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Ehlers-Danlos Syndrome

A question about Ehlers-Danlos syndrome from Dr. John McClure:
I was asked by a hematology colleague about bleeding risk in an Ehlers-Danlos patient.  He was consulted for a preoperative assessment.  The patient has a “self-reported” history of bleeding but has undergone uneventful lap-chole and vaginal deliveries. My colleague is specifically asking about the PFA 100 to screen for platelet functional defects. I’m not sure how to direct him.  I predict that her risk is “pretty low” based on history.  I also guess that the bigger risk in these patients is vascular integrity which is largely beyond our control. Any thoughts on preoperative screening for these patients?  What test?  Worthwhile?  Thoughts on a plan B if surgery becomes problematic? Thanks, John McClure. Read more »

Case Study: 26 YO Female with Chronic Bleed

Here is a case sent by Ashli Jarrell, an articulation student in the University of Cincinnati online CLS program. I will leave the answer open for a few days to get your opinion:

Hi, George.

I have an interesting case: 26 YO female. History: nosebleeds since age 3; age 5 tonsillectomy with increased bleeding post-op; age 7 admitted to hospital for bruising and nosebleeds, lymphocytic leukemia negative; required transfusions after auto accident and labor. Began menstruation at age 12 with periods lasting three weeks. At times requires FFP to control bleeding. Family history: Mom and dad are living and well; brother and sister living with no bleeding problems. Lab results: PT 23 s; PTT >200 s; TT 21 s; fibrinogen 250 mg/dL; mixed PT 11 s; mixed PTT 28 s.

Please help!
Thanks, Ashli

Please select “comment” to respond. Geo.

PFA-100 Prolonged CTs in Children

Here is a new stumper from Kim Kinney at Clarian in Indianapolis:

Hi, George. We perform many PFA-100s on both adults and children.  We have started seeing a pattern only in children.  Approximately six times in the past few months, we have seen abnormal EPI and ADP closure times with totally normal platelet aggregations.  We check all of our abnormal PFAs for platelet count, hematocrit and hemolysis.  All of these cases have had no abnormalities.  On a few cases, we have also done von Willebrand antigen, ristocetin cofactor and factor VIII levels.  All of these have been normal.  Could these be cases of mild von Willebrand disease that we cannot pick up with our traditional methods, but the PFA is picking up the abnormality?

Kim, thank you for the question, and let’s look for some answers from other PFA-100 users. Geo

Bleeding and Elevated Factor VIII

Our coagulation lab specialists at UAB have noted anecdotally that long term bleeding is accompanied by elevated factor VIII activity levels. Have others seen this, and is there a reference documenting it? Geo.

D-dimer and FDPs

Here are the results for our 12/11/08 quick question, “What assay do you use to detect abnormalities in fibrinolysis such as DIC? Read more »

Heparin Therapeutic Range

Here is a question on how to develop the PTT therapeutic range using the Brill-Edwards method in a low-volume facility. This is from Michael D. Blechner, MD, Assistant Professor of Pathology and Laboratory Medicine, University of Kentucky Medical Center:

Here at UK (University of Kentucky) we use plenty of unfractionated heparin (UFH) and it is relatively easy to identify 30+ specimens from patients on UFH with which to establish a PTT range by comparing the PTT values with anti-Xa results.
UK has recently purchased a smaller hospital across town and I have been consulted about how to establish their RR when they switch PTT reagent lots. There is very little UFH used there, making it very difficult to identify enough patients to compare PTT and anti-Xa in ex vivo samples. What is the recommended approach? Spiking? Thanks for any help.
Mike Blechner

By the way, I found a post from you from 1997 while searching the MEDLAB-L listserv on this topic… Read more »

Overuse of Platelet Concentrate

Here is an interesting finding presented yesterday at the ASH meeting detailing apparent overuse of platelet concentrate:

Click Here for link to article

When to Use the Chromogenic X Assay

A question about the chromogenic factor X assay from Eryn Cramer:

I am working as a student in an anticoagulation clinic and we are looking for information on the frequency of chromogenic factor X (CFX) monitoring before returning to INR monitoring for patients with antiphospholipid syndrome (APS).  Are there any recomendations for CFX test frequencies?  Also, it appears that the CFX varies depending on the reagents used.  How does that allow for a correlation to the INR, since INR is standardized?

I sent Eryn’s question to Dave McGlasson, who has published a great deal of research on the CFX: Read more »

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