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Bleeding in 75-YO With CLL

Here is a case sent in by a colleague. A 75 YO woman with CLL. In April, 2014, she had a prolonged PTT but was not worked up. She recently presented to an outside hospital with weakness and falls and was found to have a HGB of 5.1 g/dL and a PTT of 90 seconds. She was transferred to our institution and found to have a retroperitoneal hematoma by CT. She was transfused with RBCs, FFP, and platelets and is currently hemodynamically stable.

Initial coag studies on admission:

  • PT: 12.9 (RI 9.5–12.3 sec)
  • PTT: 58 (RI 26–37 sec)
  • PTT 50/50 mix: 64 (no incubation)
  • DRVVT/LA confirm: 42.6/36.1, negative
  • Factor VIII: initially was 3% when using lupus insensitive reagent, was able to obtain 100% with dilution
  • All other factors normal or just slightly low, however there was a non-specific inhibitor to IX, XI, and XII
  • Bethesda for factor VIII: 182.4 BU; using lupus insensitive reagent, still around 140 BU

Follow-up labs will be posted July 30. What is your diagnosis?

Comments (3)
Coagulation Factors
Jul 25, 2014 7:32pm

Looks like a fairly straight forward case of acquired hemoph
Looks like a fairly straight forward case of acquired hemophilia caused by a strong FVIII inhibitor. Although you cannot rule out a (weak) LA, the high relative inhibitory activity against FVIII (140-180 BU) vs minor inhibition of the other factors argues in favor of a strong FVIII inhibitor, which is also consistent with the clinical presentation. I continue to be amazed that clinicians order routine coags, the results come back clearly abnormal, but this is not worked up until a serious clinical event. Why ask for the tests if you are not planning to follow up abnormal results? Many cases of acquired hemophilia ‘smolder’ without consequence until some event precipitates a bleed, and then the inhibitor leads to failure of clot formation and serious bleeding.

Jul 25, 2014 10:43am

More cases of simultaneous FVIII inhibitors and LA in full t
More cases of simultaneous FVIII inhibitors and LA in full text:
An unusual etiology for elevation of activated partial thromboplastin time (aPTT) in SLE: Acquired Hemophilia and Lupus Anticoagulant.
Acquired factor VIII inhibitor and lupus anticoagulant presenting with prolonged
aPTT: a case report:

A detailed review was recently published: Prolonged activated partial thromboplastin time: difficulties in discriminating coexistent factor VIII inhibitor and lupus anticoagulant:

Jul 25, 2014 10:38am

Hello, my guess is acquired FVIII deficiency (hemophilia A),
Hello, my guess is acquired FVIII deficiency (hemophilia A), that rarely occurs in patients with blood cancers including CLL; non-specific inhibitor pattern and inconsistent level of BU might be due to lupus anticoagulant, that more affects APTT-based assays but less DRVVT (or elevated CRP that behaves as LA in APTT-based assay but not DRVVT). However, it was reported previously that sometimes (3 out of 12 or 25%) in case of simultaneous FVIII inhibitors and LA occurance “…prolonged DRVVT did not shorten with PNP sufficient to be considered positive…” from “Simultaneous Occurrence of Lupus Anticoagulant and Factor VIII Inhibitors in Hemophilia” full text link:

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