[khanar]

A 7 Year old boy presented with refractory epistaxis that has been continuous despite adequate pressure for the last 6 hours. Parents also gave history of similar episodes in his older brother.
Hematologic values were:
--RBC count 5 million
--Hemoglobin 12 gram/dl
--Hematocrit 45%
--Platelet count 230 thousands
--PT, PTT within normal
--Response to Ristocetin was normal
--Blood film normal

Which of the following is the best step in the management?

A- Platelet transfusion
B- Eptifibatide
C- Blood transfusion
D- Local fibrinolytics
E- Observation

 

[tmsid]

Juvenile Nasopharyngeal Angiofibroma ?

Maybe we have to observe this patient, as the possibility of vW Disease is out, and the PT, and PTT are normal suggesting no problems with the clotting pathway... and the plats look like they're in a normal range

There are no options suggestive of embolisation, and usually, the patients are pre treated with DES before surgery to make the tumors less vascular (and since it is mostly found in men, DES isn't that big a deal for obvious reasons) - OBSERVE is my answer...

 

[khanar]

Glanzmann Thrombosthenia

While tmsid diagnosis is a possibility but I think the question is pointing to Glanzmann thrombosthenia specially when they mentioned his brother has similar problems (autosomal recessive inheritance).
So I think the correct answer in this case is platelets.

 

[matz]

A 7 Year old boy presented with refractory epistaxis that has been continuous despite adequate pressure for the last 6 hours. Parents also gave history of similar episodes in his older brother.
Hematologic values were:
--RBC count 5 million
--Hemoglobin 12 gram/dl
--Hematocrit 45%
--Platelet count 230 thousands
--PT, PTT within normal
--Response to Ristocetin was normal
--Blood film normal

Which of the following is the best step in the management?

A- Platelet transfusion
B- Eptifibatide
C- Blood transfusion
D- Local fibrinolytics
E- Observation

hi I think there are also 2 other DDx:
1-type 2 M of vW Disease in which only BT is prolonged while PT and PTT are in normal range.
2-deficiency of factor XIII in which all the coagulation tests are normal, but there is significant active bleeding.

any idea how to prove angiofibroma of nasopharyncs?

 

[khanar]

It's Glanzmann

hi I think there are also 2 other DDx:
1-type 2 M of vW Disease in which only BT is prolonged while PT and PTT are in normal range.
2-deficiency of factor XIII in which all the coagulation tests are normal, but there is significant active bleeding.

any idea how to prove angiofibroma of nasopharyncs?

In Von Willbrand you have abnormal response to Ristocetin so that's excluded.
Factor XIII presents far earlier in the neonatal period and it's not an option in the question above.

 

[FSUSTC]

confused with aalous

I think its platelet transfusion .... due in Glanzmann thrombasthenia .. everything is normal except bleeding time ...
You said his brother has the disease and he is also .... but in autosomal recessive disease .. 25 % affected ( which means 1 child ) , 50 % carrier , 25 % no affect .... its means 75 % will not show sign symptom ... Means any one of her son will be affected not both of them ... i think so ....... But yes in X recessive if mother is carrier .. 50 % cases son will be affected ...

 

[matz]

In Von Willbrand you have abnormal response to Ristocetin so that's excluded.
Factor XIII presents far earlier in the neonatal period and it's not an option in the question above.

thanx for ur response but i meant a special type of Von Willbrand "type 2M" in which all the tests are normal even response to RCOF, except BT that is prolonged.

how can u exclude angiofibroma?

 

[FSUSTC]

In type 2M VWD - you will get low vwf activity ... Check it out
http://emedicine.medscape.com/article/959825-overview

In angiofibroma .. usually present with sign of obstruction and epistaxis is usually chronic , recurrent , unilateral .... here no prev history of recurrence