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Myelodysplastic Syndrome with Excess Blasts and Fibrosis

MedGen UID:
417429
•Concept ID:
C2826322
•
Neoplastic Process
Synonym: RAEB with Fibrosis

Definition

Myelodysplastic syndrome with excess blasts associated with significant reticulin fibrosis of the bone marrow. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMyelodysplastic Syndrome with Excess Blasts and Fibrosis

Professional guidelines

PubMed

Treatment-related leukemia in Hodgkin's disease: a multi-institution study on 75 cases.
Brusamolino E, Papa G, Valagussa P, Mandelli F, Bernasconi C, Marmont A, Bonadonna G, Tura S, Bosi A, Mango G
Hematol Oncol 1987 Apr-Jun;5(2):83-98. doi: 10.1002/hon.2900050203. PMID: 2439431

Recent clinical studies

Etiology

Primary myelodysplastic syndrome: clinical spectrum of 53 cases.
Irfan M, Kakepoto GN, Khursheed M
J Pak Med Assoc 1998 Mar;48(3):69-73. PMID: 9783031
Multiple autoimmune events after autologous bone marrow transplantation.
Lambertenghi-Deliliers GL, Annaloro C, Della Volpe A, Oriani A, Pozzoli E, Soligo D
Bone Marrow Transplant 1997 Apr;19(7):745-7. doi: 10.1038/sj.bmt.1700711. PMID: 9156254
Acute leukemia and myelodysplasia in polycythemia vera. A clinical study with long-term follow-up.
Najean Y, Deschamps A, Dresch C, Daniel MT, Rain JD, Arrago JP
Cancer 1988 Jan 1;61(1):89-95. doi: 10.1002/1097-0142(19880101)61:1<89::aid-cncr2820610115>3.0.co;2-0. PMID: 3334954
Treatment-related leukemia in Hodgkin's disease: a multi-institution study on 75 cases.
Brusamolino E, Papa G, Valagussa P, Mandelli F, Bernasconi C, Marmont A, Bonadonna G, Tura S, Bosi A, Mango G
Hematol Oncol 1987 Apr-Jun;5(2):83-98. doi: 10.1002/hon.2900050203. PMID: 2439431

Diagnosis

Acquisition of a BCR-ABL1 transcript in a patient with disease progression from MDS with fibrosis to AML with myelodysplasia-related changes.
Park SJ, Lee HW, Jeong SH, Park JS, Kim HC, Seok JY, Kim HJ, Cho SR
Ann Clin Lab Sci 2011 Fall;41(4):379-84. PMID: 22166509
A case of myelodysplastic syndrome in a liver transplant patient.
Potru R, Ahn J, Fung H, Cohen SM
Transplant Proc 2009 Nov;41(9):3947-8. doi: 10.1016/j.transproceed.2009.02.087. PMID: 19917420
Cytogenetic and myelodysplastic alterations after autologous hemopoietic stem cell transplantation.
Lambertenghi Deliliers G, Annaloro C, Pozzoli E, Oriani A, Della Volpe A, Soligo D, Lambertenghi Deliliers D, Tagliaferri E, Bertolli V, Romitti L
Leuk Res 1999 Mar;23(3):291-7. doi: 10.1016/s0145-2126(98)00139-8. PMID: 10071084
Primary myelodysplastic syndrome: clinical spectrum of 53 cases.
Irfan M, Kakepoto GN, Khursheed M
J Pak Med Assoc 1998 Mar;48(3):69-73. PMID: 9783031
5q- in a child with refractory anemia with excess blasts: similarities to 5q- syndrome in adults.
Antillón F, Raimondi SC, Fairman J, Liang H, Nagarajan L, Head D, Ribeiro RC
Cancer Genet Cytogenet 1998 Sep;105(2):119-22. doi: 10.1016/s0165-4608(98)00019-3. PMID: 9723027

Therapy

A case of myelodysplastic syndrome in a liver transplant patient.
Potru R, Ahn J, Fung H, Cohen SM
Transplant Proc 2009 Nov;41(9):3947-8. doi: 10.1016/j.transproceed.2009.02.087. PMID: 19917420
Cytogenetic and myelodysplastic alterations after autologous hemopoietic stem cell transplantation.
Lambertenghi Deliliers G, Annaloro C, Pozzoli E, Oriani A, Della Volpe A, Soligo D, Lambertenghi Deliliers D, Tagliaferri E, Bertolli V, Romitti L
Leuk Res 1999 Mar;23(3):291-7. doi: 10.1016/s0145-2126(98)00139-8. PMID: 10071084
Multiple autoimmune events after autologous bone marrow transplantation.
Lambertenghi-Deliliers GL, Annaloro C, Della Volpe A, Oriani A, Pozzoli E, Soligo D
Bone Marrow Transplant 1997 Apr;19(7):745-7. doi: 10.1038/sj.bmt.1700711. PMID: 9156254
Therapy-related myelodysplastic syndromes: FAB classification, bone marrow histology, and immunohistology in the prognostic assessment.
Orazi A, Cattoretti G, Soligo D, Luksch R, Lambertenghi-Deliliers G
Leukemia 1993 Jun;7(6):838-47. PMID: 7684797
Treatment-related leukemia in Hodgkin's disease: a multi-institution study on 75 cases.
Brusamolino E, Papa G, Valagussa P, Mandelli F, Bernasconi C, Marmont A, Bonadonna G, Tura S, Bosi A, Mango G
Hematol Oncol 1987 Apr-Jun;5(2):83-98. doi: 10.1002/hon.2900050203. PMID: 2439431

