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Life-threatening hemorrhage from acquired hemophilia A as a presenting manifestation of prostate cancer

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ABSTRACT

Acquired factor VIII deficiency (acquired hemophilia A) is a rare condition characterized by the acquisition of autoantibodies that affect the clotting activity of factor VIII (fVIII). The most common manifestation in affected patients is a hemorrhagic diathesis. This disorder is associated with autoimmune diseases, pregnancy, postpartum period, drugs, and malignancy. Management of this condition begins with attempts to arrest an acute bleed based on the site and severity of bleeding and inhibitor titer. The next priority is eradication of the fVIII antibodies using immunosuppressive therapies. We report the case of a 66-year-old male who presented with spontaneous right thigh hematoma with prolonged activated partial prothrombin time and normal prothrombin time. Mixing studies confirmed the presence of an inhibitor. Further investigation for the underlying etiology of acquired hemophilia A leads to diagnosis of prostate cancer. Treatment consisted of bypassing agents including activated factor VII and activated prothrombin plasma concentrate to arrest the bleeding. Steroids and cyclophosphamide were added to suppress the fVIII inhibitors. Concomitant treatment of locally advanced prostate cancer with chemotherapy confirmed the eradication of the inhibitors. To our knowledge, this is the first reported case of prostate cancer diagnosed and treated simultaneously with acquired hemophilia A resulting in favorable patient outcome.

No MeSH data available.


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Coagulation cascade. Factor VIII inhibitor blocking intrinsic pathway (shown by black box); mechanism of action of recombinant activated factor VII (shown by yellow blocks), which binds with activated platelets to activate factor X and generate factor Xa; activated prothrombin complex concentrate contains activated factor VII and inactivated factors II, IX, and X (shown by the symbol ★).
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Figure 0003: Coagulation cascade. Factor VIII inhibitor blocking intrinsic pathway (shown by black box); mechanism of action of recombinant activated factor VII (shown by yellow blocks), which binds with activated platelets to activate factor X and generate factor Xa; activated prothrombin complex concentrate contains activated factor VII and inactivated factors II, IX, and X (shown by the symbol ★).

Mentions: Hemostasis can be achieved by two approaches: the use of bypassing agents or raising fVIII level depending on the site and severity of bleeding. Two bypassing agents, rfVIIa and aPCC, have been used as first-line treatment. rfVIIa binds directly with activated platelets to activate factor X to produce factor Xa, activating common pathway, while aPCC contains four different coagulation factors, mostly activated factor VII and inactivated factors II, IX, and X (14, 15). Both products bypass the need for fVIII and activate common pathway to generate clot formation (Fig. 3). Retrospective studies have shown the overall efficacy rate for rfVIIa at 88% and for aPCC around 86% (16, 17). Patients with life-threatening bleeding and a very high titer level should be managed by rfVIIa or aPCC. In our patient, we were unable to achieve adequate hemostasis with rfVIIa; therefore, aPCC was used successfully to attain complete hemostasis (16–19).


Life-threatening hemorrhage from acquired hemophilia A as a presenting manifestation of prostate cancer
Coagulation cascade. Factor VIII inhibitor blocking intrinsic pathway (shown by black box); mechanism of action of recombinant activated factor VII (shown by yellow blocks), which binds with activated platelets to activate factor X and generate factor Xa; activated prothrombin complex concentrate contains activated factor VII and inactivated factors II, IX, and X (shown by the symbol ★).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5016740&req=5

Figure 0003: Coagulation cascade. Factor VIII inhibitor blocking intrinsic pathway (shown by black box); mechanism of action of recombinant activated factor VII (shown by yellow blocks), which binds with activated platelets to activate factor X and generate factor Xa; activated prothrombin complex concentrate contains activated factor VII and inactivated factors II, IX, and X (shown by the symbol ★).
Mentions: Hemostasis can be achieved by two approaches: the use of bypassing agents or raising fVIII level depending on the site and severity of bleeding. Two bypassing agents, rfVIIa and aPCC, have been used as first-line treatment. rfVIIa binds directly with activated platelets to activate factor X to produce factor Xa, activating common pathway, while aPCC contains four different coagulation factors, mostly activated factor VII and inactivated factors II, IX, and X (14, 15). Both products bypass the need for fVIII and activate common pathway to generate clot formation (Fig. 3). Retrospective studies have shown the overall efficacy rate for rfVIIa at 88% and for aPCC around 86% (16, 17). Patients with life-threatening bleeding and a very high titer level should be managed by rfVIIa or aPCC. In our patient, we were unable to achieve adequate hemostasis with rfVIIa; therefore, aPCC was used successfully to attain complete hemostasis (16–19).

View Article: PubMed Central - PubMed

ABSTRACT

Acquired factor VIII deficiency (acquired hemophilia A) is a rare condition characterized by the acquisition of autoantibodies that affect the clotting activity of factor VIII (fVIII). The most common manifestation in affected patients is a hemorrhagic diathesis. This disorder is associated with autoimmune diseases, pregnancy, postpartum period, drugs, and malignancy. Management of this condition begins with attempts to arrest an acute bleed based on the site and severity of bleeding and inhibitor titer. The next priority is eradication of the fVIII antibodies using immunosuppressive therapies. We report the case of a 66-year-old male who presented with spontaneous right thigh hematoma with prolonged activated partial prothrombin time and normal prothrombin time. Mixing studies confirmed the presence of an inhibitor. Further investigation for the underlying etiology of acquired hemophilia A leads to diagnosis of prostate cancer. Treatment consisted of bypassing agents including activated factor VII and activated prothrombin plasma concentrate to arrest the bleeding. Steroids and cyclophosphamide were added to suppress the fVIII inhibitors. Concomitant treatment of locally advanced prostate cancer with chemotherapy confirmed the eradication of the inhibitors. To our knowledge, this is the first reported case of prostate cancer diagnosed and treated simultaneously with acquired hemophilia A resulting in favorable patient outcome.

No MeSH data available.


Related in: MedlinePlus