Page 2164 - Hematology_ Basic Principles and Practice ( PDFDrive )

Chapter 128 Clinical Approach to the Patient With Bleeding or Bruising 1917

defect or a fibrinolytic disorder. 1,3–7 However, bleeding into a joint Case 5: Illustration of Changes in Bleeding Problems Over Time
after an injury or an orthopedic (e.g., arthroscopic) procedure can be
experienced by persons with other types of bleeding disorders. 1,3–7 In A 65-year-old woman presents for urgent evaluation of a bleeding
patients with severe factor deficiencies, the clinical assessment should problem, requiring treatment for a symptomatic, expanding subdural
evaluate for symptoms and signs of arthropathy and muscle wasting hematoma. She had been previously diagnosed with type 1 von Wil-
and if relevant, neurologic sequelae complicating compartment syn- lebrand disease but indicated that she had no bleeding problems
drome bleeds. (despite many challenges) until she reached 30 years of age, when
she began to experience increasing problems with bruising, menor-
rhagia, and challenge-related bleeding, including severe gum bleeds
Subdural and Intracranial Hemorrhage with routine dental cleaning. An activated partial thromboplastin time
(aPTT) had been performed and was elevated. Testing confirmed a
low level of factor VIII (14%) and a low level of ristocetin cofactor
A newborn or child presenting with spontaneous intracranial hemor- activity (less than 10%). The patient was given emergency treatment
rhage or a large cephalohematoma should be investigated for severe with plasma-derived von Willebrand factor concentrate containing
underlying bleeding disorders, such as thrombocytopenia, hemo- factor VIII. Intravenous γ-globulin was given because she had a very
philia, factor XIII deficiency, other coagulation factor deficiencies, or poor response to replacement, suggesting rapid clearance of von
a severe defect in platelets or von Willebrand factor. 1,3–5 Trauma- Willebrand factor and factor VIII. Within 24 hours of the intravenous
related subdural or intracranial hemorrhages can also be manifesta- γ-globulin administration, her von Willebrand factor and factor VIII
tions of a severe bleeding disorder. 1,3–5 In adults, ischemic strokes are levels increased above normal, consistent with acquired von Willebrand
more frequent than hemorrhagic strokes, although hemorrhagic syndrome. Additional tests indicated that she had an immunoglobulin
strokes appear to predominate with some bleeding disorders (e.g., (Ig) G paraprotein without evidence of myeloma. Several features of
her presentation suggested that her von Willebrand factor abnormalities
7
Quebec platelet disorder ), and they can be complications of anti- were probably acquired and not because of type 1 von Willebrand
thrombotic drug treatment. disease (which is more common): her increasing bleeding symptoms
over time and lack of bleeding problems during childhood or early
adulthood, her very low level of factor VIII (because of its clearance
Hematuria with von Willebrand factor), the IgG paraprotein, her poor response
to von Willebrand factor replacement, and her excellent response to
Urinary tract bleeding with an infection is a commonly reported intravenous γ-globulin. She has since been managed with intermittent
symptom, whereas spontaneous (or unexplained) hematuria can intravenous γ-globulin treatment.
complicate hemophilia and other bleeding disorders, such as Quebec
platelet disorder. 3,7,28

Bleeding at Birth, Age-Related Changes in Bleeding, Signs of Active or Recent Bleeding and Conditions Associated With
Bleeding
and Very Rare Bleeding Symptoms
Although findings from the physical examination in bleeding disorders
Many individuals cannot answer questions about bleeding at the time are often normal, it is important to look for signs of active or recent
that they were born. Nonetheless, bleeding from the umbilical stump bleeding, including the following:
or a cephalohematoma at birth can be symptoms of a bleeding dis- 1. Petechiae, perifollicular hemorrhages (typical of scurvy)
2. Oral blood blisters, particularly if the patient has
order. 4,5,7 Menarche can be associated with a marked increase in thrombocytopenia
bleeding in women with inherited or acquired bleeding problems. 3. Ecchymoses, hematomas, and skin pigmentation changes
Some individuals with inherited bleeding disorders report a reduction because of recurrent bleeds
in their bleeding symptoms as they age, which could reflect lifestyle 4. Signs of active bleeding from a site of trauma or an incision,
adaptation and age-related increases in hemostatic protein levels (e.g., including excessive blood loss into drains
von Willebrand factor or fibrinogen) and thrombin generation. 5. Sequelae of previous bleeds in individuals known or suspected
Increases in bleeding with aging can suggest an acquired problem (see to have a severe bleeding disorder, such as muscle wasting and
box on Case 5: Illustration of Changes in Bleeding Problems Over arthropathy, neurologic abnormalities from prior intracranial or
compartment syndrome bleeds
Time). 6. Pallor arising from anemia: the palms are usually notably pale
Some types of bleeds are quite rare among individuals with bleed- when the hemoglobin is less than 10 g/dL
ing disorders, including spontaneous hemorrhage into the spleen, 7. Signs of an underlying hematologic disorder, such as
which can lead to rupture (see box on Signs of Active or Recent lymphadenopathy and/or splenomegaly
Bleeding). 8. Signs of acute or chronic liver disease, such as jaundice,
hepatomegaly, spider nevi, palmar erythema, or Dupuytren
contractures
LABORATORY MANIFESTATIONS 9. Signs of an endocrine disorder, such as hypothyroidism or
Cushing syndrome
10. Vascular lesions such as telangiectasia on the face, lips,
The general investigations that are appropriate for most assessments tongue, buccal mucosa, or fingers which can suggest hereditary
of bleeding problems include (1) a complete blood count (to establish hemorrhagic telangiectasia
if there is thrombocytopenia or anemia), (2) an assessment for a low 11. Hyperextensibility if the bleeding history suggests a collagen
ferritin level to evaluate for iron deficiency (less commonly associated disorder as a potential diagnosis
with microcytosis or anemia; iron deficiency should be corrected 12. Signs suggestive of a syndromic bleeding disorder (albinism,
if bleeding risks are increased), (3) a blood group and antibody hearing impairment, absent radii)
screen, particularly for individuals with prior pregnancies or transfu-
sions and upcoming surgery or major dental procedures, and (4)
tests of renal function (creatinine) if antifibrinolytic therapy will be
considered because the dosage is dependent on renal function or if and/or if the fluid balance is difficult to assess. Tests of liver func-
an MYH9-related disorder is suspected (see box on The Laboratory tion are warranted if the person has risks for transfusion-acquired,
Manifestations of Bleeding Disorders). The bleeding time test is no chronic liver disease. Tests for liver and thyroid disease (particularly
longer recommended, because of its technical limitations and poor hypothyroidism) can be helpful if the history suggests an acquired
sensitivity to common bleeding disorders. 3,15,30 Electrolyte levels merit bleeding problem of unknown etiology. Screening for Cushing
monitoring when repeated doses of desmopressin therapy are given syndrome (24-hour urine test for free cortisol) should be restricted