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2120 Part XII: Hemostasis and Thrombosis Chapter 123: Hemophilia A and Hemophilia B 2121

Figure 123–10. Retroperitoneal pseudotumor.
A and B. Magnetic resonance imaging and com-
puted tomography scan of pseudotumor arising
from the iliopsoas muscle compressing the kid-
ney and other adjacent structures. Loculations
and calcifications can be seen. C. Gross specimen
after surgical removal, weighting approximately
6 pounds. D. Cross-section of pseudotumor shows
peripheral red hemorrhage, centrally caseified
blood and necrosis. Note the thick capsule that
surrounds the tumor.

A B

C D

Hemorrhage into the spinal canal is an uncommon neurologic bleeding at the surgical site. Bleeding may be delayed for several hours
complication in hemophilia, mostly related to trauma that can result or, occasionally, for several days. Surgery in such patients is character-
in paraplegia. Bleeding may occur within the spinal cord itself, but epi- ized by delayed wound healing because of poor clot formation. Pro-
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dural bleeding compressing the cord is more common. longed bleeding and subsequent infection of the wound hematoma may
Peripheral nerve compression is a frequent complication of muscle further complicate healing. Appropriate factor VIII replacement ther-
hematomas, particularly in the extremities. Compression of the femoral apy, sometimes supplemented by anti-fibrinolytic agents, can prevent
nerve by a hematoma in the iliopsoas muscle can result in sensory loss intraoperative and postoperative hemorrhages.
over the lateral and anterior thigh, weakness and atrophy of the quad- Dental extraction is the most frequent surgical procedure per-
riceps, and loss of the patellar reflex. The ulnar nerve is the next most formed on hemophilic patients. Loss of deciduous teeth seldom causes
frequently involved peripheral nerve. Bleeding may occur in any muscle excessive bleeding, but extraction of permanent teeth may result in
and may compress local neural blood supply. This situation can be fol- excessive hemorrhage that can persist intermittently for several days to
lowed by permanent neuromuscular defects and multiple contractures. weeks unless appropriate treatment is administered. In the untreated
patient with severe hemophilia, life-threatening, dissecting pharyngeal
Mucous Membrane Hemorrhage and/or sublingual hematomas may result from dental procedures or
Mucous membrane bleeding is common in hemophilia. Epistaxis and from administration of regional block anesthesia.
hemoptysis, often resulting from allergic reactions or trauma, can be
associated with local structural lesions involving the upper and/or lower
respiratory tract. Treatment of epistaxis by cautery or nasal packing LABORATORY FEATURES
sometimes is followed by recurrent bleeding because of sloughing of the
cauterized area or dislodging of a poorly formed clot when the pack- Patients with severe hemophilia A have a prolonged activated par-
ing is removed. Gastrointestinal hemorrhage has a 1.3 percent annual tial thromboplastin time (aPTT). The prothrombin time (PT) and
incidence and is mostly associated to older age and complications of thrombin clotting time (TCT) are normal. Different combinations of
advanced liver disease. Ingestion of antiinflammatory drugs for relief of aPTT reagents and instrumentation exhibit varying sensitivities to fac-
pain of hemophilic arthropathy is a frequent cause of upper gastrointes- tor VIII levels. In mild hemophilia, the aPTT may be only slightly pro-
tinal hemorrhage, and a history of ingestion of aspirin and other anti- longed or at the upper limit of normal, especially if factor VIII activity
inflammatory drugs should be specifically addressed (and proscribed) is 20 percent or greater. The aPTT is corrected when hemophilic plasma
when assessing the etiology of such bleeding. 38 is mixed with an equal volume of normal plasma. If the hemophilic
plasma contains an anti-factor VIII inhibitor antibody, the aPTT on
Dental and Surgical Bleeding a similar mixture is prolonged, but incubation of the mixture for 1 or
Hemophilic patients are treated with clotting factor preoperatively and 2 hours at 37°C is sometimes required to detect the prolongation.
postoperatively to prevent bleeding. Mildly or sometimes moderately A definitive diagnosis of hemophilia A should be based on a specific
affected patients may go unrecognized until surgery results in excessive assay for factor VIII activity.

Kaushansky_chapter 123_p2113-2132.indd 2120 9/21/15 4:36 PM