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Transient abnormal myelopoiesis (TAM) causing multiple organ failure.
Aparna Hoskote1, Judith Chessells, Christine Pierce
1Paediatric Intensive Care Unit, Great Ormond Street Hospital for Children, Great Ormond Street, London, WC1N 3JH, UK. hoskoa@gosh.nhs.uk
Intensive Care Medicine
|July 11, 2002
Summary
Transient Abnormal Myelopoiesis (TAM) in neonates with Down syndrome can be severe and life-threatening, often requiring intensive care. Aggressive treatment is recommended due to high mortality rates in these critical cases.
Area of Science:
- Neonatal Medicine
- Hematology
- Genetics
Background:
- Transient Abnormal Myelopoiesis (TAM) is a congenital myeloproliferative disorder associated with Down syndrome.
- Concerns exist regarding the self-limiting nature of TAM in neonates requiring intensive care.
Observation:
- A retrospective review identified four neonates with TAM and trisomy 21 admitted to the ICU.
- All presented with respiratory failure, pulmonary hypertension, and hyperviscosity within the first week of life.
Findings:
- Three out of four neonates died from multi-organ failure despite aggressive treatment including exchange transfusion and cytarabine.
- Post-mortem examinations revealed extensive thrombo-emboli in the lungs and livers.
Implications:
- TAM in critically ill neonates can lead to severe morbidity and mortality.
- Early suspicion and aggressive treatment (exchange transfusion, chemotherapy) are crucial for hydropic neonates with high white cell counts.
- Further research is needed to identify prognostic markers for TAM outcomes.