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Transient Abnormal Myelopoiesis: A Varied Spectrum of Clinical Presentation
Amitabh Singh1, Anirban Mandal2, Vijay Guru3
1Department of Pediatrics, Chacha Nehru Bal Chikitsalaya, New Delhi, India.
Abstract:
Transient myeloproliferative disorder (TMD) is a condition seen almost exclusively in newborns with Down syndrome (DS). It can have a spectrum of clinical presentation ranging from being asymptomatic with incidental detection to a stormy course and fatal outcome. We describe three cases of TMD having different clinical presentation, course, complications and outcome. All but one had Down's phenotype; one of them had a severe disease with tumor lysis syndrome and died of liver failure, while the other one had pericardial effusion and cardiac failure but survived. The third patient had a very benign course of illness requiring only supportive care. Newborns with DS should be screened for TMD by a complete blood count during their first month of life, irrespective of symptoms. With increasing knowledge about the natural history and management guidelines, the prognosis of this rare and unique entity has improved in recent years.
Insights
Transient myeloproliferative disorder (TMD) in newborns with Down syndrome (DS) presents variably. Early screening via complete blood count is crucial for improved outcomes in this unique condition.
Area of Science:
- Hematology
- Genetics
- Neonatology
Background:
- Transient myeloproliferative disorder (TMD) is a hematologic condition predominantly affecting newborns with Down syndrome (DS).
- TMD exhibits a wide range of clinical manifestations, from asymptomatic cases to severe, life-threatening presentations.
Observation:
- This report details three cases of TMD in newborns, highlighting diverse clinical presentations, disease courses, and outcomes.
- One patient experienced severe TMD with tumor lysis syndrome and fatal liver failure, while another survived cardiac complications.
- The third case demonstrated a mild course requiring only supportive care.
Findings:
- Newborns with Down syndrome should undergo screening for TMD using a complete blood count within the first month of life.
- Clinical presentation and complications of TMD can vary significantly among affected infants.
Implications:
- Increased awareness and timely screening can lead to better management and improved prognoses for infants with TMD and DS.
- Understanding the spectrum of TMD is essential for appropriate clinical management and patient care.
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