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**صلاح عبد الملك احمد عبد ا** · 08-09-2009, 09:39 PM

Open your mind

المشاركة الأصلية بواسطة ashraf shaq مشاهدة المشاركة

السلام عليكم

If we have the following situation:

patient suffers from a severe bleeding diathesis since birth given that this patient have not taken any medication

you have the following results
Normal PT, APTT, TT, Normal platelets in quantitiy and quality
What is your strategy of diagnosis?

Awaiting for your diagnostic and differential diagnostic strategies .................

يلا بدنا نسمع اشي .........................

This patient maybe have a disease in endothelial cell that lininig blood capillaries ( as Hemolytic Uremic syndrom

)

**hematologist** · 08-09-2009, 10:18 PM

Congenital plasminogen activator inhibitor-1 (PAI-1) deficiency is an extremely rare disorder characterized by a bleeding diathesis that begins in childhood due to hyperfibrinolysis as a result of decreased PAI-1 activity.

**mohammad aboghabsha** · 08-09-2009, 10:48 PM

all congenital vascular causes of purpra e.g.:
Ehler's danlos syndrome
hereditary heamorrhagic telangiectasia

**bhloool** · 09-09-2009, 01:08 AM

i say vascular disorders
:sm198::sm198:

**akram61** · 09-09-2009, 04:59 AM

[
LEFT]

Dear professor Ashraf;

This is a very good question i ll try to give you some hints:
as the bleeding is congenital and is severe you have to consider first factor XIII deficiency the factor that stabilizes fibrin clot by cross linking fibrin threads...usually it is associated with sever bleeding ,.
in my opinion this is the differential diagnosis number one and i have to measure factor XIII though it is not a routine test.
other causes that may give the same results and are associated with bleeding that is usually mild to moderate are :
defects in fibrinolysis as one of our colleagues mentioned PAI-1 defiency we call it delayed bleeding because it occures usually 24-36 hours following surgery,or other rare disorders in collagen (The vascular purpuras)
i have to tell you that i was not faced with such a case before so i am waiting for the strategy of diagnosis i am sure you will teach us a good one. )
thank you again for giving us the opportunity to open our minds[/LEFT]...

**akram61** · 09-09-2009, 09:42 AM

تصحيح للجملة الغير مرتبة

Sorry for the mis-arrangement in the previous paragraph,the correct one is below here
Dear professor Ashraf;

[/LEFTthis is a very good question i ll try to give you some hints

:
as the bleeding is congenital and is severe you have to consider first factor XIII deficiency the factor that stabilizes fibrin clot by cross linking fibrin threads...usually it is associated with severe bleeding .
in my opinion this is the differential diagnosis number one and i have to measure factor XIII though it is not a routine test.
other causes that may give the same results and are associated with bleeding that is usually mild to moderate are :
defects in fibrinolysis as one of our colleagues mentioned PAI-1 defiency,we call it delayed bleeding because it occures usually 24-36 hours following surgery,or other rare disorders in collagen (The vascular purpuras)
i have to tell you that i was not faced with such a case before so i am waiting for the strategy of diagnosis i am sure you will teach us a good one. )
thank you again for giving us the opportunity to open our minds...

**ashraf shaq** · 09-09-2009, 10:16 AM

شكرا لكل الذين شاركوا بس بدنا كمان ردود .................

**samah ahmed** · 09-09-2009, 01:23 PM

the four line investigation are normal but still patient continue to bleed.
.at this case exclude all coagulation factor except xiii
it may be plt functional abnormality or blood vessel abnormality not strucural but functional abnormality
bleeding from sever damage of BV.
mild deffiency of fviii and ix.
:sm191:

**samah ahmed** · 09-09-2009, 01:26 PM

suspected case of bleeding disorder this case NO1

**akram61** · 09-09-2009, 05:06 PM

Another less common cause would be some types of dysfibrinogenemia that can be associated by bleeding rather than thrombosis or being asymptomatic...but bleeding of such cases is always mild to moderate and the screening test for their diagnosis is the thrombin time and Reptilase time.

**ashraf shaq** · 09-09-2009, 08:26 PM

Think again

المشاركة الأصلية بواسطة akram61 مشاهدة المشاركة

Another less common cause would be some types of dysfibrinogenemia that can be associated by bleeding rather than thrombosis or being asymptomatic...but bleeding of such cases is always mild to moderate and the screening test for their diagnosis is the thrombin time and Reptilase time.

Prof Akram It is true what you have said about this hint, but we have said is a severe bleeding, and normal TT and thus we do not need to perform Reptilase time since it will be also normal.Also dysfibrinogenemia is more associated with thrombotic episodes. But what you said in first hint is more belivable.
And by the way this is a real case that I have faced since about 15 years ago.
Continue thinking Prof. Akram
Thank you very much for your participation.

**mohammad aboghabsha** · 10-09-2009, 01:00 AM

الاجابه فيييييييييييييييييييييييييييييييييييييييييييين
:sm170:

**ashraf shaq** · 10-09-2009, 06:21 PM

المشاركة الأصلية بواسطة mohammad aboghabsha مشاهدة المشاركة

الاجابه فيييييييييييييييييييييييييييييييييييييييييييين
:sm170:

لسة بنجمع الاجابات............................

**ashraf shaq** · 12-09-2009, 10:17 PM

First of all this patient suffers severe bleeding since birth which means it is a congenital disease. Second it is a severe one which means that the screening tests should be abnormal if the defect in one or more of its members, and this is not the situation. Also there no medication taken, which means it is not drug induced or related. Does not suffer from any disease I mean here there is no collagen or vascular diseases and only confined to the hemostatic system.
So, Factor XIII deficiency is highly suggestive. The second suspecious one is alpha 2 antiplasmin. The third one is PAI-1.
So the strategy is first to do Factor XIII assay (not just the solubility test since this test is only abnormal when the factor activity is only less than 1 or 2%).
and then alpha 2 antiplasmin, then PAI.
Actually I have said it is a case that I have faced, and it was associated with severe umbilical bleeding, and a diagnosis of severe factor XIII deficiency was established.
Thank you to every one who participtated in opening his mind.
and thanks to Prof. Akram.
thank you very much.

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