I can not thank you enough for your lectures. They are very very helpful and you make learning fun and very easy to understand. Thank you so much for sharing your videos.
Sir, according to Robbins pathology, defective multimer assembly you mentioned is in type 2. And also it makes sense to me too. Since type 2 is qualitative defect.
Thank you for your feed back. The reference regarding types of vWD was taken from "Harsh Mohan Text Book of Pathology 6th Edition" that said in page number 336 : "Type I disease is the most common and is characterised by mild to moderate decrease in plasma vWF (50% activity).The synthesis of vWF is normal but the release of its multimers is inhibited. Type II disease is much less common and is characterised by normal or near normal levels of vWF which is functionally defective. Type III disease is extremely rare and is the most severe form of the disease. These patients have no detectable vWF activity and may have sufficiently low factor VIII levels." But as you have mentioned, in Robbins Type 2 is said to be due to defective multimer assembly. Again in Hoffbrand's Essential Hematology 7th Edition Page 295 : Type 1 is Quantitative partial deficiency, Type 2 is functional abnormality and type 3 is said to be complete deficiency. vWD type 2 is again further classified into 2A, 2B, 2M, 2N where multimers are absent,absent/reduced, normal and normal respectively. So since type 2 is not a single mechanism I think you are safe as long you follow a specific text book. Just give reference from the text book that is followed in your institution. Hope it is helpful. Take care and stay blessed. :)
***** Thank you very much for liking my videos. Very happy to know they are helpful. I have uploaded a new video on infarction few days back. You can check it out too ! Video Link : Infarction : Definition, Causes, Types, Morphology, Factors (HD) Take care and stay blessed. Best wishes from Bangladesh. :)
Thank u so much for sharing this. My daughter was recently diagnosed with vwd type 3. I’m also thinking of starting a TH-cam channel to raise awareness anyone interested?..
If I have to have any procedures I generally need to receive large amounts of whole plasma and blood transfusions. Why would this be???? I know I've a deficiency in VIII and XI & XII.
Sir... i am not getting the types of this disease especially type 1... they r synthesized normally and released into circulation but then which release is inhibited.. and I find ur types completely differs frm the robbin's text...
hello, I,m Ady, medical student from Indonesia. thank u so much doctor. this video really help me to understand this disease. i really enjoyed your explanation
The order of steps of platelet plug formation is : platelet activation, platelet adhesion and platelet aggregation.. But you've told platelet activation after platelet adhesion
According to Robbins & Cortan Pathologic Basis of Disease 9th Edition page 117 the sequence is 1. platelet adhesion 2. Shape change 3. release reaction (together shape change & release reaction are called platelet activation) 4. recruitment and 5. platelet aggregation . So my sequence is correct according to Robbins 9th Edition. Here is quote from page 117 : "After a traumatic vascular injury, platelets encounter constituents of the subendothelial connective tissue, such as vWF and collagen. On contact with these proteins, platelets undergo a sequence of reactions that culminate in the formation of a platelet plug (Fig. 4-4B). • Platelet adhesion is mediated largely via interactions with vWF, which acts as a bridge between the platelet surface receptor glycoprotein Ib (GpIb) and exposed collagen (Fig. 4-5). Notably, genetic deficiencies of vWF (von Willebrand disease, Chapter 14) or GpIb (Bernard-Soulier syndrome) result in bleeding disorders, attesting to the importance of these factors. • Platelets rapidly change shape following adhesion, being converted from smooth discs to spiky “sea urchins” with greatly increased surface area. This change is accompanied by alterations in glycoprotein IIb/IIIa that increase its affinity for fibrinogen (see later), and by the translocation of negatively charged phospholipids (particu larly phosphatidylserine) to the platelet surface. These phospholipids bind calcium and serve as nucleation sites for the assembly of coagulation factor complexes. • Secretion (release reaction) of granule contents occurs along with changes in shape; these two events are often referred to together as platelet activation. Platelet activation is triggered by a number of factors, including he coagulation factor thrombin and ADP. Thrombin acti vates platelets through a special type of G-protein coupled receptor referred to as a protease-activated receptor (PAR), which is switched on by a proteolytic cleavage carried out by thrombin. ADP is a component of dense- body granules; thus, platelet activation and ADP release begets additional rounds of platelet activation, aphenomenon referred to as recruitment. Activated plate lets also produce the prostaglandin thromboxane A2 (TxA2), a potent inducer of platelet aggregation. Aspirin inhibits platelet aggregation and produces a mild bleed ing defect by inhibiting cyclooxygenase, a platelet enzyme that is required for TxA2 synthesis. Although the phenomenon is less well characterized, it is also sus pected that growth factors released from platelets contribute to the repair of the vessel wall following injury. • Platelet aggregation follows their activation. The conformational change in glycoprotein IIb/IIIa that occurs with platelet activation allows binding of fibrinogen, a large bivalent plasma polypeptide that forms bridges between adjacent platelets, leading to their aggregation. Predictably, inherited deficiency of GpIIb-IIIa results in a bleeding disorder called Glanzmann thrombasthenia). The initial wave of aggregation is reversible, but concurrent activation of thrombin stabilizes the platelet plug by causing further platelet activation and aggregation, and by promoting irreversible platelet contraction. Platelet contraction is dependent on the cytoskeleton and consolidates the aggregated platelets. In parallel, thrombin also converts fibrinogen into insoluble fibrin, cementing the platelets in place and creating the definitive secondary hemostatic plug. Entrapped red cells and leukocytes are also found in hemostatic plugs, in part due to adherence of leukocytes to P-selectin expressed on activated platelets." Take care and stay blessed.
