MyMedR

Fulltext A case of nasopharyngeal carcinoma with paraneoplastic leukemoid reaction: a case report

Lee DW, Teoh DC, Chong FL

Med J Malaysia, 2015 Apr;70(2):110-1.

We present a case of nasopharyngeal carcinoma complicated with hyperleucocytosis. After ruling our other causes we concluded that the hyperleucocytosis was due to paraneoplastic leukemoid reaction (PLR). The overall survival was 15 months which is rare among patients with PLR.
Keywords: Sabah

Matched MeSH terms: Leukemoid Reaction*

Fulltext Transient abnormal myelopoiesis with life-threatening complications

Abdul-Wahab, J., Naznin, M, Norlelawati, A.T., Amir Hamzah, A.R.

International Medical Journal Malaysia, 2006;5(2):1-6.

MyJurnal

Transient abnormal myelopoiesis (TAM) occurs in approximately 10% of neonates with Down syndrome. In most cases it resolves spontaneously. Life threatening complications such as cardiopulmonary and liver diseases have been described. We present here two cases which suggest that management of TAM in selected cases will have to be more aggressive.

Matched MeSH terms: Leukemoid Reaction

Fulltext GATA1 mutations in a cohort of Malaysian children with Down syndrome-associated myeloid disorder

Lum SH, Choong SS, Krishnan S, Mohamed Z, Ariffin H

Singapore Med J, 2016 Jun;57(6):320-4.

PMID: 27353457 DOI: 10.11622/smedj.2016106

INTRODUCTION: Children with Down syndrome (DS) are at increased risk of developing distinctive clonal myeloid disorders, including transient abnormal myelopoiesis (TAM) and myeloid leukaemia of DS (ML-DS). TAM connotes a spontaneously resolving congenital myeloproliferative state observed in 10%-20% of DS newborns. Following varying intervals of apparent remission, a proportion of children with TAM progress to develop ML-DS in early childhood. Therefore, TAM and ML-DS represent a biological continuum. Both disorders are characterised by recurring truncating somatic mutations of the GATA1 gene, which are considered key pathogenetic events.

METHODS: We herein report, to our knowledge, the first observation on the frequency and nature of GATA1 gene mutations in a cohort of Malaysian children with DS-associated TAM (n = 9) and ML-DS (n = 24) encountered successively over a period of five years at a national referral centre.

RESULTS: Of the 29 patients who underwent GATA1 analysis, GATA1 mutations were observed in 15 (51.7%) patients, including 6 (75.0%) out of 8 patients with TAM, and 9 (42.9%) of 21 patients with ML-DS. All identified mutations were located in exon 2 and the majority were sequence-terminating insertions or deletions (66.7%), including several hitherto unreported mutations (12 out of 15).

CONCLUSION: The low frequency of GATA1 mutations in ML-DS patients is unusual and potentially indicates distinctive genomic events in our patient cohort.

Matched MeSH terms: Leukemoid Reaction/complications; Leukemoid Reaction/genetics*

Progressive pulmonary vein stenosis in Down syndrome infant: a rare cause of pulmonary hypertension

Norazah Z, Joyce Darshinee DS

Med J Malaysia, 2018 04;73(2):119-120.

PMID: 29703879 MyJurnal

Pulmonary veins stenosis in a Down Syndrome infant with normal connection pulmonary vein is rare and precise incidence of this disease is unknown. We report a case of progressive multiple pulmonary vein stenosis in a Down Syndrome infant with congenital heart disease and transient myeloproliferative leukaemia. This case-report aims to improve awareness among physicians and sonographers of this disease and the importance of pulmonary vein assessment in congenital heart disease with unexplained pulmonary hypertension.

Matched MeSH terms: Leukemoid Reaction

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