A fetal case of transient abnormal myelopoiesis with severe liver failure in Down syndrome: prognostic value of serum markers.

Abstract

The authors recently encountered a lethal case of Down syndrome with transient abnormal myelopoiesis (TAM). Although the peripheral white blood cell count and blast cells had improved without specific treatment, the patient died of severe coagulopathy due to liver fibrosis when he was 5 years old. The prognosis of TAM with liver fibrosis was poor. The patient had high levels of N-terminal peptide of III procollagen, type IV collagen, and hyaluronic acid. These serum makers are noninvasive indicators of liver fibrosis and may be useful as prognostic indicators of TAM in Down syndrome.

• Authors: Yusuke Shiozawa, Hiroo Fujita, Junya Fujimura, Kyoko Suzuki, Hiroaki Sato, Masahiro Saito, Toshiaki Shimizu, Yuichiro Yamashiro
• Journal: Pediatric hematology and oncology (Pediatr Hematol Oncol) 2004 Apr-May Vol. 21 Issue 3 Pg. 273-8 ISSN: 0888-0018 [Print] England
• PMID: 15202167 (Publication Type: Case Reports, Journal Article, Review)

Chemical References

• Biomarkers
• Collagen Type IV
• Peptide Fragments
• Procollagen
• procollagen Type III-N-terminal peptide
• Hyaluronic Acid

Topics

• Biomarkers (blood)
• Collagen Type IV (blood)
• Disseminated Intravascular Coagulation
• Down Syndrome (blood, complications, diagnosis)
• Fatal Outcome
• Female
• Fetal Diseases (blood, diagnosis)
• Humans
• Hyaluronic Acid (blood)
• Infant, Newborn
• Liver Cirrhosis (blood, diagnosis, etiology)
• Liver Failure (blood, diagnosis, etiology)
• Male
• Myelopoiesis
• Peptide Fragments (blood)
• Pregnancy
• Procollagen (blood)
• Prognosis