CauseEtiology and epidemiologyPresentationIndices and other laboratory testing
Neonatal7
Blood lossHemorrhage (placental abruption, subgaleal, traumatic); maternal-fetal and twin-twin transfusion Tachypnea, pallor, and mental status change (irritability, poor feeding); > 20 percent loss of blood volume results in shock and cardiopulmonary collapseAnemia with normal indices; reticulocyte count is initially normal, then increases; positive Kleihauer-Betke test in maternal-fetal hemorrhage
Accounts for 5 to 10 percent of all cases of severe neonatal anemia
IsoimmunizationABO incompatibility, Rh incompatibility Jaundice and mild anemia; infants with severe isoimmunization (e.g., untreated Rh incompatibility) may present with hydrops fetalisPositive Coombs test; elevated bilirubin level; normocytic anemia with elevated reticulocyte count
Rh incompatibility occurs in 10.6 per 10,000 live births; 50 percent of these infants develop anemia
Congenital hemolytic anemiaSpherocytosis, G6PD deficiencyHyperbilirubinemia and moderate jaundiceLow enzyme activity; with hemolysis, smear may show poikilocytosis, reticulocytosis, Heinz bodies, and bite cells (in G6PD deficiency) or spur cells (in pyruvate kinase deficiency)
Congenital infectionParvovirus B19, human immunodeficiency virus, syphilis, rubella, sepsisPallor, irritability, and other findings associated with infection (e.g., deafness)Normocytic anemia with low reticulocyte count
Diamond-Blackfan syndromeCongenital pure red cell aplasia resulting from increased apoptosis in erythroid precursors Neonatal pallor progressing to symptomatic anemia; average age of diagnosis is 3 months; about 30 percent have other abnormalitiesMacrocytic anemia with low reticulocyte count
Affects 7 per 1 million live births
Fanconi anemiaIncreased susceptibility of progenitor cells in bone marrow leads to increased apoptosis, progressing to pancytopeniaAverage age of diagnosis is 8 years, but associated congenital abnormalities may facilitate early diagnosis (e.g., café-au-lait spots; microsomy; low birth weight; thumb, renal, skeletal, and eye abnormalities)Microcytic anemia and reticulocytopenia, thrombocytopenia, or leukopenia; DNA sequencing can detect genetic mutations for Fanconi anemia complementation groups
Infancy to toddlerhood2
Iron deficiencyInadequate dietary intake, chronic occult blood loss (excessive cow's milk consumption, inflammatory bowel disease, Meckel diverticulum, parasites) Usually asymptomatic; severe cases can present with fatigue, pallor, or dyspnea; rarely occurs before 6 months of age; highest risk is at 6 to 36 months of ageMicrocytic anemia with elevated RBC distribution width; peripheral smear shows hypochromic microcytes and may show target cells; iron and ferritin levels and iron saturation are low; transferrin level is elevated
Prevalence is 8 to 15 percent
Concurrent infectionBacterial or viral infection leading to cytokine-mediated decrease in iron utilization and RBC productionPresenting symptoms usually result from infectious processNormocytic or mildly microcytic, low/normal serum iron level with low transferrin level; ferritin level may be elevated because it is an acute phase reactant
Blood lossTrauma, gastrointestinal bleedingTachypnea, tachycardia, pallor, hypotensionHgb levels may initially be normal, followed by anemia with normal indices
Disorder of Hgb structure or synthesisThalassemia, sickle cell diseaseAnemia in thalassemia may range from mild and asymptomatic to severe, depending on number of heme chains affected; sickle cell disease presents with hemolysis, pain crises, dactylitis, and aplastic crisis; symptoms are rarely present at birth but typically develop in the first yearMicrocytic anemia, low RBC distribution width, and low Mentzer index in thalassemia; Hgb electrophoresis may show Hgb F; smear with basophilic stippling; hemolysis, reticulocytosis, and Hgb S on electrophoresis in sickle cell disease
RBC enzyme defectsG6PD deficiency, pyruvate kinase deficiency Neonatal hyperbilirubinemia and hemolytic anemia when exposed to oxidative stressLow enzyme activity; with hemolysis smear may show poikilocytosis, reticulocytosis, Heinz bodies, and bite cells (in G6PD deficiency) or spur cells (in pyruvate kinase deficiency)
10 percent of the black population has G6PD deficiency
RBC membrane defectsSpherocytosis, elliptocytosisHyperbilirubinemia, splenomegaly, gall bladder disease, and aplastic crisis; autosomal dominant, so family history is positive in about 75 percent of patientsMacrocytosis, reticulocytosis, elevated bilirubin and lactate dehydrogenase levels; spherocytes or elliptocytes on smear; osmotic fragility test is commonly done but not specific
Acquired hemolytic anemiasAntibody-mediated hemolysis, drug-induced hemolysis, hemolytic uremic syndrome, disseminated intravascular coagulationJaundice, fatigue, dyspneaPositive Coombs test and spherocytes visible on smear in antibody-mediated hemolysis; schistocytes visible on smear in hemolytic uremic syndrome or disseminated intravascular coagulation
Transient erythro-blastopenia of childhoodTransient immune reaction against erythroid progenitor cellsAnemia after toxin ingestion or viral illness, usually in children 6 months to 3 years of ageNormocytic anemia, initially with reticulocyte count of 0; anemia resolves within 2 months
Leukemia, myelofibrosisUsually spontaneous, but rates are increased in patients with prior radiation exposure or chemotherapyAnemia causes pallor, fatigue, and dyspnea; patients with leukemia may present with petechiae, low-grade fever, nonspecific bone pain, gum swelling, or rashNormocytic anemia with decreased reticulocyte count; leukopenia, leukocytosis, or thrombocytopenia; peripheral smear shows blast cells
Lead poisoningRisk factors include young age, living in a home built before 1970 or in areas where soil is contaminated, and pica (as in iron deficiency)In addition to anemia, patients may present with abdominal pain, altered mental status, renal disease, and hypertensionMicrocytic anemia may be concurrent with iron deficiency; peripheral smear may show basophilic stippling; hemolysis may be present
Late childhood and adolescence2
Iron deficiencySecond peak in iron deficiency occurs in adolescence because of growth spurt, menstruation, and poor dietary iron intakePallor, fatigue, dyspneaSame as for infants and toddlers, above
Chronic diseaseRenal disease, liver disease, hypothyroidism, other chronic illnessesUsually mild and asymptomaticNormocytic or mildly microcytic, low/normal serum iron level with low transferrin level; ferritin level may be elevated because it is an acute phase reactant
Blood lossSame as for infants and toddlers, above
Menstruation in adolescent girls
Disorders of Hgb synthesis or RBC membrane defectsSame as for infants and toddlers, above
Acquired hemolytic anemiasSame as for infants and toddlers, above
Leukemia and other bone marrow disordersSame as for infants and toddlers, above