Page 2159 - Hematology_ Basic Principles and Practice ( PDFDrive )

C H A P T E R 128

CLINICAL APPROACH TO THE PATIENT WITH

BLEEDING OR BRUISING

Catherine P.M. Hayward

Bruising and bleeding problems are common reasons for a hematology can be triggered or exacerbated by therapies for atherosclerotic disease
1
referral. The management of an acute bleed (e.g., life-threatening (e.g., aspirin and/or P2Y12 inhibitors), venous thromboembolic
hemorrhage from an acquired factor VIII inhibitor, an anticoagulant disease (prophylaxis or treatment with anticoagulants), inflammatory
drug, or a postpartum hemorrhage) should be the first priority because states (e.g., treatment with prednisone or other glucocorticoids) and
diagnostic test results are often not immediately available. However, pain (prescription or nonprescription use of nonsteroidal antiinflam-
1–3
most assessments of bruising and bleeding symptoms are not urgent. matory drugs). Bruising can also reflect systemic or topical corti-
The assessment of bleeding or bruising can be challenging because costeroid use and the effects of age- or sun-exposure related changes
some individuals without bleeding problems often experience bleed- in the skin (i.e., senile purpura). 1,3
1–3
ing symptoms (e.g., bruising with trauma, nosebleeds). Further- Without treatment, severe bleeding disorders typically cause
more, most individuals referred for assessment of bleeding or bruising abnormal bleeding with all major hemostatic challenges. On the
will have experienced some bleeding symptoms that may or may not other hand, bleeding may not occur with every challenge in persons
3
reflect an underlying bleeding disorder. Because many individuals with mild defects and lower bleeding risk (e.g., those with a 5- to
referred to a hematologist for an evaluation for a bleeding disorder 10-fold increased risk for bleeding). Age influences the bleeding
will be diagnosed with a bleeding disorder, there is a high pretest history by increasing the likelihood of exposures to hemostatic chal-
probability for a bleeding disorder among such patients. 3 lenges and the development of sequelae such as arthropathy in
6,7
Some referrals for bleeding disorder assessment are for asymptom- patients with severe hemophilia. A severe unexplained bleed with
atic problems (e.g., abnormal coagulation tests caused by vitamin K surgery may be considered suspicious of a bleeding problem. However,
deficiency, evaluation of a familial bleeding problem after diagnosis if the person reports that prior challenges did not result in bleeding,
of other relatives). Mild bleeding symptoms (e.g., bruising, nose- this narrows down the possibilities to a mild inherited bleeding dis-
bleeds, possible abnormal bleeding with prior surgery) are more order, an acquired bleeding disorder, an iatrogenic condition (e.g.,
common than severe symptoms (e.g., severe bleed from anticoagulant bleeding while on anticoagulant therapy or a technical problem
therapy, life-threatening postpartum hemorrhage, joint bleed) among during surgery that caused bleeding) or a problem that is not related
referred individuals. Sometimes the bleeding symptoms reflect an to a congenital or acquired bleeding disorder (e.g., a postpartum
underlying bone marrow disorder (e.g., bleeding associated with hemorrhage after a Caesarian section from uterine atony or retained
thrombocytopenia caused by leukemia, or a platelet function defect products of conception). A bleeding disorder assessment needs to
induced by a myelodysplastic or myeloproliferative syndrome), which consider both familial and personal bleeding symptoms (see box on
can develop as a complication of some inherited bleeding disorders Case 2: Illustration of The Importance of Assessing Both Personal
(e.g., inherited thrombocytopenia caused by a RUNX1, ANKRD or and Familial Bleeding Problems).
FLI1 mutation). Some causes of bleeding, such as vitamin C defi- The timing of bleeding with challenges is evaluated to determine
ciency (scurvy), are rare in developed countries. When considering whether the bleeding problem reflects a common bleeding disorder,
possible causes of bleeding or bruising, it is important to identify such as von Willebrand disease, a platelet function disorder, an
symptoms that are of concern to the patient and/or the referring undefined mucocutaneous bleeding problem, or a rarer cause, such
physician and to determine the extent of bleeding that the person has as a defect or deficiency in a coagulation factor or fibrinolytic
1,3
1,3
experienced. The next step is to formulate a differential diagnosis protein (see box on Case 3: Illustration of the Importance of Assess-
and plan for bleeding symptom management (e.g., control of menor- ing Bleeding Problems Over Time; see also boxes on Cases 1 and 2).
rhagia), including strategies to minimize future bleeding risks from Although patients will often know whether bleeding began on the
exposure to surgery and other invasive procedures. 3 day of a challenge, their recall of timing details is often better when
Fig. 128.1 provides a general guide to the steps involved in clinical procedures were done without general anesthesia (e.g., dental extrac-
assessment. Bleeding-history assessment tools provide a detailed tions, biopsies). 1,3,7 Bleeding within a few hours or on the same day
framework to evaluate the medical history and to determine which of a challenge (e.g., surgery or dental extraction) is most suggestive
symptoms should be considered more suspicious of an underlying of a defect involving von Willebrand factor or platelets. 1,3,7 Delayed
4–8
bleeding problem. Bleeding-history assessment tools have been bleeding (beginning 1 or more days after a challenge) is most sugges-
used to standardize and quantify bleeding for research purposes, and tive of a coagulation or fibrinolytic defect (see boxes on Cases 1 and
some have a reasonable utility for ruling out a bleeding disorder when 2). 1,3,7 However, the onset of some bleeding, such as postpartum
5
performing an initial assessment for bleeding problems (see box on hemorrhage, can be delayed in persons with von Willebrand disease
Influences on Presenting Problems). While a very high bleeding score or platelet function disorders. 1,3,9–13 Postpartum bleeding may be
4–8
is consistent with a bleeding problem, there is overlap of scores for absent or inconsistent in women with mild bleeding disorders because
individuals with and without bleeding problems. 4,5,7,8 These tools pregnancy increases the levels of some hemostatic proteins, including
provide evidence that some bleeding symptoms are uncommon unless fibrinogen and von Willebrand factor. Bleeding during pregnancy
there is a bleeding disorder (e.g., joint bleeds, bruises that are as large after implantation is uncommon with most bleeding disorders,
or larger than an orange or that track downward). 2,4,5,7,8 Nonetheless, although it can be severe in individuals with bleeding disorders who
further research is needed to determine the accuracy of recommended develop placental abruption (e.g., from an untreated fibrinogen dis-
bleeding assessment tools for detecting a bleeding disorder at the time order or factor XIII deficiency). Intracranial bleeding in a child may
of bleeding disorder assessment (see box on Case 1: Illustration of a suggest a severe defect or deficiency of factor XIII, a coagulation
Mild, Inherited Bleeding Problem). factor deficiency, or a fibrinolytic inhibitor, but other conditions
Some bleeding symptoms, such as menorrhagia or nosebleeds, are should be considered if the diagnostic tests for these conditions are
not specific to any particular type of bleeding problem. 1,3,7 Bleeding negative.

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