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Chapter 6: Classification, Clinical Manifestations, and Evaluation of Disorders of Hemostasis

Marcel Levi; Uri Seligsohn; Kenneth Kaushansky

SUMMARY

SUMMARY

Evaluation of a hemostatic disorder is commonly initiated when (1) a patient or referring physician suspects a bleeding tendency, (2) a bleeding tendency is discovered in one or more family members, (3) an abnormal coagulation assay result is obtained from an individual as part of a routine examination, (4) an abnormal assay result is obtained from a patient during preparation for surgery, or (5) a patient has unexplained diffuse bleeding during or after surgery or following trauma. Evaluation of a possible hemostatic disorder in each of these scenarios is a stepwise process that requires knowledge of the various classes of hemostatic disorders commonly found under the particular circumstances. The patient’s history, the results of physical examination, and an initial set of hemostatic tests usually enable a tentative diagnosis. However, more specific tests are commonly necessary to make a definitive diagnosis. This chapter reviews the necessary steps.

CLASSIFICATION OF HEMOSTATIC DISORDERS

Hemostatic disorders can conveniently be classified as either hereditary or acquired (Table 6–1). Alternatively, hemostatic disorders can be classified according to the mechanism of the defect. Of the acquired disorders, the thrombocytopenias are the most frequently encountered entities. Thrombocytopenias can result from reduced production of platelets, excessive destruction caused by antibodies or other consumptive processes, or pooling of platelets in the spleen, as in hypersplenism (Chap. 7); however, if hypersplenism is the sole cause of a hemostatic disorder, it is rarely severe enough to cause pathologic bleeding.

Table 6–1.Classification of Disorders of Hemostasis
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Table 6–1. Classification of Disorders of Hemostasis
Major Types Disorders Examples
Acquired Thrombocytopenias Autoimmune and alloimmune, drug-induced, hypersplenism, hypoplastic (primary, myelosuppressive therapy, myelophthisic marrow infiltration), disseminated intravascular coagulation (DIC), thrombotic thrombocytopenic purpura, hemolytic uremic syndrome (Chaps. 7, 19, and 22)
Liver diseases Cirrhosis, acute hepatic failure, liver transplantation (Chap. 18), thrombopoietin deficiency
Renal failure
Vitamin K deficiency Malabsorption syndrome, hemorrhagic disease of the newborn, prolonged antibiotic therapy, malnutrition, prolonged biliary obstruction
Hematologic disorders Acute leukemias (particularly promyelocytic), myelodysplasias, monoclonal gammopathies, essential thrombocythemia
Acquired antibodies against coagulation factors Neutralizing antibodies against factors V, VIII, and XIII, accelerated clearance of antibody-factor complexes, e.g., acquired von Willebrand disease, hypoprothrombinemia associated with antiphospholipid antibodies (Chaps. 16, 17, and 21)
DIC Acute (sepsis, malignancies, trauma, obstetric complications) and chronic (malignancies, giant hemangiomas, retained products of conception) (Chap. 19)
Drugs Antiplatelet agents, anticoagulants, antithrombins, and thrombolytic, hepatotoxic, and nephrotoxic agents (Chaps. 23–25)
Vascular Nonpalpable purpura (“senile,” solar, and factitious purpura), use of corticosteroids, vitamin C deficiency, child abuse, thromboembolic, purpura fulminans; palpable purpura (Henoch-Schönlein, vasculitis, dysproteinemias; Chap. 12), amyloidosis
Inherited Deficiencies of coagulation factors Hemophilia A (factor VIII deficiency), hemophilia B (factor IX deficiency), deficiencies of fibrinogen factors II, V, VII, X, XI, and XIII, and von Willebrand disease (Chaps. ...

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