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Amegakaryocytosis

MedGen UID:
362704
•Concept ID:
C1879691
•
Disease or Syndrome

Definition

A non-neoplastic, congenital or non-congenital disorder charcaterized by marked reduction or absence of megakaryocytes and thrombocytopenia. [from NCI]

Professional guidelines

PubMed

Plasma thrombopoietin levels as additional tool in clinical management of thrombocytopenic neonates.
Porcelijn L, Huiskes E, Onderwater-Van Den Hoogen L, Folman CC, Zwaginga JJ, De Haas M
Platelets 2020;31(1):62-67. Epub 2019 Feb 13 doi: 10.1080/09537104.2019.1572877. PMID: 30759044

Recent clinical studies

Etiology

The expression of cytokine and cytokine receptor genes in long-term bone marrow culture in congenital and acquired bone marrow hypoplasias.
Stark R, Andre C, Thierry D, Cherel M, Galibert F, Gluckman E
Br J Haematol 1993 Apr;83(4):560-6. doi: 10.1111/j.1365-2141.1993.tb04691.x. PMID: 7686032
Bone marrow in megakaryocytic disorders.
Burkhardt R
Hematol Oncol Clin North Am 1988 Dec;2(4):695-733. PMID: 3065325

Diagnosis

Plasma thrombopoietin levels as additional tool in clinical management of thrombocytopenic neonates.
Porcelijn L, Huiskes E, Onderwater-Van Den Hoogen L, Folman CC, Zwaginga JJ, De Haas M
Platelets 2020;31(1):62-67. Epub 2019 Feb 13 doi: 10.1080/09537104.2019.1572877. PMID: 30759044
Neonatal Alloimmune Thrombocytopenia With Amegakaryocytosis, B Lymphopenia, and Villitis.
Dony A, Buenerd A, Pondarre C
Pediatrics 2018 Apr;141(Suppl 5):S506-S509. doi: 10.1542/peds.2016-1340. PMID: 29610181
Hematogones in immune thrombocytopenic purpura: diagnostic implication.
Akyay A, Falay M, Oztürkmen S, Biçakci Z, Tavil B, Ozet G, Olcay L
Turk J Pediatr 2011 Mar-Apr;53(2):219-24. PMID: 21853665
Inherited thrombocytopenia: Congenital amegakaryocytic thrombocytopenia and thrombocytopenia with absent radii.
Geddis AE
Semin Hematol 2006 Jul;43(3):196-203. doi: 10.1053/j.seminhematol.2006.04.003. PMID: 16822462

Therapy

Plasma thrombopoietin levels as additional tool in clinical management of thrombocytopenic neonates.
Porcelijn L, Huiskes E, Onderwater-Van Den Hoogen L, Folman CC, Zwaginga JJ, De Haas M
Platelets 2020;31(1):62-67. Epub 2019 Feb 13 doi: 10.1080/09537104.2019.1572877. PMID: 30759044
Neonatal alloimmune amegakaryocytosis. Case report.
Bizzaro N, Dianese G
Vox Sang 1988;54(2):112-4. doi: 10.1111/j.1423-0410.1988.tb01627.x. PMID: 3376462

Prognosis

Plasma thrombopoietin levels as additional tool in clinical management of thrombocytopenic neonates.
Porcelijn L, Huiskes E, Onderwater-Van Den Hoogen L, Folman CC, Zwaginga JJ, De Haas M
Platelets 2020;31(1):62-67. Epub 2019 Feb 13 doi: 10.1080/09537104.2019.1572877. PMID: 30759044

Clinical prediction guides

Plasma thrombopoietin levels as additional tool in clinical management of thrombocytopenic neonates.
Porcelijn L, Huiskes E, Onderwater-Van Den Hoogen L, Folman CC, Zwaginga JJ, De Haas M
Platelets 2020;31(1):62-67. Epub 2019 Feb 13 doi: 10.1080/09537104.2019.1572877. PMID: 30759044

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