Page 2264 - Hematology_ Basic Principles and Practice ( PDFDrive )

Chapter 135 Hemophilia A and B 2011

Hip Joint Bleeds Hemophilic Pseudotumors
Hemorrhage into the hip joint is uncommon compared with other joints. Pseudotumors are a rare but very problematic complication in hemo-
However, because the clinical features of hip bleeds are less distinctive philia. The most common type of pseudotumor arises as a result of
than those of more exposed joints, it is possible that the incidence of repeated hemorrhages into a muscle with insufficient resorption of
hip bleeding is underestimated. Patients with a hip bleed maintain the blood between hemorrhages. Pseudotumors become walled-off cystic
joint in a partially flexed position, the position of lowest pressure. This structures surrounded by a fibrous membrane. They may become
position is similar to that seen in patients with an iliopsoas muscle multivacuolated over time, and parts may become calcified. These
bleed, causing these entities to be confused. cystic lesions frequently expand into adjacent structures, leading to
The management of acute hemarthrosis of the hip joint is somewhat their destruction. Skeletal fractures and bony deformities may arise
different from that of other joints because of the vascular anatomy of from such lesions. Another and rarer type of pseudotumor, generally
the hip joint, which renders the head of the femur vulnerable to ische- only seen in adult patients, arises from within the bone itself and is
mia in the context of a bleed, causing raised intraarticular pressure. often secondary to subperiosteal bleeding. This type of pseudotumor
Pain in the hip joint region may be caused by a range of conditions (hip is typically observed in the long bones of the lower extremities and in
joint bleed, iliopsoas muscle bleed, bleeds into surrounding muscles, the pelvis. Pseudotumors arising distally are more common in young
retroperitoneal bleed, and appendicitis). Consequently, without children and most often occur in the hand. Pseudotumors may be
appropriate imaging, a hip joint bleed may be easily misdiagnosed. associated with pain from rapid growth or nerve compression.
Ultrasonography remains the preferred modality for investigation of Pseudotumors are usually diagnosed by radiologic means (ultra-
hip pain because plain radiographs lack sufficient sensitivity to detect sonography or MRI). A pseudotumor may be misdiagnosed as a
a joint bleed. Persistent pain despite appropriate factor replacement neoplasm (e.g., Ewing sarcoma or osteosarcoma) or as an infection
may indicate impending avascular necrosis, and urgent joint aspira- (e.g., osteomyelitis or tuberculous abscess). Biopsy of such lesions
tion by an experienced interventionalist (using ultrasound guidance) is contraindicated because of the potential for significant bleeding or
or surgeon should be considered. Graded physiotherapy should be infection. Small pseudotumors, particularly distal ones or pseudotu-
instituted when there is symptomatic improvement. Follow-up imaging mors in patients with inhibitors, are often treated conservatively with
studies (MRI, bone scan, or both) should be considered for assessment aggressive clotting factor replacement along with immobilization of the
of avascular necrosis. affected limb.
Unfortunately, in some instances, factor replacement alone is insuf-
ficient, and complete surgical excision is needed. This carries potential
may be the most reflective of a patient’s joint disability status. Plain morbidity and even mortality and should only be undertaken by skilled
radiographs are relatively inexpensive and widely available but are surgeons in conjunction with appropriate hemophilia specialists.
insensitive to the soft tissue changes seen in the early stages of joint Attempts have been made at embolization of such pseudotumors,
and radiation therapy has been successfully used for treatment of small
disease and furthermore involve radiation exposure. Magnetic reso- pseudotumors of the hand.
nance imaging (MRI) is most sensitive to early joint (soft tissue)
changes and does not expose patients to radiation but is limited by
high cost, more limited availability, and the need for general anesthe-
sia in very young children. Work is ongoing on the use of point-of- Hematuria can rarely be caused by renal calculi because these are
care ultrasonography to evaluate acute and chronic changes in joints. thought to be more common in males with hemophilia compared
Ultrasonography does not require sedation in young children; is with the normal nonhemophilic male population. There is no con-
much less expensive than MRI; and differentiates between synovium sensus on how best to manage hematuria in patients with hemophilia,
and hemosiderin, which is not always possible with MRI. However, but in general, increased oral fluids along with bed rest are recom-
ultrasonography also has limitations: (1) it is very operator dependent, mended. If despite these measures, bleeding continues or is particu-
and the interpretation of ultrasound findings can be subjective, and larly severe, factor replacement should be given; the use of steroids
(2) some structures within a joint (e.g., cartilage) are not readily to manage hematuria in patients with hemophilia has been reported,
visualized. Automated ultrasonography is also being developed to but there is no conclusive evidence that steroids provide any addi-
address some of the former limitations of this imaging modality. tional benefit. Fortunately, hematuria in patients with hemophilia is
not associated with progressive loss of renal function, and as such, its
natural history is probably benign.
Mucous Membrane Bleeding The use of antifibrinolytic agents (tranexamic acid or epsilon
aminocaproic acid) is contraindicated in hematuria because of the
Epistaxis is not a prominent feature of hemophilia, but it certainly risk of ureteral obstruction by clots.
can occur. Oral bleeding is, however, quite common in patients with
hemophilia. Often one of the first presentations of hemophilia is
bleeding from the frenulum after trauma. Tongue bleeding, caused Gastrointestinal Bleeding
by a child biting the tongue, is also reasonably common. This can
become an emergency, either because of significant blood loss or from GI bleeding can occur in patients with hemophilia, particularly in
tongue swelling to the point of airway obstruction. Excessive bleeding adults, in whom it is often associated with the chronic use of non-
with loss of deciduous teeth and eruption of secondary dentition can steroidal antiinflammatory drugs (NSAIDs) for hemophilic arthropa-
occur but is again not common in hemophilia. Fortunately, most thy. Another potential explanation for GI tract bleeding is esophageal
bleeding from the mouth can be controlled with antifibrinolytic varices in patients with portal hypertension secondary to long-
agents, such as tranexamic acid (Cyklokapron) or epsilon aminoca- standing hepatitis C. Such patients may present with massive life-
proic acid (Amicar). Factor replacement may be required for more threatening melena or hematemesis.
serious cases. For dental surgery, particularly if it requires a nerve
block, factor replacement or DDAVP (1-deamino-8 D-arginine
vasopressin, desmopressin) needs to be given to raise the factor level Neurologic Bleeding
to at least 30% of normal.
ICH is the most dangerous hemorrhagic event in hemophilic patients.
It is at present, along with HIV and hepatitis C, one of the three
Hematuria leading causes of death in persons with hemophilia. In both neonates
and children with hemophilia, ICH is the leading cause of death.
In the past, hematuria was a reasonably common occurrence in ICH is also one of the leading causes of morbidity (mental retarda-
patients with hemophilia. Hematuria may be associated with trauma tion, seizure disorders, and motor dysfunction) in children with
14
but most often is spontaneous, episodic, and usually painless. hemophilia. The incidence of ICH is highest in neonates and is