Transient myeloproliferative syndrome

Classification according to ICD-10
D47.7	Other specified neoplasms of uncertain or unknown behavior of lymphatic, haematopoietic and related tissues
ICD-10 online (WHO version 2019)

The transient myeloproliferative syndrome (TMD) is a leukemoid response in the newborn . Most of those affected have Down syndrome .

Synonyms are: transient abnormal myelopoiesis; Transient myeloproliferative disease

The first description comes from 1951 by the American pathologist William G. Bernhard and colleagues.

distribution

Changes in the blood count are common in Down syndrome, especially during childhood. The risk of leukemia is 10 to 20 times higher. In addition, TMD often occurs during the newborn period or as a toddler.

root cause

The disease is based on mutations on chromosome 21 locus q11.2.

Clinical manifestations

Clinical criteria are:

TMD develops already in the womb ( intrauterine ), can be diagnosed already then or during the first months of life and regresses spontaneously and completely.

Nevertheless, various organs can be damaged and about 20% can die. Acute myeloid leukemia , megakaryoblastic subtype (AmkL) , will develop in around 10% of those affected with TMD within the next 5 years .

literature

H. Boztug, A. Schumich, U. Pötschger, N. Mühlegger, A. Kolenova, K. Reinhardt, M. Dworzak: Blast cell deficiency of CD11a as a marker of acute megakaryoblastic leukemia and transient myeloproliferative disease in children with and without Down syndrome. In: Cytometry. Part B, Clinical cytometry. Volume 84, number 6, 2013 Nov-Dec, pp. 370-378, doi: 10.1002 / cyto.b.21082 , PMID 23450818 .
ME King, R. Hueckel: Transient myeloproliferative disease: keeping a high index of suspicion. In: Journal of pediatric oncology nursing: official journal of the Association of Pediatric Oncology Nurses. Volume 29, Number 5, 2012 Sep-Oct, pp. 289-293, doi: 10.1177 / 1043454212452340 , PMID 22907684 (review).
NL Seibel, A. Sommer, J. Miser: Transient neonatal leukemoid reactions in mosaic trisomy 21. In: The Journal of pediatrics. Volume 104, Number 2, February 1984, pp. 251-254, doi: 10.1016 / s0022-3476 (84) 81006-9 , PMID 6229618 .

Individual evidence

↑ ^a ^b Transient myeloproliferative syndrome. In: Orphanet (Rare Disease Database).
^ WG Bernhard, I. Gore, RA Kilby: Congenital leukemia. In: Blood. Volume 6, Number 11, November 1951, pp. 990-1001, PMID 14869361 .
↑ Uptodate
↑ Leukemia, transient, of Down syndrome. In: Online Mendelian Inheritance in Man . (English)
↑ JA Tamblyn, A. Norton, L. Spurgeon, V. Donovan, A. Bedford Russell, J. Bonnici, K. Perkins, P. Vyas, I. Roberts, MD Kilby: Prenatal therapy in transient abnormal myelopoiesis: a systematic review . In: Archives of Disease in Childhood. Fetal and neonatal edition. Volume 101, number 1, January 2016, pp. F67-F71, doi: 10.1136 / archdischild-2014-308004 , PMID 25956670 (review).
↑ N. Bhatnagar, L. Nizery, O. Tunstall, P. Vyas, I. Roberts: Transient Abnormal Myelopoiesis and AML in Down Syndrome: an Update. In: Current hematologic malignancy reports. Volume 11, number 5, 10 2016, pp. 333–341, doi: 10.1007 / s11899-016-0338-x , PMID 27510823 , PMC 5031718 (free full text) (review).

[Orpha-1] Transient myeloproliferative syndrome. In: Orphanet (Rare Disease Database).

[2] WG Bernhard, I. Gore, RA Kilby: Congenital leukemia. In: Blood. Volume 6, Number 11, November 1951, pp. 990-1001, PMID 14869361 .

[3] Uptodate

[4] Leukemia, transient, of Down syndrome. In: Online Mendelian Inheritance in Man . (English)

[5] JA Tamblyn, A. Norton, L. Spurgeon, V. Donovan, A. Bedford Russell, J. Bonnici, K. Perkins, P. Vyas, I. Roberts, MD Kilby: Prenatal therapy in transient abnormal myelopoiesis: a systematic review . In: Archives of Disease in Childhood. Fetal and neonatal edition. Volume 101, number 1, January 2016, pp. F67-F71, doi: 10.1136 / archdischild-2014-308004 , PMID 25956670 (review).

[6] N. Bhatnagar, L. Nizery, O. Tunstall, P. Vyas, I. Roberts: Transient Abnormal Myelopoiesis and AML in Down Syndrome: an Update. In: Current hematologic malignancy reports. Volume 11, number 5, 10 2016, pp. 333–341, doi: 10.1007 / s11899-016-0338-x , PMID 27510823 , PMC 5031718 (free full text) (review).