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Chapter 116: Classification, Clinical Manifestations, and Evaluation of Disorders of Hemostasis

Marcel Levi; Uri Seligsohn; Kenneth Kaushansky

INTRODUCTION

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.

Acronyms and Abbreviations

aPTT, activated partial thromboplastin time; DIC, disseminated intravascular coagulation; ELISA, enzyme-linked immunosorbent assay; PT, prothrombin time; RCF, ristocetin cofactor.

CLASSIFICATION OF HEMOSTATIC DISORDERS

Hemostatic disorders can conveniently be classified as either hereditary or acquired (Table 116–1). 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. 119); however, if hypersplenism is the sole cause of a hemostatic disorder, it is rarely severe enough to cause pathologic bleeding.

Table 116-1.Classification of Disorders of Hemostasis
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Table 116-1. 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. 117, 129, and 132)
Liver diseases Cirrhosis, acute hepatic failure, liver transplantation (Chap. 128), 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 (Chaps. 85,86,87 and 106)
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. 126, 127, and 131)
DIC Acute (sepsis, malignancies, trauma, obstetric complications) and chronic (malignancies, giant hemangiomas, retained products of conception) (Chap. 129)
Drugs Antiplatelet agents, anticoagulants, antithrombins, and thrombolytic, hepatotoxic, and nephrotoxic agents (Chaps. 25 and 133–135)
Vascular Nonpalpable purpura (“senile,” solar, and factitious purpura), use of corticosteroids, vitamin C deficiency, child abuse, thromboembolic, purpura fulminans; palpable-purpura (Henoch-Schönlein, vasculitis, ...

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