The investigations show adequate VWF levels and therefore exclude VWD. It is shocking that this patient was not managed immediately in an urgent/emergency fashion for severe anemia with intravenous iron and rapid assessment by obgyn (5 days is too much). Furthermore, this patient may have bled enough to be in DIC and consumptive coagulopathy and developed VTE as a consequence. The link between elevation in factor 8 and VTE is weak as it does not accurately predict recurrence or first thrombosis.
There has been some population level data to suggest it may predict recurrence but that evidence hasn’t been reproduced to my knowledge. Being an acute phase reactant, factor 8 levels are useless in diagnosis and follow up in my opinion and do not change management. One caveat, is that recently an Italian family with recurrent VTEs were described with a duplication of the factor 8 gene and extremely elevated levels - but this is exceedingly rare.
Feb 7, 2023·edited Feb 8, 2023Liked by Med Mal Reviewer
I'm a hematologist and I share the opinion on weak and undisclosed connection between elevated VIII and thrombosis risk. I also have a feeling that knowing the test results would not change approach of a gyncologist as there would be no evidence based argument to witheld therapies to control bleeding in such serious prolonged major bleeding episode. It is evident that serious bleeding diathesis was present despite elevated factor VIII levels which is also somewhat contradictory with the view that she had inherited pro-thrombotic condition (however possible). I treated a young female patient with VTE that developed proximal leg thrombosis due to untreated mioma of uterus due to compressive effect of the tumor. We do not know what was the cause of such serious vaginal bleeding in the described case.
I'm wondering what the CBC's Hgb/Hct showed, I've seen the POC Hgb be wildly off. Either way yes, the pediatrician would either have to get a stat CBC or send the patient to the ED to determine the need for transfusion. Agree with everyone's sentiment about needing to understand why the patient was bleeding so much. Would need to see the patient's other risk factors for DVT as well.
Glad to see a hematologist comment. Thanks! I came to comment re: transfusion that depending on her symptoms and her young age, she probably would have been a candidate for iron infusion instead of blood transfusion despite her Hgb of 6. Given today’s EMRs, it’s unfortunate that the Ob/Gyns did not have access to her results, although, honestly, what would they have done with them? Was there ever an U/S ordered? We still don’t really know why she was bleeding so heavily. Polyp(s), fibroid(s), bizarre AVM, thyroid issues, PCOS, cancer, etc etc.
Hematologist here, thanks for this case.
The investigations show adequate VWF levels and therefore exclude VWD. It is shocking that this patient was not managed immediately in an urgent/emergency fashion for severe anemia with intravenous iron and rapid assessment by obgyn (5 days is too much). Furthermore, this patient may have bled enough to be in DIC and consumptive coagulopathy and developed VTE as a consequence. The link between elevation in factor 8 and VTE is weak as it does not accurately predict recurrence or first thrombosis.
There has been some population level data to suggest it may predict recurrence but that evidence hasn’t been reproduced to my knowledge. Being an acute phase reactant, factor 8 levels are useless in diagnosis and follow up in my opinion and do not change management. One caveat, is that recently an Italian family with recurrent VTEs were described with a duplication of the factor 8 gene and extremely elevated levels - but this is exceedingly rare.
I'm a hematologist and I share the opinion on weak and undisclosed connection between elevated VIII and thrombosis risk. I also have a feeling that knowing the test results would not change approach of a gyncologist as there would be no evidence based argument to witheld therapies to control bleeding in such serious prolonged major bleeding episode. It is evident that serious bleeding diathesis was present despite elevated factor VIII levels which is also somewhat contradictory with the view that she had inherited pro-thrombotic condition (however possible). I treated a young female patient with VTE that developed proximal leg thrombosis due to untreated mioma of uterus due to compressive effect of the tumor. We do not know what was the cause of such serious vaginal bleeding in the described case.
I'm wondering what the CBC's Hgb/Hct showed, I've seen the POC Hgb be wildly off. Either way yes, the pediatrician would either have to get a stat CBC or send the patient to the ED to determine the need for transfusion. Agree with everyone's sentiment about needing to understand why the patient was bleeding so much. Would need to see the patient's other risk factors for DVT as well.
Glad to see a hematologist comment. Thanks! I came to comment re: transfusion that depending on her symptoms and her young age, she probably would have been a candidate for iron infusion instead of blood transfusion despite her Hgb of 6. Given today’s EMRs, it’s unfortunate that the Ob/Gyns did not have access to her results, although, honestly, what would they have done with them? Was there ever an U/S ordered? We still don’t really know why she was bleeding so heavily. Polyp(s), fibroid(s), bizarre AVM, thyroid issues, PCOS, cancer, etc etc.