for von Willebrands Coagulation Disorder.
Definition
True
Term
What will the PT & PTT be for Hemophilia B (Christmas Disease)?
Definition
INCREASED PTT
NORMAL PT
Term
What other test would you use to diagnose Hemophilia B (Christmas Disease)?
Definition
Factor IX Assay
Term
True or False:
Hemophilia B (Christmas Disease) will Correct with Aged Serum
Definition
True
Term
True or False:
Hemophilia B (Christmas Disease) will NOT Correct with Absorbed Plasma
Definition
True
Term
True or False:
Hemophilia B (Christmas Disease) will NOT Correct with Aged Serum
Definition
False
Term
True or False:
Hemophilia B (Christmas Disease) will Correct with Absorbed Plasma
Definition
False
Term
What will you see on the blood smear of a DIC Positive Patient?
Definition
Schistocytes
Term
What will the D-Dimer test be for a patient who is Positive for DIC?
Definition
Positive D-Dimer Test
Term
What will the Fibrinogen and Platelet Count be for a patient who has DIC?
Definition
DECREASED Fibrinogen
DECREASED Platelet Count
Term
What will the PT & PTT be for a patient who has DIC?
Definition
INCREASED PT
INCREASED PTT
Term
What will the TT & FSP be for a patient who has DIC?
Definition
INCREASED TT
INCREASED FSP
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