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Transient myeloproliferative syndrome(MST)

MedGen UID:
331782
Concept ID:
C1834582
Finding; Neoplastic Process
Synonyms: LEUKEMIA, TRANSIENT; MST; TRANSIENT ABNORMAL MYELOPOIESIS
Modes of inheritance:
not inherited
MedGen UID:
832438
Concept ID:
CN227390
Intellectual Product
Source: Orphanet
Describes a disorder that is not inherited.
not inherited (Orphanet)
SNOMED CT: Transient abnormal myelopoiesis (450934005); Transient abnormal myelopoiesis (721307000)
 
Cytogenetic location: 21q11.2
OMIM®: 159595
HPO: HP:0005534
Orphanet: ORPHA420611

Definition

A myeloid proliferation occurring in newborns with Down syndrome. It is clinically and morphologically indistinguishable from acute myeloid leukemia and is associated with GATA1 mutations. The blasts display morphologic and immunophenotypic features of megakaryocytic lineage. In the majority of patients the myeloid proliferation undergoes spontaneous remission. [from NCI]

Clinical features

Leukocytosis
MedGen UID:
9736
Concept ID:
C0023518
Disease or Syndrome
A transient increase in the number of leukocytes in a body fluid.
Transient myeloproliferative syndrome
MedGen UID:
331782
Concept ID:
C1834582
Neoplastic Process
A myeloid proliferation occurring in newborns with Down syndrome. It is clinically and morphologically indistinguishable from acute myeloid leukemia and is associated with GATA1 mutations. The blasts display morphologic and immunophenotypic features of megakaryocytic lineage. In the majority of patients the myeloid proliferation undergoes spontaneous remission.

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 myeloid proliferation occurring in newborns with Down syndrome. It is clinically and morphologically indistinguishable from acute myeloid leukemia and is associated with GATA1 mutations. The blasts display morphologic and immunophenotypic features of megakaryocytic lineage. In the majority of patients the myeloid proliferation undergoes spontaneous remission.

Recent clinical studies

Etiology

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
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
de Alarcon PA, Patil S, Golberg J, Allen JB, Shaw S
Cancer 1987 Sep 1;60(5):987-93. PMID: 2955884
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

Sajid N, Ahmed N, Mahmood S
J Coll Physicians Surg Pak 2010 Sep;20(9):576-80. doi: 09.2010/JCPSP.576580. PMID: 20810047
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
Al Mulhim I
Indian J Pediatr 1990 Mar-Apr;57(2):253-5. PMID: 2147168
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
Morgan R, Hecht F, Cleary ML, Sklar J, Link MP
Blood 1985 Dec;66(6):1466-8. PMID: 2415189

Prognosis

Sajid N, Ahmed N, Mahmood S
J Coll Physicians Surg Pak 2010 Sep;20(9):576-80. doi: 09.2010/JCPSP.576580. 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

Sajid N, Ahmed N, Mahmood S
J Coll Physicians Surg Pak 2010 Sep;20(9):576-80. doi: 09.2010/JCPSP.576580. 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
de Alarcon PA, Patil S, Golberg J, Allen JB, Shaw S
Cancer 1987 Sep 1;60(5):987-93. PMID: 2955884
Denegri JF, Rogers PC, Chan KW, Sadoway J, Thomas JW
Blood 1981 Oct;58(4):675-7. PMID: 6456025

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