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Overview of hemolytic anemias in children

Michael Recht, MD, PhD
Section Editor
Donald H Mahoney, Jr, MD
Deputy Editor
Carrie Armsby, MD, MPH


Anemia is among the most frequent laboratory abnormalities seen by a practicing pediatrician. Approximately 20 percent of all children in the United States and 80 percent of children in developing countries will be anemic at some time before their 18th birthdays [1]. Anemia is caused by one of three broad mechanisms: decreased production of red blood cells, increased loss of red blood cells, or destruction of red blood cells. Worldwide, the vast majority of childhood anemias are due to iron deficiency, due to either inadequate dietary intake or blood loss associated with gastrointestinal infections such as hookworm. However, the hemolytic anemias are associated with excessive morbidity and mortality.

The approach to a child with hemolytic anemia is discussed here. An overall approach to the anemic child, including the characteristics that suggest a hemolytic process, is discussed separately. (See "Approach to the child with anemia".)


The hemolytic process — After release from the bone marrow, mature, nonnucleated erythrocytes (red blood cells, RBCs) survive for 100 to 120 days in the circulation [2]. In the steady state, approximately 1 percent of the circulating erythrocytes are destroyed daily (ie, senescent RBCs) and are replaced by an equal number of new erythrocytes released from the bone marrow (ie, reticulocytes) (picture 1 and picture 2). The basic pathophysiology of the hemolytic anemias is a reduced erythrocyte lifespan, ranging from nearly normal to remarkably shortened. (See "Red blood cell survival: Normal values and measurement".)

In compensation for a reduced RBC lifespan, the bone marrow increases its output of erythrocytes, a response mediated by increased production of erythropoietin. As an example, in adults with hereditary spherocytosis, the bone marrow can increase its output of erythrocytes six- to eight-fold. With this maximal response, erythrocyte survival can be reduced to a value as low as 20 to 30 days without the onset of anemia (ie, fully compensated hemolysis). The limits of erythrocyte production in other hemolytic states have not been determined, particularly in infants and children, but they probably are lower in infants than in adults. (See "Regulation of erythropoiesis".)

As a result of increased RBC production in response to hemolysis, the reticulocyte count often exceeds 2 percent, with an absolute reticulocyte count usually greater than 100,000/microL [3]. When a chronic hemolytic process is present, hyperplasia of the erythropoietic marrow elements occurs, with reversal of the myeloid-to-erythroid ratio from the normal 3:1 to 1:1 or less (picture 3 and picture 4). In the severe, chronic hemolytic processes of childhood (eg, thalassemia major, congenital spherocytosis, sickle cell disease), hypertrophy of the marrow may expand the medullary spaces, producing bony changes, particularly in the skull and hands [4]. (See "Diagnosis of hemolytic anemia in the adult" and "Clinical manifestations and diagnosis of the thalassemias", section on 'Skeletal changes' and "Overview of the clinical manifestations of sickle cell disease", section on 'Skeletal complications'.)


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Literature review current through: Sep 2016. | This topic last updated: Sep 26, 2016.
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