Dear Dr Haque, thank you for this informative video, how I wish you had been around to teach us at medical school! Keep up the good work may God bless. Khuda aap ko abaad rakhe amin. Dr Farah Ahmed
Hello Dr. Farah. Thank you for liking my videos. Glad to know it was helpful. More videos coming soon ! ( Next Upload " Emphysema" within 2 days). Take care & stay blessed.
I'm binge watching all ur videos now, before my examss,they are very helpful sir!
Thank you for your feedback. Take care and stay safe.
Excellence is visible all through. Nowhere one can find such clarity on a complex subject like von Willebrand's disease.👍🙏
Thank you sir for your feedback. Take care and stay safe. Best wishes from Bangladesh. :)
very informative and very very helpful...also very valuable informations
+Tofael Ahmed Thank you for liking my video. Glad to know it was helpful. Best wishes from Bangladesh. :)
I can not thank you enough for your lectures. They are very very helpful and you make learning fun and very easy to understand. Thank you so much for sharing your videos.
+MrJany82 Thank you for liking my video. Take care and stay blessed. Best wishes from Bangladesh. :)
I thank you for such wonderful videos
they are very much helpful and easy to understand.
Thanks
Thank you for liking my videos. Best wishes from Bangladesh. :)
Sir, according to Robbins pathology, defective multimer assembly you mentioned is in type 2. And also it makes sense to me too. Since type 2 is qualitative defect.
Thank you for your feed back. The reference regarding types of vWD was taken from "Harsh Mohan Text Book of Pathology 6th Edition" that said in page number 336 : "Type I disease is the most common and is characterised by mild to moderate decrease in plasma vWF (50% activity).The synthesis of vWF is normal but the release of its multimers is inhibited.
Type II disease is much less common and is characterised by normal or near normal levels of vWF which is functionally defective.
Type III disease is extremely rare and is the most severe form of the disease. These patients have no detectable vWF activity and may have sufficiently low factor VIII levels."
But as you have mentioned, in Robbins Type 2 is said to be due to defective multimer assembly.
Again in Hoffbrand's Essential Hematology 7th Edition Page 295 : Type 1 is Quantitative partial deficiency, Type 2 is functional abnormality and type 3 is said to be complete deficiency. vWD type 2 is again further classified into 2A, 2B, 2M, 2N where multimers are absent,absent/reduced, normal and normal respectively. So since type 2 is not a single mechanism I think you are safe as long you follow a specific text book. Just give reference from the text book that is followed in your institution. Hope it is helpful. Take care and stay blessed. :)
You made my life easier!! Thank you so much :)
***** Thank you very much for liking my videos. Very happy to know they are helpful. I have uploaded a new video on infarction few days back. You can check it out too ! Video Link : Infarction : Definition, Causes, Types, Morphology, Factors (HD) Take care and stay blessed. Best wishes from Bangladesh. :)
Thank you sir
Thank u so much for sharing this. My daughter was recently diagnosed with vwd type 3. I’m also thinking of starting a TH-cam channel to raise awareness anyone interested?..
Thank you for your feedback. Take care and stay blessed. :)
kis age me daignos huaa kya symtums the
Sir how we can differentiate Glanzmens T from VWB disease by platelet aggregation study
thnkuu so much
Allah Bless u olywzzz 💕
really u help me...thanQ Dr
+SrOiYaH Thank you for liking my video. Take care and stay blessed. Best wishes from Bangladesh. :)
informative video... keep it up
Thank you for liking my videos. Best wishes from Bangladesh. :)
EXCELLENT AS BEFORE ALWAYS CONCEPT CLEARING LECTURES.THANK YOU.DR.NISAR AHMAD PAKISTAN
Thank you for liking my video. Take care and stay blessed. Best wishes from Bangladesh. :)
If I have to have any procedures I generally need to receive large amounts of whole plasma and blood transfusions. Why would this be???? I know I've a deficiency in VIII and XI & XII.
