Medication Summary
Medications are dictated by underlying disorders.
Chronic anemia due to intrinsic RBC abnormalities such as hemoglobinopathies, unstable hemoglobins, red cell membrane abnormalities, and red cell enzyme abnormalities do not warrant specific medications.
Nutritional anemia can be treated with supplements of deficient factors.
Recombinant erythropoietin has been useful in managing anemia related to chronic renal failure, rheumatoid arthritis, AIDS, and more recently in patients with DMT1 mutation. [18] Hemoglobin levels and general feelings of well-being have been much improved in patients since recombinant erythropoietin became commercially available.
Vitamins
Class Summary
These agents are essential for normal metabolism and DNA synthesis.
Cyanocobalamin (Vitamin B12, Athlete, CaloMist)
This agent is used to treat megaloblastic anemia resulting from vitamin B-12 deficiency. Deoxyadenosylcobalamin and hydroxocobalamin are active forms of vitamin B-12 in humans. Vitamin B-12 synthesized by microbes but not humans or plants. Deficiency may result from intrinsic factor deficiency (pernicious anemia), partial or total gastrectomy, or diseases of the distal ileum. Deficiency initially and typically manifests as macrocytic anemia, although neurologic symptoms may be present. They can also cause confusion or delirium in elderly patients.
It is essential for normal erythropoiesis and is required for healthy neuronal function and normal function of rapidly growing cells.
Iron Salts
Class Summary
These agents are useful for the treatment of iron deficiency anemia.
Ferrous sulfate
This is the mainstay treatment for patients with iron deficiency anemia. It is used as a building block for hemoglobin synthesis in treating anemia. It allows transportation of oxygen via hemoglobin and is necessary for oxidative processes of living tissue. Treatment should continue for about 2 months after correction of anemia and the etiological cause in order to replenish the body stores of iron. Ferrous sulfate is the most common and inexpensive form of iron used. Tablets contain 50-60 mg of iron salt. Other ferrous salts are used and may cause less intestinal discomfort because they contain smaller doses of iron (25-50 mg). Oral solutions of ferrous iron salts are available for use in pediatric populations.
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.
The authors recommend 6 mg/kg/d of elemental iron for 6 months for infants and children with severe iron deficiency anemia. Please note the dose is based on the weight of elemental iron, not of iron salts.
Colony Stimulating Factors
Class Summary
These agents are used to manage anemia related to chronic renal failure, rheumatoid arthritis, and AIDS.
Epoetin alfa (Procrit, Epogen)
This is a purified glycoprotein produced from mammalian cells and modified with the gene coding for human erythropoietin (EPO). The amino acid sequence is identical to that of endogenous EPO. Its biological activity mimics human urinary EPO, which stimulates division and differentiation of committed erythroid progenitor cells and induces the release of reticulocytes from bone marrow into the blood stream.
Chelation Agents
Class Summary
Iron overload (usually from multiple transfusions) may require chelation therapy, which usually begins when the ferritin level is greater than 1000 ng/mL.
Deferoxamine mesylate (Desferal)
Deferoxamine is freely soluble in water. Approximately 8 mg of iron is bound by 100 mg of deferoxamine. Deferoxamine is excreted in urine and bile and discolors the urine red. It readily chelates iron from ferritin and hemosiderin but not from transferrin. It is most effective when provided to the circulation continuously by means of infusion. Deferoxamine may be administered by IM injection, slow infusion, SC bolus, or continuous infusion. It does not effectively chelate other trace metals of nutritional importance.
Deferasirox (Exjade, Jadenu, Jadenu Sprinkle)
Deferasirox is available as a tablet for oral suspension. It is an oral iron-chelating agent demonstrated to reduce liver iron concentration in adults and children who receive repeated RBC transfusions. Deferasirox binds iron with high affinity in a 2:1 ratio. It is approved for treatment of treat chronic iron overload due to multiple blood transfusions. Treatment initiation is recommended when evidence of chronic iron overload (ie, transfusion of about 100 mL/kg packed RBCs [about 20 U for 40-kg person] and serum ferritin level consistently >1000 µg/L) is noted.
Deferiprone (Ferriprox)
1,2 dimethyl-3-hydroxypyridine-4-one is a member of a family of hydroxypyridine-4-one (HPO) chelators that requires 3 molecules to fully bind iron (III), each molecule providing 2 coordination sites (bidentate chelation). Its half-life is approximately 2 hours. The inactive metabolite is predominantly excreted in urine. Deferiprone is indicated for transfusional iron overload caused by thalassemia syndromes when current chelation therapy is inadequate.
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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.
