Here is another thought-provoking email from Dr. Jay Lozier at the NIH. I need some expert help with this one.
From Dr. Lozier: “I am in the position of hematology consultant who runs the coag lab. We see some interesting cases from time to time. I see a fair amount of storage pool deficiency that we diagnose on the basis of aggregations and electron micrographs to confirm.
“Just diagnosed a dysfibrinogenemia that manifests itself as an isolated prolonged prothrombin time (PT) that did not correct with a mix. Fibrinogen activity (Stago, Clauss method) was 87 mg/dL, but fibrinogen antigen was >400 mg/dL (Mancini radial immunodiffusion). Had not seen that presentation before; no bleeding.
Might be a good post/question to put out there: the PT that doesn’t correct with a mix…”
Jay Lozier, MD, PhD, FACP
NIH Clinical Center Department of Laboratory Medicine
Thank you, Dr. Lozier, and yes, this is a good question to put out there. Why should a dysfibrinogenemia fail to correct in a PT mixing study? I’m looking for an answer out there!
And while we are at it, let’s also go into more detail on typical aggregation patterns in storage pool deficiency, and what sorts of patients present this way. I’d like to see some real-life examples, as I hope they support the stuff we write in textbooks. Geo.