Preleukaemic disorders in children: Hereditary disorders and myelodysplastic syndrome

• Published in: Current diagnostic pathology Vol. 4; no. 1; pp. 45 - 50
• Main Author: van den Tweel, J.G.
• Format: Journal Article
• Language: English
• Published: Elsevier Ltd 1997
• ISSN: 0968-6053; 1532-2041
• DOI: 10.1016/S0968-6053(97)80007-5

The diagnosis of preleukaemic disorders in childhood is often a difficult diagnostic problem. Several factors contribute to the complexity of making such a diagnosis. Firstly, most pathologists have little experience with paediatric bone marrows and there is little information about the normal histology of bone marrows in new born and young children. A second factor of importance is ignorance about the spectrum of diseases in young children that can develop into leukaemia; most of these diseases are very rare and nearly never seen outside specialized children hospitals. A third factor complicating the diagnosis of these diseases is that the bone marrow changes are sometimes very subtle and difficult to diagnose. This paper describes the three main groups of preleukaemic disorders in children. The first group includes the hereditary preleukaemic disorders which are represented both by single-cell cytopenias as well as pancytopenias. The second group comprises the primary myelodysplastic syndromes which include all the adult forms of the FAB-classification as well as juvenile chronic myeloid leukaemia and infantile monosomy 7. The third group are those children who developed a secondary myelodysplasia as a late complication of radiotherapy and/or chemotherapy. The different disease entities within each of these groups are described and criteria for their diagnoses are discussed.