8/13/2019 20070814 s 00012 p 341

1/2

Practice

The case: An 85-year -old man pre-

sented with bruising of his hands,which he first noticed after shovellingsnow 3 days earlier. His past medicalhistory included benign prostatic hy-pertrophy and glaucoma. He was nottaking anticoagulants or nonsteroidalanti-inflammatory drugs. Over the last3 days the bruising had become exten-sive, encompassing the palmar anddorsal aspect of both hands andspreading to the lower forearms (Fig-ure 1). The patient had no other bruis-ing, nosebleeds, hematuria, bloodystools or hemoptysis, and he reportedhaving no joint or muscle pains. He didnot have a history of liver disease, nordid he have any personal or family his-tory of bleeding or clotting disorders.

On examination, the patient lookedwell and was afebrile, and his vitalsigns were stable. Aside from the ex-tensive bruising of both hands, noother bruising, petechiae or sites of ac-tive bleeding were discovered, andthere was no evidence of hemarthrosis.

Initial laboratory investigations re-vealed a normal complete blood count,with a hemoglobin level of 130 g/L, aplatelet count of 199 109/L and nor-mal electrolyte levels. Kidney and liverfunction were normal, as was the bloodglucose level. The D-dimer level wasslightly elevated, at 250500 (normal

< 250) ng/mL. The prothrombin time

and international normalized ratiowere normal; however, the activatedpartial thromboplastin time was ele-vated, at 117 (normal < 35) seconds. A1:1 mixing assay initially showed a cor-rected activated partial thromboplastintime of 41 seconds; however, a time-delayed 1:1 mix could not correct thethromboplastin time, which suggestedthat clotting factor inhibitors werepresent in the patients blood. Becauseonly the activated partial thromboplas-tin time was affected, we assayed forclotting factors specific to the intrinsicpathway and determined that the pa-tient had a factor VIII deficiency (titre< 0.01 [normal 0.51.5] U/mL). Furtherassays revealed factor VIII inhibitors inthe patients serum, at a level of 12.0(normal 0) Bethesda units. Acquiredhemophilia was diagnosed.

The patient was admitted to hospi-tal and given oral prednisone therapy(60 mg/d). Because no sites of activebleeding were identified, no additional

treatment was initiated. His activatedpartial thromboplastin time graduallyimproved, and no further bleeding orbruising occurred. He was dischargedhome 4 days later and given a taperedcourse of prednisone.

One month after the patient com-pleted the course of prednisone, his ac-

tivated partial thromboplastin time wasagain prolonged. He required a com-bined course of cyclophosphamide andprednisone. His condition is currentlymaintained on 50 mg of cyclophospha-mide daily, with a normal activatedpartial thromboplastin time and no fur-

ther bleeding.Investigations into the cause of this

patients acquired hemophilia includedchest radiograph, computed tomogra-phy of the chest and abdomen, andblood work to rule out malignant or au-toimmune diseases. Findings were nor-mal, and the acquired hemophilia wasassumed to be idiopathic in nature.

Acquired hemophilia A is rare, with anestimated annual incidence of 1 permillion people. This potentially life-threatening hemorrhagic disorder iscaused by the development of autoanti-bodies to factor VIII of the coagulationcascade. Its distribution is bimodal, withthe first peak occurring among youngwomen in the postpartum period andthe second among elderly patients, inwhom it is frequently associated withautoimmune disease, malignant diseaseand allergic drug reactions (Box 1).

The pattern of bleeding in acquired

hemophilia differs from that in congeni-tal hemophilia: bleeding tends to occurin soft tissue, muscle, the retroperitonealspace and the gastrointestinal and geni-tourinary tracts. Unlike in congenitalhemophilia, hemarthrosis is rare. Alongwith severe bleeding, patients may expe-rience complications such as compart-

Acquired hemophilia A presenting in an elderly man

Teaching Case Report

DOI:10.1503/cmaj.070168

Figure 1: Hands of elderly man showing bruising on palmar and dorsal aspects of both hands that extended to forearms.

@ See related article page 339

CMAJ August 14, 2007 177(4) 2007 Canadian Medical Association or its licensors

341

8/13/2019 20070814 s 00012 p 341

2/2