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aniline have been reported to cause cyclic or rebound methemoglobinemia. Consultation
with toxicology or poison control is advised.

INEFFECTIVE OR DECREASED RED BLOOD CELL PRODUCTION
CLINICAL PEARLS AND PITFALLS
Decreased RBC production may be due to congenital abnormalities (e.g.,
Diamond–Blackfan anemia [DBA]), marrow infiltrative processes (e.g.,
malignancy, fibrosis, lysosomal storage disease), or acquired insults (e.g.,
viral suppression, toxins, nutritional deficiency).
β-Thalassemia major usually presents only after 2 to 3 months of age with
the switch from γ- to β-globin chain production; it may present with severe
anemia causing a hematologic emergency.
Iron replacement therapy for iron-deficiency anemia consists of 3 to 6
mg/kg/day of elemental iron given orally as ferrous sulfate at night on an
empty stomach (and ideally with ascorbic acid) as a single daily dose. IV or
SQ formulations do not provide a clinical advantage in the absence of
malabsorption.
The goal of transfusion for chronic, severe anemia is relief of symptoms, not
restoration of a normal hemoglobin level. If transfusion is necessary, provide
small aliquots of RBCs (5 mL/kg) slowly with close monitoring and
consideration of furosemide administration.

Current Evidence
Thalassemia
The thalassemias are mutations or deletions of the globin genes that result in reduced or
loss of production of α- or β-globin chains, the building blocks of hemoglobin.
Hemoglobin A (normal adult hemoglobin α2β2) begins rising just prior to birth as the
body switches production from γ- to β-globin chains, and increases steadily over the
first year of life to become the predominant hemoglobin.
The β-thalassemia gene mutations occur most commonly in populations living in
regions bordering the Mediterranean, North Africa, the Middle East, Central and

Southeast Asia, and India. In β-thalassemia, reduced (β+ ) or no (β0) β-globin chains are
produced. β-Thalassemia major phenotype results from β0/β0 or β0/β+ , intermedia
phenotype from β+ /β+ , and carrier state from β/β+ .
In the first year of life, infants with β-thalassemia major usually develop a sallow
complexion, increased fatigue, and poor weight gain and linear growth. Physical
examination shows pallor, icterus, and enlargement of the liver and spleen. In
thalassemia major, the hemoglobin level may decrease to 3 or 4 g/dL, and the mean