Chronic anemia has no precise definition. Anemia that persists for 6 months or more (eg, hereditary spherocytosis [HS]) is clearly chronic; however, anemia that lasts only 2 months (eg, iron deficiency that is being treated) should also be considered chronic anemia, and other explanations must be sought.

Chronic anemia can be primary or secondary.

Primary chronic anemia

Primary chronic anemias are the true chronic anemias, in which anemia (defined as a hemoglobin level more than 2 standard deviations below the mean reference value for age) is part of the basic disease process. The basic disease process is hematologic (eg, sickle cell disease, HS), and the degree of anemia varies markedly from etiology to etiology and from patient to patient, even with the same etiology. (See Etiology and Workup.)

Secondary chronic anemia

Secondary chronic anemias are chronic anemias that may provide a diagnostic clue to an underlying pathology. They are the consequence of a nonhematologic problem (eg, chronic blood loss, chronic renal failure, osteomyelitis, inflammatory bowel disease, tuberculosis).

Etiology

Chronic anemia is classified into the following 3 primary categories:

  • Decreased red cell production
  • Increased red cell destruction (hemolysis)
  • Blood loss

Clinical Presentation

Patients with chronic anemia are usually asymptomatic, even with remarkably low levels of hemoglobin. Symptoms more often relate to the underlying cause.

Hemoglobin levels as low as 5-6 g/dL are well tolerated in most patients, and patients do not require transfusion. Parents, however, frequently note that patients become much more active following a transfusion.

Inquire carefully regarding any evidence of blood loss (eg, hemoptysis, hematochezia, melina, hematuria, menorrhagia). In endemic areas, a history of papulovesicular skin lesions on the feet may suggest a diagnosis of hookworm infestation.

Age is always an important consideration. Nutritional iron deficiency is seen in older infants and toddlers (aged 6 mo to 3 y), whereas iron deficiency due to blood loss occurs in menstruating girls. The deficiency can be surprisingly severe, but transfusion is indicated only in the rare circumstance of impending high-output cardiac failure.

The patient’s sex must always be considered in hemolytic anemias. Severe G-6-PD deficiency may be seen as a chronic nonspherocytic anemia, usually in males.

Dietary history is important with regard to the amount and source of milk ingested by infants and toddlers and to their risk of chronic iron deficiency (24 oz of milk/d or more is a clear risk factor for nutritional iron deficiency in infants and young children). Continue reading »

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Acquired PRCA of the adult type may be idiopathic or related to drugs, particularly phenytoin and chloramphenicol. Immune-mediated PRCA occurs in many idiopathic cases as well as those with thymoma, systemic lupus erythematosus, and chronic lymphocytic leukemia. In vitro erythroid colony assays may reveal the presence of serum lymphocyte inhibitors of erythropoiesis, and about two-thirds will respond to immunosuppression or cytotoxic agents. RBC transfusions are the primary treatment.

B19 parvovirus infects erythroid progenitors and causes transient or chronic RBC aplasia. The chronic type occurs because of persistence of parvovirus in immunodeficient patients who cannot produce neutralizing antibody. The patients have severe transfusion-dependent anemia, and bone marrows show reduced erythroid precursors. The few RBC precursors are giant pronormoblasts. Diagnosis requires demonstration of parvovirus genome (DNA) in serum, blood, or bone marrow cells. Treatment with intravenous γ-globulin (IVIgG) is usually effective.
Transient aplastic crisis (TAC) caused by parvovirus occurs only once in patients with underlying hemolytic anemias . Occasionally, neutrophils and platelets will also be decreased. Diagnostic levels of IgM antibody appear in the first week after infection. In utero infection with parvovirus results in up to 10% fetal death during the first and second trimesters, and neonatal hydrops fetalis occurs occasionally.
Transient erythroblastopenia of childhood (TEC) is an acquired condition in previously hematologically normal children and usually involves only anemia, reticulocytopenia, and marrow erythroblastopenia. The mean age of diagnosis is 26 months, with the majority between 1 and 3 years of age. Although many patients have had an antecedent viral illness, no specific virus has been implicated, and parvovirus has usually been excluded. Pallor and tachycardia are the only relevant findings. The anemia may require one or two transfusions, but patients usually recover spontaneously within 1 to 2 months. Bone marrow shows erythroid hypoplasia, and Continue reading »
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Lymphocytes account for about 30% of the circulating WBCs in a newborn. The proportion of lymphocytes then increases rapidly within the 1st mo, reaching an average of 60% by 2 yr of age. The normal lymphocyte count in children <2 yr of age is 3,000–9,500/?L and in adults is 1,000–4,800/?L. At 6 yr of age, the lower limit of normal is 1,500/?L.

Almost 65% of blood T lymphocytes are CD4 (helper) T lymphocytes. Most patients with lymphocytopenia have a reduction in the absolute number of T lymphocytes, particularly in the number of CD4 T lymphocytes. The average number of CD4 T lymphocytes in adult blood is 1,100/?L (range, 300–1,300/?L), and the average number of CD8 (suppressor) T lymphocytes is 600/?L (range, 100–900/?L), with the normal CD4:CD8 ratio of 1.8–2.0.

Lymphocytopenia by itself usually causes no symptoms and is often detected in the evaluation of other illnesses, particularly recurrent viral, fungal, and parasitic infections. Lymphocyte subpopulations can be measured by multiparameter flow cytometry, which uses the pattern of antigen expression to classify and characterize these cells.

INHERITED CAUSES OF LYMPHOCYTOPENIA

Inherited immunodeficiency disorders may have a quantitative or qualitative stem cell abnormality resulting in ineffective lymphocytopoiesis. Other disorders such as Wiskott-Aldrich Continue reading »

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