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Detailed information of TRANSIENT ERYTHROBLASTOPENIA OF CHILDHOOD
TRANSIENT ERYTHROBLASTOPENIA OF CHILDHOOD
DEFINITION:
A disorder characterized by an acquired anemia in a previously
healthy child.
EPIDEMIOLOGY:
- incidence: ? (>400 cases reported)
- more common than Diamond-Blackfan Anemia
- age of onset:
- 90% >1 year of age (only 10% >3 years)
- mean age: 26 months (M) and 29 months (F)
- risk factors:
- see etiologic agents below
- M > F (1.4:1)
PATHOGENESIS:
- exposure to etiologic agent -> humoral or cellular
autoimmune response -> transient immune suppression of
erythropoiesis -> depressed BFU-E and/or CFU-E -> decreased
erythroid progenitors
- autoimmune responses:
- humoral - antibodies to BFU-E and/or CFU-E
- cellular - inhibitory mononuclear cells
- etiologic agents may also directly affect BFU-E and/or CFU-E,
i.e., viruses
CLINICAL FEATURES:
- pallor, lethargy, tachycardia
- gradual onset
- complications: seizures, transient ischemic attacks
2. Others
- no congenital anomalies
- normal height and weight for age
INVESTIGATIONS:
- Hb: normocytic, normochromic (mean = 58 g/L), normal HbF,
low reticulocytes (< 0.5%)
- normal WBC (neutropenia in only 25%), platelet, haptoglobin,
i antigen, serum iron and TIBC, ferritin, osmotic fragility, RBC
adensoine deaminase
- elevated erythropoietin
- recovery stage: elevated HbF, i antigen, macrocytosis
2. Bone Marrow
- transient erythroblastopenia in an otherwise normal bone
marrow
- maturational arrest of erythroblasts
- myeloid/erythroid ratio ranges from 5-100:1
- recovery stage: "stress erythropoiesis"
MANAGEMENT:
- observe
- transfuse only if cardiovascular compromise
- no role for prednisone, anabolic steroids, or other
immunosuppressive therapies
- most patients well within 1-2 months of diagnosis
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Pediatric Database - TRANSIENT ERYTHROBLASTOPENIA OF CHILDHOOD
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