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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.
See: This record
Not genetically inherited (Orphanet)
 
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.
See: Feature record | Search on this feature
Leukocytosis
MedGen UID:
9736
•Concept ID:
C0023518
•
Disease or Syndrome
An abnormal increase in the number of leukocytes in the blood.
See: Feature record | Search on this feature
Show allHide all

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.
See: Condition Record
Transient myeloproliferative syndrome

Professional guidelines

PubMed

Management of Down Syndrome-Associated Leukemias: A Review.
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
Rapid next-generation sequencing aids in diagnosis of transient abnormal myelopoiesis in a phenotypically normal newborn.
Aziz-Bose R, Wachter F, Chiarle R, Lindeman NI, Kim AS, Degar BA, Davies K, Pikman Y
Blood Adv 2022 May 10;6(9):2893-2896. doi: 10.1182/bloodadvances.2021006865. PMID: 35090166Free PMC Article
Guidelines for the investigation and management of Transient Leukaemia of Down Syndrome.
Tunstall O, Bhatnagar N, James B, Norton A, O'Marcaigh AS, Watts T, Greenough A, Vyas P, Roberts I, Wright M; British Society for Haematology
Br J Haematol 2018 Jul;182(2):200-211. Epub 2018 Jun 19 doi: 10.1111/bjh.15390. PMID: 29916557

Recent clinical studies

Etiology

GATA1 mutations in transient leukemia and acute megakaryoblastic leukemia of Down syndrome.
Hitzler JK, Cheung J, Li Y, Scherer SW, Zipursky A
Blood 2003 Jun 1;101(11):4301-4. Epub 2003 Feb 13 doi: 10.1182/blood-2003-01-0013. PMID: 12586620
Myelodysplastic and myeloproliferative disorders of childhood: a study of 167 patients.
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
Infants with Down's syndrome. Use of cytogenetic studies and in vitro colony assay for granulocyte progenitor to distinguish acute nonlymphocytic leukemia from a transient myeloproliferative disorder.
de Alarcon PA, Patil S, Golberg J, Allen JB, Shaw S
Cancer 1987 Sep 1;60(5):987-93. doi: 10.1002/1097-0142(19870901)60:5<987::aid-cncr2820600512>3.0.co;2-m. PMID: 2955884
Transient myeloproliferative syndrome in a phenotypically normal infant.
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

Diagnosis

Two myeloid leukemia cases with rare FLT3 fusions.
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
Myelodysplastic and myeloproliferative disorders of childhood: a study of 167 patients.
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
Down's syndrome with transient myeloproliferative syndrome.
Al Mulhim I
Indian J Pediatr 1990 Mar-Apr;57(2):253-5. doi: 10.1007/BF02722097. PMID: 2147168
Transient myeloproliferative syndrome in a phenotypically normal infant.
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
Congenital leukaemia and the neonatal myeloproliferative disorders associated with Down's syndrome.
Weinstein HJ
Clin Haematol 1978 Feb;7(1):147-54. PMID: 148990

Prognosis

Clinicopathological features of transient myeloproliferative syndrome and congenital leukaemia.
Sajid N, Ahmed N, Mahmood S
J Coll Physicians Surg Pak 2010 Sep;20(9):576-80. PMID: 20810047
Transient myeloproliferative syndrome/transient acute myeloid leukemia in a newborn with Down syndrome: a case report and literature review.
D'Angelo G, Merlo P
Lab Hematol 2003;9(1):38-41. PMID: 12661826
Myelodysplastic and myeloproliferative disorders of childhood: a study of 167 patients.
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
Transient myeloproliferative syndrome in a phenotypically normal infant.
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
Congenital leukaemia and the neonatal myeloproliferative disorders associated with Down's syndrome.
Weinstein HJ
Clin Haematol 1978 Feb;7(1):147-54. PMID: 148990

Clinical prediction guides

Two myeloid leukemia cases with rare FLT3 fusions.
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
Clinicopathological features of transient myeloproliferative syndrome and congenital leukaemia.
Sajid N, Ahmed N, Mahmood S
J Coll Physicians Surg Pak 2010 Sep;20(9):576-80. PMID: 20810047
Transient myeloproliferative syndrome/transient acute myeloid leukemia in a newborn with Down syndrome: a case report and literature review.
D'Angelo G, Merlo P
Lab Hematol 2003;9(1):38-41. PMID: 12661826
Myelodysplastic and myeloproliferative disorders of childhood: a study of 167 patients.
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
Infants with Down's syndrome. Use of cytogenetic studies and in vitro colony assay for granulocyte progenitor to distinguish acute nonlymphocytic leukemia from a transient myeloproliferative disorder.
de Alarcon PA, Patil S, Golberg J, Allen JB, Shaw S
Cancer 1987 Sep 1;60(5):987-93. doi: 10.1002/1097-0142(19870901)60:5<987::aid-cncr2820600512>3.0.co;2-m. PMID: 2955884

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