Pure Red Cell Aplasia |
Cause | Clinical Findings | Laboratory Findings | Treatment |
Diamond-Blackfan anemia (DBA) | Typically occurs in infancyCongenital abnormalitiesgrowth retardationcraniofacial abnormalitiese.g., hypertelorism and a flat nasal bridgethumb abnormalitiese.g., triphalangeal thumbsPredisposition to canceracute myelogenous leukemiamyelodysplastic syndromeEtiologycongenital impairments affecting ribosome synthesis | Macrocytic, normochromic, and non-megaloblastic anemiaReticulocytopeniaIncreased HbF | Corticosteroids and blood transfusionsHematopoietic cell transplantation in patients unresponsive to steroids |
Transient erythroblastopenia of childhood (TEC) | Transient/temporary red cell aplasiaDBA is chronicThe child is otherwise healthyEtiologyunknown | Normocytic anemia at the time of diagnosisReticulocytopenia | Patients typically recover 1-2 months without treatmentRBC transfusions may be needed for disabling symptoms |
Parvovirus B19 | Patients with an underlying hemolytic process may experience an aplastic crisis with parvovirus B19 infectione.g., sickle cell anemia, hereditary spherocytosis, and pyruvate kinase deficiencyEtiologyviral infection of erythroid progenitors via a P-antigen receptor resulting in lytic destruction of proerythroblasts | Proerythroblasts and absent erythroid precursors may be seen in the bone marrow | Otherwise healthy children do not require treatmentPatients with an underlying hemolytic process receive RBC transfusions for symptomatic anemia |
Paraneoplastic syndrome | Hemoglobin decline in a patient who has not had a transfusion and previously responded to erythropoietinEtiologyunderlying malignancy can result in the production of anti-erythropoietin antibodiesmalignanciesthymomamyelodysplastic syndromes | Bone marrow aspiratesevere erythroid hypoplasiavery little RBC precursorsPresent anti-erythropoietin antibodies | Transfusions for symptomatic anemiaDiscontinue recombinant erythropoietin (EPO) productsImmunosuppression |