Hi my family history all members have Von Willebrand disease factor 8. We can take Covid-19 vaccine or not.lot of confusion please help me.
Tnks a lot sir ❣️❣️
Thank you for your feedback. Take care and stay blessed.
There are people born to teach
Thank you for your feedback. Take care and stay blessed. More videos coming soon!
Thankyou sir... For making this video... I found it helpful!!!
What is the reasons to have a low vWF-CB and the rest normal or light normal? Or what does it mean.
Sir... i am not getting the types of this disease especially type 1... they r synthesized normally and released into circulation but then which release is inhibited.. and I find ur types completely differs frm the robbin's text...
Is it variably dominant or recessive depending on type of vwd right ?
Good
Thank you for your feedback. Take care and stay blessed.
I really enjoyed with your perfect explanation for lectures thank you so much doctor
We hope to continue 💓
Thank you for your feedback. Take care and stay blessed.
hello, I,m Ady, medical student from Indonesia. thank u so much doctor. this video really help me to understand this disease. i really enjoyed your explanation
+Andi Ashady Fitrah Pawallangi Thank you for liking my video. Take care and stay blessed. Best wishes from Bangladesh. :)
Sir vwB disease is clotting disorder or bleeding ??
Is there an explanation for bleeding desorder
There’s no S. vonWillebrand
Thankyou you for this concise explanation 🙌
Thank you for liking my video. Take care and stay blessed. Best wishes from Bangladesh. :)
I need your help in solving questions
Fantastic! Thank you so much.
Thank you for your feedback. Take care and stay blessed. :)
Every lectures r helpful 😍😍😍oh Allah...thank you soo soo soo soo much sir...make more sir for us kindly......
Thank you Dr. Rabiul I liked your vedious, I wanna you contact to ask information about departments of pathology.
von Willebrand is properly pronounced... von Villebrand
Excellent presentation.Would benefit many,keep doing
Thank you for your feedback. Take care and stay blessed. :)
nyc❤️✌️✌️
Thank you Dr Rabiul Haque ,am in South Africa ,I would like to know more about ristocetin test
- via YouPak(.com)
So helpful lecture thanks
Thank you so much! This is a great refresher for a nurse like me.
William Banaag Thank you for liking my videos. As always, best wishes from Bangladesh. :)
Very helpful. Your lecture helped me understand vwd
+Khudsi Tucker Thank you for liking my video. Glad to know it was helpful. Best wishes from Bangladesh. :)
The order of steps of platelet plug formation is : platelet activation, platelet adhesion and platelet aggregation.. But you've told platelet activation after platelet adhesion
According to Robbins & Cortan Pathologic Basis of Disease 9th Edition page 117 the sequence is 1. platelet adhesion 2. Shape change 3. release reaction (together shape change & release reaction are called platelet activation) 4. recruitment and 5. platelet aggregation . So my sequence is correct according to Robbins 9th Edition. Here is quote from page 117 :
"After a traumatic vascular injury, platelets encounter constituents of the subendothelial connective tissue, such as vWF and collagen. On contact with these proteins, platelets undergo a sequence of reactions that culminate in the formation of a platelet plug (Fig. 4-4B).
•
Platelet adhesion is mediated largely via interactions with vWF, which acts as a bridge between the platelet surface receptor glycoprotein Ib (GpIb) and exposed collagen (Fig. 4-5). Notably, genetic deficiencies of vWF
(von Willebrand disease, Chapter 14) or GpIb (Bernard-Soulier syndrome) result in bleeding disorders, attesting to the importance of these factors.
•
Platelets rapidly change shape following adhesion, being converted from smooth discs to spiky “sea urchins” with greatly increased surface area. This change is accompanied by alterations in glycoprotein IIb/IIIa that
increase its affinity for fibrinogen (see later), and by the translocation of negatively charged phospholipids (particu larly phosphatidylserine) to the platelet surface. These phospholipids bind calcium and serve as nucleation sites for the assembly of coagulation factor complexes.
