A finite life span is a distinct characteristic of red cells. Hence, a logical, time-honored classification of anemias is in three groups: (1) decreased production of red cells, (2) increased destruction of red cells, and (3) acute blood loss. Decreased production is covered in Chaps. 7, 9, and 11; increased destruction and acute blood loss are covered in this chapter.
All patients who are anemic as a result of either increased destruction of red cells or acute blood loss have one important element in common: the anemia results from overconsumption of red cells from the peripheral blood, whereas the supply of cells from the bone marrow is normal (indeed, it is usually increased). On the other hand, these two groups differ in that physical loss of red cells from the bloodstream or from the body itself, as in acute hemorrhage, is fundamentally different from destruction of red cells within the body, as in hemolytic anemias. Therefore, the clinical aspects and pathophysiology of anemia in these two groups of patients are quite different, and they will be considered separately.
With respect to primary etiology, anemias due to increased destruction of red cells, which we know as hemolytic anemias (HAs), may be inherited or acquired; from a clinical point of view, they may be more acute or more chronic, and they may vary from mild to very severe; the site of hemolysis may be predominantly intravascular or extravascular. With respect to mechanisms, HAs may be due to intracorpuscular causes or to extracorpuscular causes (Table 10-1). But before reviewing the individual types of HA, it is appropriate to consider what they have in common.
TABLE 10-1Classification of Hemolytic Anemiasa ||Download (.pdf) TABLE 10-1 Classification of Hemolytic Anemiasa
| ||Intracorpuscular Defects ||Extracorpuscular Factors |
|Hereditary || |
|Familial (atypical) hemolytic-uremic syndrome |
|Acquired ||Paroxysmal nocturnal hemoglobinuria (PNH) || |
Mechanical destruction (microangiopathic)
GENERAL CLINICAL AND LABORATORY FEATURES
The clinical presentation of a patient with anemia is greatly influenced in the first place by whether the onset is abrupt or gradual, and HAs are no exception. A patient with autoimmune HA or with favism may be a medical emergency, whereas a patient with mild hereditary spherocytosis or with cold agglutinin ...