A Case Report of Neonatal Death due to Transient Myeloproliferative Disorder with Hepatic Fibrosis in Trisomy 21

Zhang X* and Zhao W

Zhongda Hospital, Southeast University, China

^*Correspondance to: Xiaoyun Zhang 

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Abstract:

Objective: We present a case of neonatal death due to transient myeloproliferative disorder with hepatic fibrosis in trisomy 21. Case Report: Transient Myelodysplastic Disorder (TMD) occurs in 3% to 10% of newborns with trisomy 21 (DS) and is characterized by clonal proliferation of maternal cells in the blood/bone marrow, with most newborns being asymptomatic. However, in rare cases, die prematurely from organ failure, including liver fibrosis. In this report, we present a non-dysmorphic neonate, with a negative noninvasive prenatal screening of maternal blood for trisomy 21, who with ruddy skin and good birth score then he was dead within one day genetic testing suggesting trisomy 21 chimerism (mos, x3, ~50%) and an autopsy pathology report suggesting extramedullary hemopoiesis and liver fibrosis. Conclusion: Transient myeloproliferative disorder with hepatic fibrosis in trisomy 21 is an uncommon clinical condition in which accurate diagnosis is difficult and Sudden onset. With the increasing prevalence of NIPT detection, clinicians need to be vigilant about chromosome chimerism, and pay attention to the occurrence of liver fibrosis and DIC when neonatal test indicators indicate extramedullary hematopoiesis.

Keywords:

21-trisomy; Transient myeloproliferative disorder; Liver fibrosis; Case Report

Cite the Article:

Zhang X, Zhao W. A Case Report of Neonatal Death due to Transient Myeloproliferative Disorder with Hepatic Fibrosis in Trisomy 21. Clin Case Rep Int. 2024; 8: 1704..