Bleeding and bruising

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Chapter 7 BLEEDING AND BRUISING

Theodore X. O’Connell

General Discussion

When a child presents with bruising or bleeding, the challenge for the physician is to ascertain whether the patient’s symptoms are appropriate to the hemostatic stress or whether further investigation of an underlying disorder is warranted. The main differential diagnoses are physiologic or accidental bleeding, nonaccidental injury, or a bleeding diathesis. The target of further investigation should be patients with bruising over the trunk, neck, or face, regardless of limb or mucosal bleeding, those with excessive blood loss after minor surgery, or a positive family history.

Bruising caused by accidental injury is common around the age of 1 year, when most infants have started cruising. As a guideline, normal bruising is restricted to the lower limbs; is not associated with petechiae, purpura, or mucosal bleeding; and the family history is negative. Child abuse should be suspected if there is significant bruising or bleeding with no history of trauma or a history inconsistent with the severity of injury. However, in a child with a bleeding diathesis, the child may have bruising or bleeding without a history of trauma. If a child has bruising in a recognizable pattern such as a belt or hand, then suspected abuse must be reported regardless of the outcome of laboratory tests.

Abnormal bleeding or bruising may cause significant anxiety for the patient and may be a sign of a serious inherited or acquired disorder. A history of bleeding following dental extraction, minor surgery, or childbirth suggests an underlying hemostatic disorder. Bleeding that is severe enough to require a blood transfusion merits particular attention. A family history of bleeding abnormalities suggests an inherited systemic disorder, such as von Willebrand disease.

Bleeding from a platelet disorder typically is localized to superficial sites such as the skin or mucous membranes and usually is easily controlled. However, bleeding from hemostatic or plasma coagulation defects may occur hours or days after injury and is difficult to control with local measures. This type of bleeding often occurs into muscles, joints, or body cavities.

Immune thrombocytopenic purpura is the most common hemostatic disorder of childhood to present with easy bruising and is usually associated with petechiae, purpura, and mucosal bleeding. Autosomal dominantly inherited von Willebrand disease is the most common congenital disorder of hemostasis. It often presents with easy bruising as the sole symptom, though mucosal bleeding is common. Purpura and petechiae are not common. Mild hemophilia A (factor VIII deficiency) and B (factor IX deficiency) are much less common but may present with symptoms similar to those of von Willebrand disease. Moderate and severe hemophilia A or B present in infancy with atypical bruising and later with hemarthrosis. Family history is absent in the 30% of sporadic hemophilia.

Initial laboratory tests are outlined below. It is important that results are compared against age-specific ranges because test results and coagulation factor levels vary with age. The pattern of abnormalities obtained using first line tests in association with the clinical presentation and history may indicate an underlying disorder (Table 7-1). However, some significant bleeding disorders may have normal test results. Conversely, some abnormal results are not associated with bleeding.

Table 7-1 Patterns of Coagulation Results and the Differential Diagnosis

Test Results Differential Diagnosis Possible Follow-up Laboratory Studies

PT normal

aPTT normal

Platelet count normal

Von Willebrand disease

Platelet function disorder

Factor XIII deficiency

Fiibrinolytic defect

PFA-100

Von Willebrand studies

Urea clot lysis test

Euglobulin clot lysis

Alpha-2-antiplasmin, PAI-1, TPA

PT normal

aPTT prolonged

Platelet count normal

PTT inhibitor

Von Willebrand disease

Hemophilia A or B

Factor XI deficiency

Heparin contamination

PTT mixing study

Factor assays (VIII, IX, XI)

Von Willebrand studies

Thrombin time/reptilase time

PT prolonged

aPTT normal

Platelet count normal

PT inhibitor

Vitamin K deficiency

Warfarin

Factor VII deficiency

PT mixing study

Factor assays (II, VII, IX, X)

PT prolonged

aPTT prolonged

Platelet count normal

Circulating inhibitor

Liver dysfunction

Vitamin K deficiency

Factor deficiency (II, V, X, or fibrinogen)

Dysfibrinogenemia

PT/PTT mixing studies

Thrombin time/reptilase time

Fibrinogen

Factor assays

PT prolonged

aPTT prolonged

Platelet count low

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