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

aPTT, activated partial thromboplastin time; DIC, disseminated intravascular coagulation; ITP, immune thrombocytopenia purpura; PAI-1, plasminogen activator inhibitor-1; PT, prothrombin time; TCT, thrombin clot time: TPA, tissue plasminogen activator.

From Allen GA, Glader B. Approach to the bleeding child. Pediatr Clin North Am 2002;49:1239–1256, with permission.

A thorough history is the most important step in establishing the presence of a hemostatic disorder and in guiding initial laboratory testing. Figure 7-1 provides an approach to investigation of easy bruising or bleeding.

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Figure 7-1 An approach to investigation of easy bruising or bleeding.

(From Vora A, Makris M. An approach to investigation of easy bruising. Arch Dis Child 2001;84:488–491, with permission.)

Causes of Bleeding

Suggested Work-up

Complete blood cell count (CBC) To evaluate for thrombocytopenia and anemia
Peripheral blood smear To evaluate the cell lines and confirm thrombocytopenia
Prothrombin time (PT) To evaluate plasma coagulation function
Activated partial thromboplastin time (aPTT) To evaluate plasma coagulation function
Fibrinogen measurement To evaluate the functional activity of fibrinogen

Additional Work-up

Factor VIII, factor IX, von Willebrand factor antigen and activity (Ristocetin cofactor) Recommended in all cases of non-accidental injury
Mixing study To evaluate an abnormal PT or aPTT. Normalization of the PT or aPTT following a mixing study indicates a factor deficiency
Coagulation-factor assays (factors VIII, IX, and XI) Indicated when a factor deficiency is suggested by mixing studies or family history
Factor VII assay If there is an isolated prolongation of the PT to evaluate for vitamin K deficiency or liver disease
Thrombin time and reptilase time Indicated if there is an isolated prolongation of the aPTT and heparin contamination from the catheter is suspected. Prolongation of the thrombin time with normal reptilase time is suggestive of heparin contamination. If both the thrombin time and reptilase time are prolonged, the most likely cause is a low fibrinogen concentration
Thrombin time Used when both the PT and aPTT are prolonged to test for fibrinogen conversion to fibrin. When the thrombin time is prolonged, it signifies low fibrinogen activity, the presence of fibrin split products, or heparin contamination
Factor VIII, von Willebrand factor antigen, von Willebrand factor activity (ristocetin cofactor assay), and factor IX assay If von Willebrand disease is suspected
PFA-100 platelet function screen A newer substitute for the bleeding time. The time to occlusion is prolonged in patients with most types of von Willebrand disease and some platelet disorders
PT, aPTT, thromboplastin time, thrombin time, platelet count, factor VIII assay, factor V assay, fibrinogen, and D-dimer If disseminated intravascular coagulation is suspected