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1986 Part XII: Hemostasis and Thrombosis Chapter 116: Classification, Clinical Manifestations, and Evaluation of Disorders of Hemostasis 1987

TABLE 116-2. Clinical Manifestations Typically Associated disorders. Excessive bruising is more common in women than men.
Moreover, women frequently note that the severity of their bruising
with Specific Hemostatic Disorders
varies with the phase of their menstrual cycle, although the most
Clinical Manifestations Hemostatic Disorders severe phase of the cycle may differ in different women. Features that
Mucocutaneous bleeding Thrombocytopenias, platelet help establish the severity of skin hemorrhage include the size of the
dysfunction, von Willebrand bruises, the frequency of bruising, whether the bruises occur sponta-
disease neously or only with trauma, and the appearance of bruises on regions
Cephalhematomas in newborns, Severe hemophilias A and B, of the body that usually are not traumatized, such as the trunk and
hemarthroses, hematuria, and severe deficiencies of factor back. The color of the bruise may yield information. Red bruises on
intramuscular, intracerebral, and VII, X, or XIII, severe type 3 the extensor surfaces of the arms and hands indicate loss of support-
retroperitoneal hemorrhages von Willebrand disease, ing tissues, as occurs in Cushing syndrome, glucocorticoid therapy,
afibrinogenemia senile purpura, and damage from chronic sun exposure. Jet-black
Injury-related bleeding and mild Mild and moderate hemo- bruises may be caused by warfarin-induced skin necrosis and similar
spontaneous bleeding philias A and B, severe factor disorders. Easy bruising can also occur in patients with Ehlers-Danlos
XI deficiency, moderate defi- syndrome manifested by distensible skin or extraordinary ligament
ciencies of fibrinogen and laxness, and in patients with hyperflexibility of the thumb. 8
factors II, V, VII, or X, combined 5. Tooth extractions are common hemostatic challenges and may be
factors V and VIII deficiency, helpful in defining the risk of bleeding. Molar extractions are greater
α -antiplasmin deficiency hemostatic challenges than extractions of other teeth. Objective data
2
Bleeding from stump of umbilical Afibrinogenemia, hypofibrino- regarding excessive bleeding based on the need for blood products
cord and habitual abortions genemia, dysfibrinogenemia, or the need to pack or suture the extraction site are valuable.
factor XIII deficiency 6. Excessive bleeding in response to razor nicks is common in patients
Impaired wound healing Factor XIII deficiency with platelet disorders or von Willebrand disease.
7. Hemoptysis almost never is the presenting symptom of a bleeding
Facial purpura in newborns Glanzmann thrombasthenia, disorder and is rare even in patients with serious bleeding disor-
severe thrombocytopenia
ders. However, blood-tinged sputum in association with upper
Recurrent severe epistaxis and Hereditary hemorrhagic respiratory tract infections may be more common in patients with
chronic iron deficiency anemia telangiectasias hemostatic disorders.
8. Hematemesis, like hemoptysis, almost never is the presenting
symptom of a hemostatic disorder. However, a hemostatic disor-
der may lead to hematemesis because of an anatomic abnormal-
ity in the upper gastrointestinal tract and bleeding may be more
CLINICAL MANIFESTATIONS severe than expected. Some hemostatic disorders more likely result
in hematemesis because of a combination of effects, such as liver
Individual hemorrhagic symptoms often require detailed analysis disease with deficient synthesis of coagulation proteins and with
before the significance of the symptoms and the resulting diagnosis or esophageal varices and aspirin ingestion with gastritis.
therapy can be determined. Some of the more common symptoms are 9. Hematuria is rarely the presenting symptom of a hemostatic disor-
discussed below, and Table 116–2 summarizes clinical manifestations der except for the hemophilias. However, hemostatic disorders can
that are typical for specific hemostatic disorders.
exacerbate hematuria caused by other disorders, including simple
1. Epistaxis is one of the most common signs of platelet disorders and von urinary tract infections.
Willebrand disease. It also is the most common symptom of hereditary 10. Rectal bleeding in individuals with normal hemostasis most often
hemorrhagic telangiectasia. In the latter condition, epistaxis almost results from hemorrhoids. However, von Willebrand disease and
always becomes more severe with advancing age. Epistaxis is not platelet disorders may contribute to repeated episodes of rectal
uncommon in normal children, but it usually resolves before puberty. bleeding when associated with a number of different underlying
Dry air heating systems can provoke epistaxis even in otherwise causes, including diverticula, hemorrhoids, or angiodysplasia.
normal individuals. Bleeding confined to a single nostril more likely Melena is also only rarely the presenting symptom of a hemor-
results from a local vascular problem than a systemic coagulopathy. rhagic disorder. However, repeated episodes of melena may occur
2. Gingival hemorrhage is very common in patients with both qual- in patients with hemorrhagic disorders.
itative and quantitative platelet abnormalities and von Willebrand 11. Menorrhagia is common in women with platelet disorders and von
disease. Occasional gum bleeding occurs in normal individuals, Willebrand disease. In general, menstrual bleeding is considered
especially if they use a hard bristle tooth brush and dental hygiene excessive if the patient indicates she has heavy flow for more than
procedures. Thus, establishing whether the bleeding is excessive 3 days or total flow for more than 7 days. However, an objective
may be difficult. Frequent gingival hemorrhage can occur in indi- distinction between menorrhagia (loss of more than 80 mL blood
viduals with normal hemostasis if they have gingivitis. per period) and normal blood loss can only be made by a visual
3. Oral mucous membrane bleeding in the form of blood blisters is a assessment technique using pictorial charts of towels or tampons. 7
common manifestation of severe thrombocytopenia. Such bleeding 12. Postpartum hemorrhage. Childbirth poses a considerable hemo-
usually has a predilection for sites where teeth can traumatize the static challenge. Consequently, patients with bleeding disorders
inner surface of the cheek. commonly manifest excessive bleeding during or after labor neces-
4. Skin hemorrhage in the form of petechiae and ecchymoses are com- sitating blood transfusion. An exception may be mild and moderate
mon manifestations of hemostatic disorders. However, skin hem- von Willebrand disease due to the vast increase in von Willebrand
orrhage also is common among individuals without hemostatic factor during pregnancy.

Kaushansky_chapter 116_p1985-1992.indd 1987 9/18/15 10:12 AM