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Mix Corrects with NP, But not with Deficient Plasmas

From “Japgi,” I have a 6 year old boy, well till a month back, when he had a post-traumatic gum bleed. The bleed was stopped after about half an hour, but a small ooze restarted and stayed for 3 days. His prothrombin time (PT) is 14 seconds (RI 11–16), activated partial thromboplastin time (APTT, PTT) is 46.4 seconds (RI 30–40). The prolonged PTT corrected with normal plasma 1:1 is 38.1 seconds. There was no correction with 1:1 mix of factor VIII-deficient plasma, 43.1 seconds; factor IX-deficient plasma, 43.1; or factor XI-deficient plasma, 41.1. The entire exercise was performed on two separate samples a week apart. There is no history of bleeding in the family, transfusions. After the first test the patient has been running a cold with fever. What is this and what can we do to confirm? Thank fully there are no further bleeds.

Hello, and thank you for your question. The results of your factor VIII-deficient, factor IX-deficient, and factor XI-deficient plasma mixes leads me to suggest there may be a lupus anticoagulant (LA) present even though the prolonged PTT appears to correct with normal plasma. It is unlikely the boy has deficiencies of all three factors. This is not unusual; there are examples of weak LAs that fail detection in immediate mixing studies. In childhood, as in adulthood, weak, transient LAs may be detected as a side-effect of acute viral infection. To follow up, I suggest a full LA profile, including DRVVT confirmation, at least 12 weeks subsequent to the last assay that was performed.

Of course, the possibility of an LA does not explain the bleeding. Assuming he has a chronic bleeding condition, I suggest a platelet count and perhaps platelet aggregometry, if available, to search for a platelet defect. The “small ooze’ description suggests the possibility of a mild platelet defect or thrombocytopenia.

If you are able to repeat the assays after 12 weeks, please send us the information, it will be interesting and helpful to continue to follow your patient and his laboratory results. Meanwhile, I anticipate some of our participants may provide additional recommendations.

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