期刊
INTERNATIONAL JOURNAL OF HEMATOLOGY
卷 113, 期 5, 页码 662-667出版社
SPRINGER JAPAN KK
DOI: 10.1007/s12185-020-03066-7
关键词
Transient leukemia; Transient abnormal myelopoiesis; Low blast; Acute megakaryoblastic leukemia; Liver failure
类别
资金
- Health and Labor Sciences Research Grants [201128038B, 201324122A]
- Ministry of Health, Labor, and Welfare of Japan
- Ministry of Education, Culture, Sports, Science, and Technology of Japan
- Japan Agency for Medical Research and Development (AMED) (Innovative Cancer Medical Practice Research Project) [16ek0109055h0003, 18ck0106435h0001]
Transient abnormal myelopoiesis (TAM) is a clonal myeloproliferation that occurs in 5-10% of neonates with Down syndrome, with a potential risk of developing acute megakaryoblastic leukemia. Low blast percentage TAM cases may be at risk of liver fibrosis and liver failure. Patients with TAM should be carefully monitored for liver disease and leukemia.
Transient abnormal myelopoiesis (TAM) is a unique clonal myeloproliferation characterized by immature megakaryoblasts that occurs in 5-10% of neonates with Down syndrome (DS). Although TAM regresses spontaneously in most patients, approximately 20% of TAM cases result in early death, and approximately 20% of survivors develop acute megakaryoblastic leukemia (AMKL). We retrospectively reviewed records of 35 DS patients with TAM to determine the correlation between clinical characteristics and blast percentage. Thirteen of the 35 patients were classified as low blast percentage TAM (LBP-TAM), defined as TAM with a peak peripheral blast percentage <= 10%. Although no patient with LBP-TAM experienced systemic edema, disseminated intravascular coagulation, or early death, eight patients had elevated direct bilirubin levels (> 2 mg/dl) and one developed AMKL. All patients with LBP-TAM had serum markers of liver fibrosis that exceeded the normal limits, and two patients underwent liver biopsy to clarify the etiology of pathological jaundice. Taken together, our results suggest that patients with LBP-TAM may be at risk of liver fibrosis and liver failure, similarly to patients with classical TAM. Although these patients generally have a good prognosis, they should be carefully monitored for potential development of liver disease and leukemia.
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