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Transient myeloproliferative syndrome(TAM)

MedGen UID:
331782
Concept ID:
C1834582
Finding; Neoplastic Process
Synonyms: LEUKEMIA, TRANSIENT; TRANSIENT ABNORMAL MYELOPOIESIS; Transient myeloproliferative syndrome (disease)
SNOMED CT: Transient abnormal myelopoiesis (450934005); Transient abnormal myelopoiesis (721307000)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Gene (location): GATA1 (Xp11.23)
 
HPO: HP:0005534
Monarch Initiative: MONDO:0008040
OMIM®: 159595
Orphanet: ORPHA420611

Definition

A unique clonal neoplastic disorder that is linked to trisomy 21, is restricted to neonatal period, and spontaneously regresses. It often has characteristics of megakaryocytic lineage and is associated with GATA1 mutations in myeloblasts. [from HPO]

Clinical features

From HPO
Transient myeloproliferative syndrome
MedGen UID:
331782
Concept ID:
C1834582
Neoplastic Process
A unique clonal neoplastic disorder that is linked to trisomy 21, is restricted to neonatal period, and spontaneously regresses. It often has characteristics of megakaryocytic lineage and is associated with GATA1 mutations in myeloblasts.
Leukocytosis
MedGen UID:
9736
Concept ID:
C0023518
Disease or Syndrome
An abnormal increase in the number of leukocytes in the blood.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTransient myeloproliferative syndrome

Conditions with this feature

Transient myeloproliferative syndrome
MedGen UID:
331782
Concept ID:
C1834582
Neoplastic Process
A unique clonal neoplastic disorder that is linked to trisomy 21, is restricted to neonatal period, and spontaneously regresses. It often has characteristics of megakaryocytic lineage and is associated with GATA1 mutations in myeloblasts.

Professional guidelines

PubMed

Custo S, Tabone E, Aquilina A, Gatt A, Riva N
Hamostaseologie 2024 Aug;44(4):242-254. Epub 2024 Feb 14 doi: 10.1055/a-2232-5480. PMID: 38354834
Verma A, Lupo PJ, Shah NN, Hitzler J, Rabin KR
JAMA Oncol 2023 Sep 1;9(9):1283-1290. doi: 10.1001/jamaoncol.2023.2163. PMID: 37440251
Michiels JJ, Budde U, van der Planken M, van Vliet HH, Schroyens W, Berneman Z
Best Pract Res Clin Haematol 2001 Jun;14(2):401-36. doi: 10.1053/beha.2001.0141. PMID: 11686107

Recent clinical studies

Prognosis

Sajid N, Ahmed N, Mahmood S
J Coll Physicians Surg Pak 2010 Sep;20(9):576-80. PMID: 20810047
D'Angelo G, Merlo P
Lab Hematol 2003;9(1):38-41. PMID: 12661826
Luna-Fineman S, Shannon KM, Atwater SK, Davis J, Masterson M, Ortega J, Sanders J, Steinherz P, Weinberg V, Lange BJ
Blood 1999 Jan 15;93(2):459-66. PMID: 9885207
Hanna MD, Melvin SL, Dow LW, Williams D, Dahl G, Mirro J
Am J Pediatr Hematol Oncol 1985 Spring;7(1):79-81. PMID: 4037246
Weinstein HJ
Clin Haematol 1978 Feb;7(1):147-54. PMID: 148990

Clinical prediction guides

Zhang H, Paliga A, Hobbs E, Moore S, Olson S, Long N, Dao KT, Tyner JW
Cold Spring Harb Mol Case Stud 2018 Dec;4(6) Epub 2018 Dec 17 doi: 10.1101/mcs.a003079. PMID: 30559310Free PMC Article
Sajid N, Ahmed N, Mahmood S
J Coll Physicians Surg Pak 2010 Sep;20(9):576-80. PMID: 20810047
D'Angelo G, Merlo P
Lab Hematol 2003;9(1):38-41. PMID: 12661826
Luna-Fineman S, Shannon KM, Atwater SK, Davis J, Masterson M, Ortega J, Sanders J, Steinherz P, Weinberg V, Lange BJ
Blood 1999 Jan 15;93(2):459-66. PMID: 9885207
Denegri JF, Rogers PC, Chan KW, Sadoway J, Thomas JW
Blood 1981 Oct;58(4):675-7. PMID: 6456025

Supplemental Content

Table of contents

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