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Paris-Trousseau thrombocytopenia(TCPT)

MedGen UID:
365037
Concept ID:
C1956093
Disease or Syndrome
Synonyms: CHROMOSOME 11q23 DELETION SYNDROME; Paris-Trousseau syndrome; TCPT
 
Monarch Initiative: MONDO:0008557
OMIM®: 188025
Orphanet: ORPHA851

Definition

Paris-Trousseau thrombocytopenia (TCPT) is a contiguous gene syndrome characterized by mild bleeding tendency, variable thrombocytopenia (THC), dysmorphic facies, abnormal giant alpha-granules in platelets and dysmegakaryopoiesis. [from ORDO]

Clinical features

From HPO
Radial deviation of finger
MedGen UID:
322852
Concept ID:
C1836189
Finding
Bending or curvature of a finger toward the radial side (i.e., towards the thumb). The deviation is at the metacarpal-phalangeal joint, and this finding is distinct from clinodactyly.
Clinodactyly
MedGen UID:
1644094
Concept ID:
C4551485
Congenital Abnormality
An angulation of a digit at an interphalangeal joint in the plane of the palm (finger) or sole (toe).
Congenital hypertrophic pyloric stenosis
MedGen UID:
196010
Concept ID:
C0700639
Congenital Abnormality
Pyloric stenosis, also known as infantile hypertrophic pyloric stenosis, is an uncommon condition in infants characterized by abnormal thickening of the pylorus muscles in the stomach leading to gastric outlet obstruction. Clinically infants are well at birth. Then, at 3 to 6 weeks of age, the infants present with projectile vomiting, potentially leading to dehydration and weight loss.
Intellectual disability
MedGen UID:
811461
Concept ID:
C3714756
Mental or Behavioral Dysfunction
Intellectual disability, previously referred to as mental retardation, is characterized by subnormal intellectual functioning that occurs during the developmental period. It is defined by an IQ score below 70.
Thrombocytopenia
MedGen UID:
52737
Concept ID:
C0040034
Disease or Syndrome
A reduction in the number of circulating thrombocytes.
Prolonged bleeding time
MedGen UID:
56231
Concept ID:
C0151529
Finding
Prolongation of the time taken for a standardized skin cut of fixed depth and length to stop bleeding.
Abnormal bleeding
MedGen UID:
264316
Concept ID:
C1458140
Pathologic Function
An abnormal susceptibility to bleeding, often referred to as a bleeding diathesis. A bleeding diathesis may be related to vascular, platelet and coagulation defects.
Micrognathia
MedGen UID:
44428
Concept ID:
C0025990
Congenital Abnormality
Developmental hypoplasia of the mandible.
Trigonocephaly
MedGen UID:
82713
Concept ID:
C0265535
Congenital Abnormality
Wedge-shaped, or triangular head, with the apex of the triangle at the midline of the forehead and the base of the triangle at the occiput.
Ptosis
MedGen UID:
2287
Concept ID:
C0005745
Disease or Syndrome
The upper eyelid margin is positioned 3 mm or more lower than usual and covers the superior portion of the iris (objective); or, the upper lid margin obscures at least part of the pupil (subjective).

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVParis-Trousseau thrombocytopenia

Professional guidelines

PubMed

Gruel Y, De Maistre E, Pouplard C, Mullier F, Susen S, Roullet S, Blais N, Le Gal G, Vincentelli A, Lasne D, Lecompte T, Albaladejo P, Godier A; Members of the French Working Group on Perioperative Haemostasis Groupe d’intérêt en hémostase périopératoire GIHP
Anaesth Crit Care Pain Med 2020 Apr;39(2):291-310. Epub 2020 Apr 13 doi: 10.1016/j.accpm.2020.03.012. PMID: 32299756
Leblanc C, Vasse C, Minodier P, Mornand P, Naudin J, Quinet B, Siriez JY, Sorge F, de Suremain N, Thellier M, Kendjo E, Faye A, Imbert P
Med Mal Infect 2020 Mar;50(2):127-140. Epub 2019 Mar 15 doi: 10.1016/j.medmal.2019.02.005. PMID: 30885541
Donadieu J, Fenneteau O, Beaupain B, Mahlaoui N, Chantelot CB
Orphanet J Rare Dis 2011 May 19;6:26. doi: 10.1186/1750-1172-6-26. PMID: 21595885Free PMC Article

Recent clinical studies

Etiology

Stevenson WS, Rabbolini DJ, Beutler L, Chen Q, Gabrielli S, Mackay JP, Brighton TA, Ward CM, Morel-Kopp MC
Blood 2015 Oct 22;126(17):2027-30. Epub 2015 Aug 27 doi: 10.1182/blood-2015-06-650887. PMID: 26316623

Diagnosis

Smith MP, Cramer EM, Savidge GF
Baillieres Clin Haematol 1997 Feb;10(1):125-48. doi: 10.1016/s0950-3536(97)80054-6. PMID: 9154319

Prognosis

Stevenson WS, Rabbolini DJ, Beutler L, Chen Q, Gabrielli S, Mackay JP, Brighton TA, Ward CM, Morel-Kopp MC
Blood 2015 Oct 22;126(17):2027-30. Epub 2015 Aug 27 doi: 10.1182/blood-2015-06-650887. PMID: 26316623

Clinical prediction guides

Stevenson WS, Rabbolini DJ, Beutler L, Chen Q, Gabrielli S, Mackay JP, Brighton TA, Ward CM, Morel-Kopp MC
Blood 2015 Oct 22;126(17):2027-30. Epub 2015 Aug 27 doi: 10.1182/blood-2015-06-650887. PMID: 26316623

Supplemental Content

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