A 12-year-old boy underwent a tooth extraction and experienced persistent bleedi

Question

A 12-year-old boy underwent a tooth extraction and experienced persistent bleeding following the procedure. The patient had a history of bruising and frequent epistaxis. The patient’s mother also experienced easy bruising and menorrhagia. Physical examination revealed several ecchymotic lesions on the lower extremities. Laboratory findings are described below

Test

Result

Reference Range

PT

12.0 seconds

10.0-14.0 seconds

APTT

40 seconds

23-36 seconds

Bleeding Time

>15 minutes

2-9 minutes

Platelet Count

300 x 109/L

150-400 x 109/L

Factor VIII activity

30%

50-150%

vWF:Ag

45%

50-150%

vWFR:Co

41%

50-150%

RIPA

Depressed Response

Normal Response

1 What disorder is present?

2. What does vWF:Ag measure?

3. What does vWFR:Co measure?

4. What confirmatory testing should be performed?

Test

Result

Reference Range

PT

12.0 seconds

10.0-14.0 seconds

APTT

40 seconds

23-36 seconds

Bleeding Time

>15 minutes

2-9 minutes

Platelet Count

300 x 109/L

150-400 x 109/L

Factor VIII activity

30%

50-150%

vWF:Ag

45%

50-150%

vWFR:Co

41%

50-150%

RIPA

Depressed Response

Normal Response

Explanation / Answer

Answers

1. von Willebrand Disease

the bleeding disorders are mainly occurs due to the lack of proper hemostatic mechanisms. The The hemorrhagic diseases are hereditary or acquired. The above case the boy and his mother has bledding problem so,maximum hereditary disease. The most frequent hereditary disease is Von Willebrand disease (VWD). This is mainly due to the lack of deficiency of von Willebrand factor. von Willebrand factor is a glycoprotein synthesized by endothelial cells and present in plasma an platelets.

There are three types of von willebrand diseases.

Type 1 -- Partial deficiency of VWF

Type 2 --- qualitative defects in VWF

Type 3 -- Severe deficiency of VWF and reduction in clotting factor VIII.

Question 2. the Ristocetin Cofactor assay (VWF:RCo) used to determines function of the von willebrand protein.

The von willebrand disease is diagnosed by the different tests

Hematological test the bleeding time , activated partial thromboplastin time and platelets count.

In von willebrand diseaseneed some special tests to confirm. These are

1. the ristocetin cofactor assay (VWF:RCo)

2. VWF protein concentration immunoassay (VWF:Ag)

3. the FVIII coagulation assay

4. VWF binding to immobilized collagen (VWF:CB).

Question 3. vWF:Ag is used to measure the amount of the VWF protein present in the blood.

It is also one of the major reason for the development of von willebrand disease.

Question 4. The Ristocetin cofactor assay (VWF:RCo) is considered the most specific test for confirmation. The severity of diseases is identified by the reduction degree of VWF:RCo and FVIII ratio.

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