Approach Considerations
Chronic anemia merits prompt, if not immediate, attention.
Common sense should prevail in recognizing that, although anemia may be quite profound, the patient is usually well. In this circumstance, it is prudent not to follow the hemoglobin level too closely and thereby create unnecessary apprehension in the family. When physiologic adaptive mechanisms are in place, most children do well, and what is abnormal in others becomes normal in these children. At this point, the art of medicine takes precedence over the science of medicine.
Patients with chronic anemias rarely need inpatient care, even during the diagnostic process. Deal with complications on an ad hoc basis.
Go to Anemia and Anemia of Prematurity for complete information on these topics.
Transfusion
Exclusion of impending high-output cardiac failure is the most important issue. High-output failure is the only reason that blood transfusion is necessary.
RBC transfusions must be performed cautiously; rapid expansion of intravascular volume may cause congestive heart failure in a well-compensated patient. Two or more small aliquots of RBCs may need to be administered, with a few hours of reequilibration between transfusions.
Rapid volume expansion with normal saline, usually performed in the emergency department in chronically anemic children, also may cause congestive heart failure. One needs to assess the benefits and risks of volume expansion in this situation before one decides to give a normal saline bolus.
Elective surgery can usually be performed without preoperative transfusion as long as blood is available.
For patients who require long-term transfusional support, identification of a limited number of dedicated blood donors is desirable. Donors are selected on the basis of detailed antigenic crossmatching with the patient in hopes of avoiding development of immune-mediated hemolysis. Obtain serum ferritin values every 6-10 units of transfusion. If ferritin exceeds 1000 µg/L, start deferasirox or deferoxamine therapy.
Splenectomy
Splenectomy is often indicated in patients with HS, but not necessarily during the pediatric age. Delay splenectomy until patients are aged 8-9 years, by which time immunity to encapsulated bacteria is well established. Typically, this is also before hemolysis sufficient to result in bilirubin gallstones has occurred. However, in some patients, repetitive splenic sequestration occurs, necessitating earlier splenectomy. Intrafamilial variation is common in terms of severity of anemia; thus, history of childhood splenectomy in a parent does not necessarily indicate a need for splenectomy in the affected child.
Prevention of Anemia
Dietary iron deficiency anemia can be prevented by starting supplemental iron when infants are weaned off breast milk or regular formula at age 8-12 months; 1-2 mg/kg/d of elemental iron is usually sufficient to prevent iron deficiency anemia. (See current American Academy of Pediatrics guidelines. [13] ) This should be continued until the child more or less eats regular table foods as the main source of calories. Treatment of iron deficiency anemia needs a higher dose of iron, usually 6 mg/kg/d of elemental iron, for at least 3 months.
Infants of vegan mothers who exclusively breast feed may develop megaloblastic anemia and neurologic signs due to B12 deficiency. These infants should be given B12 supplementation.
There are concerns regarding the potential deleterious developmental effects of excess iron exposure in early childhood. In a randomized evaluation of low-iron versus iron-supplemented formulas in Chile, a 10-year follow-up suggests the presence of subtle neurocognitive deficits specifically in those infants with higher hemoglobin levels at the time of randomization. [16]
Consultations
Many chronic anemias can be diagnosed and managed by generalists. However, when subtle distinctions in morphology or interpretation of laboratory data relative to hemolytic anemia are important, a pediatric hematologist is usually needed. Certainly, when bone marrow aspiration and biopsy are contemplated, the experience of a pediatric hematologist is essential.
Unless readily explained by an increased reticulocyte count or another physiologic condition, macrocytosis usually warrants consultation with a hematologist.
A number of children with hypochromic anemia outside the dietary iron deficiency age are ultimately found to have chronic GI bleeding. It is essential in this situation to work closely with a pediatric gastroenterologist.
Although rare, congenital sideroblastic anemia may present as hypochromic, microcytic anemia in infants. Ringed sideroblasts, ie, erythroid precursors with pathologic, perinuclear iron-loaded mitochondria, occur in bone marrow, characterizing this disorder of abnormal erythropoiesis. Bone marrow examination is necessary to establish the diagnosis. [17]
Long-Term Monitoring
Monitor growth and development, nutritional status, degree of splenomegaly, formation of gallstones, onset of puberty, endocrine status, and any potential complications related to underlying disorders.
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Blood smear from a black male with glucose-6-phosphate dehydrogenase (G-6-PD) deficiency that resulted in acute hemolysis. Note blister (helmet or bite) cells and very dense spherocytic cells. The blood smear is virtually pathognomic of this disorder.
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Blood smear of hereditary spherocytosis (HS). Note many spherocytic cells. Not all patients with HS are anemic.
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Blood smear of hemoglobin C trait. Note numerous target cells. Target cells are a characteristic of this hemoglobinopathy. The trait patient has no anemia. Target cells are also seen in patients with iron deficiency anemia, thalassemia, sickle cell disease, and liver disease.
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Blood smear of a patient with homozygous sickle cell disease. Note several sickle cells, a nucleated RBC, and a red cell with Howell-Jolly body (indicated by an arrow), evidence of functional asplenia.
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A blood smear showing a few schistocytes. This patient had Kaposi type hemangioendothelioma with periodic microangiopathic hemolysis and disseminated coagulopathy (Kasabach-Merritt phenomenon).
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A blood smear of a patient with beta thalassemia trait. Note red cells pointed by arrows. Multiple bluish dots in the cells are called basophilic stipplings and consist of aggregated ribosomes. They are often present in immature red cells such as reticulocytes.
