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Suggest treatment for symptoms of inherited platelet disorder

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Posted on Mon, 25 Jul 2016
Question: Ideas on managing or working up this bleeding disorder? I've had 8 surgeries including one recent all with some bleeding complication but prior work-ups for VWF, basic factor screening levels, PT/PTT all normal but an abnormal TEG years ago, see attached image. My Hg/Hct and plts run high normal. Life-long bleeding bleeding symptoms. Extensive family history of bleeding problems as well including mortality X2. Planning on seeking detailed work-up again after this surgical intervention but since that will take another long referral process I'd love any input or other ideas. Thanks.
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Answered by Dr. Hardik Sanghvi (7 hours later)
Brief Answer:
Find detail answer below.

Detailed Answer:
Hi, dear
I have gone through your question.
I can understand your concern.

You have history of bleeding after surgery.

Your basic factor screening level, vWF, PT, APTT all are normal. More over you have extensive family history of bleeding tendency.

You may have some inherited hemostasis problem.
Like platelet function disorder or hypofibrinogenemia or some other disorder.

Please provide me following details.

Do you have any other bleeding complication without surgery?

Do you have any patechia or mucosal bleeding?

Do you have any history of joint bleeding?

Do you have any history of blood thinner medicine.?

Do you have any history of spontaneous bleeding or bleeding after trauma.

Please send me your all reports including complete blood count and coagulation profile reports, liver function test and others.

Please send me your all detail history and reports. So that I can help you further. Thanks for using health care magic. Wish you a very good health.
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
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Follow up: Dr. Hardik Sanghvi (59 minutes later)
Thanks for your reply. In regards to your questions: Yes, I have a history of prolonged nosebleeds, heavy menses, a hemorrhagic ovarian cyst requiring surgery, easy bruising.
No joint bleeding but some chronic pains.
NEVER any blood thinner or herbal supplements that cause bleeding.
The only spontaneous bleeding would be nosebleeds/hemorrhagic ovarian cyst and just bad bruising w trauma, never experienced serious trauma, thank goodness!
My sister died from a massive hemorrhagic stroke after becoming coagulopathic post lap cholecystectomy 3 yrs ago. My maternal Grandmother died from a hemorrhagic stroke around age 40. My mother hemorrhaged severely after a normal delivery, in fact pronounced dead but then survived.
I have no lab reports w me at this time as I'm in a new state abd having surgery explorationin morning for bleeding after recent breast surgery. I can tell you my liver function was last checked in fall and normal. My Hg runs around 15, plts around 350. Otherwise I hope you were able to see my old thromboelastogram attached to original question.
Just hoping the more people I ask the greater the chances someone can help me get to the bottom of this.
doctor
Answered by Dr. Hardik Sanghvi (5 hours later)
Brief Answer:
Most likely to be platelet function disorders.

Detailed Answer:
Hi, dear
I have gone through your question.
I can understand your concern.

As you don't have any reports. It is very difficult to say exact cause but still I will try my level best to reach the probable cause from your details.

You have strong family history of bleeding. It suggest inherited disease.

Your factory study and Pt, APTT are normal so we can rule out all clotting factor associated disorder.

You don't have any drug history and liver disease.

You have mucosal bleeding, nosebleeding, bruising after trauma, menorrhagia, and post surgical bleeding and abnormal TEG.

These all findings suggest some platelet disorder.

Your platelet count is high so it is most likely platelet function disorder.

It can be BERNARD-SOULIER syndrome, Glanzmann's thrombasthenia or some other.

If you are in my clinic I suggest you to go for following reports.

Bleeding time, Peripheral smear examination for platelet aggregation.
Platelet functional study including aggregation to ADP, colloagen & ristocetin, clot retraction study and platelet adhasion study.
These will give you exact cause of your bleeding.

Then you should take treatment according to cause.

Hope I have answered your question. If you have still any doubts then feel free to ask me. I will be happy to answer. Thanks for using health care magic. Wish you a very good health.

Please give me follow up.


Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
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Answered by
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Dr. Hardik Sanghvi

Hematologist

Practicing since :2008

Answered : 7043 Questions

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Suggest treatment for symptoms of inherited platelet disorder

Brief Answer: Find detail answer below. Detailed Answer: Hi, dear I have gone through your question. I can understand your concern. You have history of bleeding after surgery. Your basic factor screening level, vWF, PT, APTT all are normal. More over you have extensive family history of bleeding tendency. You may have some inherited hemostasis problem. Like platelet function disorder or hypofibrinogenemia or some other disorder. Please provide me following details. Do you have any other bleeding complication without surgery? Do you have any patechia or mucosal bleeding? Do you have any history of joint bleeding? Do you have any history of blood thinner medicine.? Do you have any history of spontaneous bleeding or bleeding after trauma. Please send me your all reports including complete blood count and coagulation profile reports, liver function test and others. Please send me your all detail history and reports. So that I can help you further. Thanks for using health care magic. Wish you a very good health.