Prognosis

Acquisition of a BCR-ABL1 transcript in a patient with disease progression from MDS with fibrosis to AML with myelodysplasia-related changes.
Park SJ, Lee HW, Jeong SH, Park JS, Kim HC, Seok JY, Kim HJ, Cho SR
Ann Clin Lab Sci 2011 Fall;41(4):379-84. PMID: 22166509
The Wilms' tumor gene WT1 is a good marker for diagnosis of disease progression of myelodysplastic syndromes.
Tamaki H, Ogawa H, Ohyashiki K, Ohyashiki JH, Iwama H, Inoue K, Soma T, Oka Y, Tatekawa T, Oji Y, Tsuboi A, Kim EH, Kawakami M, Fuchigami K, Tomonaga M, Toyama K, Aozasa K, Kishimoto T, Sugiyama H
Leukemia 1999 Mar;13(3):393-9. doi: 10.1038/sj.leu.2401341. PMID: 10086730
Acute leukemia and myelodysplasia in polycythemia vera. A clinical study with long-term follow-up.
Najean Y, Deschamps A, Dresch C, Daniel MT, Rain JD, Arrago JP
Cancer 1988 Jan 1;61(1):89-95. doi: 10.1002/1097-0142(19880101)61:1<89::aid-cncr2820610115>3.0.co;2-0. PMID: 3334954
Bone marrow transplantation for myelodysplastic syndromes.
Bélanger R, Gyger M, Perreault C, Bonny Y, St-Louis J
Br J Haematol 1988 May;69(1):29-33. doi: 10.1111/j.1365-2141.1988.tb07598.x. PMID: 3289604
Treatment-related leukemia in Hodgkin's disease: a multi-institution study on 75 cases.
Brusamolino E, Papa G, Valagussa P, Mandelli F, Bernasconi C, Marmont A, Bonadonna G, Tura S, Bosi A, Mango G
Hematol Oncol 1987 Apr-Jun;5(2):83-98. doi: 10.1002/hon.2900050203. PMID: 2439431

Clinical prediction guides

The Wilms' tumor gene WT1 is a good marker for diagnosis of disease progression of myelodysplastic syndromes.
Tamaki H, Ogawa H, Ohyashiki K, Ohyashiki JH, Iwama H, Inoue K, Soma T, Oka Y, Tatekawa T, Oji Y, Tsuboi A, Kim EH, Kawakami M, Fuchigami K, Tomonaga M, Toyama K, Aozasa K, Kishimoto T, Sugiyama H
Leukemia 1999 Mar;13(3):393-9. doi: 10.1038/sj.leu.2401341. PMID: 10086730
Myelodysplastic syndrome resembling chronic myeloproliferative disorders in clinicopathological aspects.
Fukuda S, Nagai H, Kotani S, Sezaki T, Murakami M, Kitajima K, Sanada H
Int J Hematol 1998 Jul;68(1):79-85. doi: 10.1016/s0925-5710(98)00037-1. PMID: 9713171
Acute leukemia and myelodysplasia in polycythemia vera. A clinical study with long-term follow-up.
Najean Y, Deschamps A, Dresch C, Daniel MT, Rain JD, Arrago JP
Cancer 1988 Jan 1;61(1):89-95. doi: 10.1002/1097-0142(19880101)61:1<89::aid-cncr2820610115>3.0.co;2-0. PMID: 3334954
Bone marrow transplantation for myelodysplastic syndromes.
Bélanger R, Gyger M, Perreault C, Bonny Y, St-Louis J
Br J Haematol 1988 May;69(1):29-33. doi: 10.1111/j.1365-2141.1988.tb07598.x. PMID: 3289604
Treatment-related leukemia in Hodgkin's disease: a multi-institution study on 75 cases.
Brusamolino E, Papa G, Valagussa P, Mandelli F, Bernasconi C, Marmont A, Bonadonna G, Tura S, Bosi A, Mango G
Hematol Oncol 1987 Apr-Jun;5(2):83-98. doi: 10.1002/hon.2900050203. PMID: 2439431

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