•
Secretion (release reaction) of granule contents occurs along with changes in shape; these two events are often referred to together as platelet activation. Platelet activation is triggered by a number of factors, including he coagulation factor thrombin and ADP. Thrombin acti vates platelets through a special type of G-protein coupled receptor referred to as a protease-activated receptor (PAR), which is switched on by a proteolytic cleavage carried out by thrombin. ADP is a component of dense-
body granules; thus, platelet activation and ADP release begets additional rounds of platelet activation, aphenomenon referred to as recruitment. Activated plate lets also produce the prostaglandin thromboxane A2
(TxA2), a potent inducer of platelet aggregation. Aspirin inhibits platelet aggregation and produces a mild bleed ing defect by inhibiting cyclooxygenase, a platelet enzyme that is required for TxA2 synthesis. Although the phenomenon is less well characterized, it is also sus
pected that growth factors released from platelets contribute to the repair of the vessel wall following injury.
•
Platelet aggregation follows their activation. The conformational change in glycoprotein IIb/IIIa that occurs with platelet activation allows binding of fibrinogen, a large bivalent plasma polypeptide that forms bridges
between adjacent platelets, leading to their aggregation. Predictably, inherited deficiency of GpIIb-IIIa results in a bleeding disorder called Glanzmann thrombasthenia). The initial wave of aggregation is reversible, but concurrent activation of thrombin stabilizes the platelet plug by causing further platelet activation and aggregation, and by promoting irreversible platelet contraction. Platelet contraction is dependent on the cytoskeleton and consolidates the aggregated platelets. In parallel, thrombin also converts fibrinogen into insoluble fibrin, cementing the platelets in place and creating the definitive secondary hemostatic plug. Entrapped red cells and leukocytes are also found in hemostatic plugs, in part due to adherence of leukocytes to P-selectin expressed on activated platelets."
Take care and stay blessed.
@@RabiulHaque ok sir, thank you for the clarification
@@RabiulHaque And one more doubt sir, vW factor is produced by activated platelets right 🤔
Thank you for this video! would you mind to discuss further the different types of type 2 VWD? thanks again and more power!
if only my Lecturers can make it this simple. many thanks
Thank you for your feedback. Take care and best wishes.
Very informative thanku so much
Thank you for your feedback. Take care and stay blessed. :)
Respected sir
Doctor.. Do you have videos for lymphoma and leukemia?!
thanks a lot superb teaching sir...
Thank you for liking my videos. Best wishes from Bangladesh. :)
Thank.you.very.much.sir.
Thank you for your feedback. Take care and stay blessed. :)
Thank you
This was so useful, thank you so much!!
Thank you for liking my videos. Best wishes from Bangladesh.
I love this video... Thank you so much
Thank you for your feedback. Take care and stay safe. Best wishes from Bangladesh. :)
thanks Sir,so informative
Thank you for your feedback. Take care and stay blessed. :)
I have von willebrand’s disease 2b
magnificent lecture.
Thank you for liking my videos. Best wishes from Bangladesh. :)
excellent .many thanks
sherif saad Thank you for liking my videos. As always, best wishes from Bangladesh. :)
Excellent
Thank you for your feedback. Take care and stay safe.
Very very helpful.. Great explanation.. I watched almost all ur lectures of bleeding disorders.. And they are really very helpful.. Thank u soo much❤❤
Thank you for your feedback. Take care and stay blessed. :)
Dear Dr Haque, thank you for this informative video, how I wish you had been around to teach us at medical school! Keep up the good work may God bless. Khuda aap ko abaad rakhe amin. Dr Farah Ahmed
Hello Dr. Farah. Thank you for liking my videos. Glad to know it was helpful. More videos coming soon ! ( Next Upload " Emphysema" within 2 days). Take care & stay blessed.
thank u sir...
Thank you for your feedback. Take care and stay blessed. :)
thank u
+Nidhi Gupta Thank you for liking my video. Take care and stay blessed. Best wishes from Bangladesh. :)
Too much helpful video
Thank you for your feedback. Take care and stay safe.
Thanks.
+Khudsi Tucker Thank you for liking my video. Best wishes from Bangladesh. :)
I have type three severe
Nice one
Thank you for your feedback. Take care and best wishes.
thamk you
Thank you for your feedback. Take care and stay blessed.
thank you so much for the video....very informatve
Thank you for your feedback. Take care and stay blessed. :)
thank you very much, your lecture is superb
sorizai Thank you for liking my video. Very happy to know it was helpful. Take care & stay blessed. Best wishes from Bangladesh.
Are u an Indian ?
Thank you for your question. I am Bangladeshi. Take care and stay safe. :)
@@RabiulHaque yes 😊😊
Thnku ......
Thank you for your feedback. Take care and stay blessed. :)
this is just awesome
Thank you for your feedback. Take care and stay blessed. :)