Page 2160 - Hematology_ Basic Principles and Practice ( PDFDrive )

Chapter 128 Clinical Approach to the Patient With Bleeding or Bruising 1913

Assess reason for referral, previous Case 1: Illustration of a Mild, Inherited Bleeding Problem
diagnosis/investigations, and patient’s
concerns about bleeding. A 77-year-old man who is starting treatment for multiple myeloma was
discovered to have a prolonged activated partial thromboplastin time.
Review of his records indicated that the abnormality was present on
a previous admission for spinal cord compression, which was treated
Evaluate the history for unprovoked, unexpected, significant, and with surgery. He required 4 units of packed red blood cells several
recurrent bleeding (current and previous). Assess for symptoms days after this surgery because of delayed postoperative bleeding.
of bruising, prolonged bleeding with cuts, nosebleeds, gum and There was no other bleeding history. He was found to have mild factor
oral bleeding, gastrointestinal bleeding, joint or muscle bleeds, IX deficiency, unrelated to the myeloma, and his daughter proved to
urinary tract bleeding, and other bleeding (e.g., intracranial, be a carrier of this defect.
umbilical stump). Evaluate the drug history and family history
of bleeding problems. Evaluate other medical problems.
Determine the nature and timing of any abnormal bleeding
with challenges (right away, within hours or days after) and
the severity (e.g., required transfusion, longer hospital stay, Case 2: Illustration of the Importance of Assessing Both Personal and
developed large hematomas). Familial Bleeding Problems
A 22-year-old woman was referred for evaluation of a possible platelet
disorder. She had a history of menorrhagia (4 days out of 7 days
If symptoms suggest an underlying bleeding problem, evaluate of menstrual flow were heavy when not on treatment), prolonged
whether the cause could be an acquired or congenital problem nosebleeds in childhood, and hematuria with urinary tract infections.
(e.g., symptoms from childhood, positive family history). She did not have thrombocytopenia, and she had no exposure to
major hemostatic challenges. Her father, uncle, and grandfather had a
striking bleeding history, and two of them had thrombocytopenia. The
bleeding in her relatives included joint bleeds with trauma and severe,
If bleeding problems are new, consider potential reasons and delayed-onset bleeding after trauma and surgery (usually more than a
triggers (e.g., a first major hemostatic challenge could be the first day later), which continued for weeks despite platelet transfusions. One
presentation of a mild bleeding disorder; trigger could be drugs, of these relatives reported no bleeding when he had a tooth extracted
development of an immune disorder, or blood, endocrine, liver, while receiving fibrinolytic inhibitor therapy. Although menorrhagia is
or renal disease). not specific to any one type of bleeding disorder, the delayed bleeding
in affected relatives suggests a possible autosomal dominant disorder
and either a fibrinolytic defect or a factor defect or deficiency (e.g.,
dysfibrinogenemia; the latter had been excluded in previous tests of the
Formulate a differential diagnosis for the potential inherited and affected relatives). Because of the family history of thrombocytopenia,
acquired causes that should be investigated. joint bleeds, and delayed bleeding, which did not respond well to
platelet transfusions, testing was done for the Quebec platelet disorder.
Fig. 128.1 STEPS TO EVALUATE BLEEDING AND BRUISING Genetic testing for duplication mutation of the urokinase plasminogen
PROBLEMS. activator gene confirmed this diagnosis in the patient and her relatives.
This case illustrates the importance of evaluating both the personal and
family bleeding history and highlights the fact that bleeding-symptom
severity can vary among affected family members, in part because of
Influences on Presenting Problems their different exposures to challenges and treatments.
When evaluating a bleeding history, it is important to recognize that the
presenting problems are influenced by the following factors:
1. The nature and severity of the defect, and the presence of single Case 3: Illustration of the Importance of Assessing Bleeding Problems
or multiple risk factors for bleeding Over Time
2. Whether the bleeding problem is congenital or acquired
3. Antecedent exposure to hemostatic challenges (such as surgery, A 72-year-old man was referred for evaluation of a severe bleed after
dental extraction, menses, and childbirth) and the risk for receiving a single dose of low-molecular-weight heparin for uncon-
bleeding with each of these challenges firmed deep vein thrombosis. He had a history of a similar bleeding
4. The presence of other medical problems (e.g., renal, hepatic, or episode several years previously while on warfarin treatment for atrial
thyroid disease), including anemia fibrillation. There was no other bleeding history, and the patient subse-
5. Variability in the bleeding symptoms experienced by individuals quently developed a spontaneous iliopsoas bleed. He had undergone
without bleeding disorders (e.g., nosebleeds, bruising) and by numerous surgeries earlier in life without any bleeding problems, and
individuals with known bleeding disorders, even within families there was no family history of bleeding. The bleeding history suggested
with the same defect the possibility of an acquired bleeding problem, possibly acquired
6. Local factors (e.g., sun-damage to the skin, vascular lesions, von Willebrand disease or an acquired factor deficiency. Diagnostic
diverticular disease, or cancerous lesions in the gastrointestinal testing indicated that he had acquired factor XIII deficiency. This case
tract) and the possibility of nonaccidental trauma illustrates the fact that there may be more than one risk factor for
7. Treatments that increase the risk for bleeding (e.g., antiplatelet bleeding: in this case, several exposures to anticoagulants triggered
drugs, such as aspirin and nonsteroidal antiinflammatory drug bleeding in a patient with an acquired factor deficiency. On initial
used for pain control, anticoagulant therapy, etc.) treatment of his iliopsoas bleed with factor XIII concentrate, there
8. Whether treatments were used to prevent or control bleeding was partial neutralization of the infused factor followed by accelerated
9. Whether treatments prescribed for other reasons may have clearance, consistent with acquired factor XIII deficiency secondary
reduced bleeding (e.g., reduced menstrual bleeding while on oral to an autoantibody.
contraceptives to prevent pregnancy)

more suspicious, require treatment, and/or are highly predictive of a
EPIDEMIOLOGY bleeding problem. Some individuals have multiple risk factors for
1–3
bleeding (e.g., low von Willebrand factor levels, exposure to drugs
An understanding of the epidemiology of bleeding problems that inhibit platelet function after a surgical procedure associated
3
requires distinction between symptoms that rarely represent a with a high risk for bleeding). Although some symptoms, such
pathologic condition and do not require investigation or therapy as nosebleeds, easy bruising, and menorrhagia, are quite prevalent
(e.g., isolated “easy bruising”, brief nosebleeds) and those that are in the general population (10% or more report these symptoms),