Pediatric Chronic Anemia Treatment & Management
- Author: Susumu Inoue, MD; Chief Editor: Max J Coppes, MD, PhD, MBA more...
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.
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.
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 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. ) 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.
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 diagosis.
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.
Dowling MM, Quinn CT, Plumb P, Rogers ZR, Rollins NK, Koral K. Acute silent cerebral ischemia and infarction during acute anemia in children with and without sickle cell disease. Blood. 2012 Nov 8. 120(19):3891-7. [Medline].
Baker C, Grant AM, George MG, Grosse SD, Adamkiewicz TV. Contribution of Sickle Cell Disease to the Pediatric Stroke Burden Among Hospital Discharges of African-Americans-United States, 1997-2012. Pediatr Blood Cancer. 2015 Dec. 62 (12):2076-81. [Medline].
Skeppner G, Kreuger A, Elinder G. Transient erythroblastopenia of childhood: prospective study of 10 patients with special reference to viral infections. J Pediatr Hematol Oncol. 2002 May. 24(4):294-8. [Medline].
Penchansky L, Jordan JA. Transient erythroblastopenia of childhood associated with human herpesvirus type 6, variant B. Am J Clin Pathol. 1997 Aug. 108(2):127-32. [Medline].
Prassouli A, Papadakis V, Tsakris A, Stefanaki K, Garoufi A, Haidas S. Classic transient erythroblastopenia of childhood with human parvovirus B19 genome detection in the blood and bone marrow. J Pediatr Hematol Oncol. 2005 Jun. 27(6):333-6. [Medline].
Iolascon A, Camaschella C, Pospisilova D, Piscopo C, Tchernia G, Beaumont C. Natural history of recessive inheritance of DMT1 mutations. J Pediatr. 2008 Jan. 152(1):136-9. [Medline].
Global Burden of Disease Pediatrics Collaboration, Kyu HH, Pinho C, et al. Global and National Burden of Diseases and Injuries Among Children and Adolescents Between 1990 and 2013: Findings From the Global Burden of Disease 2013 Study. JAMA Pediatr. 2016 Mar 1. 170 (3):267-87. [Medline].
Henderson S, Timbs A, McCarthy J, Gallienne A, Van Mourik M, Masters G. Incidence of haemoglobinopathies in various populations - the impact of immigration. Clin Biochem. 2009 Dec. 42(18):1745-56. [Medline].
Williams TN, Weatherall DJ. World distribution, population genetics, and health burden of the hemoglobinopathies. Cold Spring Harb Perspect Med. 2012 Sep. 2(9):a011692. [Medline].
Weatherall DJ. The inherited diseases of hemoglobin are an emerging global health burden. Blood. 2010 Jun 3. 115(22):4331-6. [Medline].
Baker RD, Greer FR. Diagnosis and prevention of iron deficiency and iron-deficiency anemia in infants and young children (0-3 years of age). Pediatrics. 2010 Nov. 126(5):1040-50. [Medline].
Wiseman DH, May A, Jolles S, et al. A novel syndrome of congenital sideroblastic anemia, B-cell immunodeficiency, periodic fevers, and developmental delay (SIFD). Blood. 2013 Jul 4. 122 (1):112-23. [Medline]. [Full Text].
Iolascon A, De Falco L. Mutations in the gene encoding DMT1: clinical presentation and treatment. Semin Hematol. 2009 Oct. 46(4):358-70. [Medline].
Finberg KE. Iron-refractory iron deficiency anemia. Semin Hematol. 2009 Oct. 46(4):378-86. [Medline].