U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Acute panmyelosis with myelofibrosis

MedGen UID:
87279
Concept ID:
C0334674
Neoplastic Process
Synonyms: Acute (Malignant) Myelofibrosis; acute (malignant) myelofibrosis; Acute (Malignant) Myelosclerosis; acute (malignant) myelosclerosis; Acute myelodysplasia with myelofibrosis; acute myelodysplasia with myelofibrosis; Acute Myelofibrosis; Acute myelofibrosis; acute myelofibrosis; Acute Myelosclerosis; Acute myelosclerosis; acute myelosclerosis; Acute Panmyelosis; acute panmyelosis; Acute Panmyelosis with Myelofibrosis; acute panmyelosis with myelofibrosis; APMF
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019455
Orphanet: ORPHA86843

Definition

A rare unclassified acute myeloid leukemia characterized by an acute panmyeloid proliferation with blasts constituting more than 20% of cells in the bone marrow or peripheral blood, accompanied by fibrosis of the bone marrow. Patients typically present with acute onset of severe constitutional symptoms, bone pain, and pancytopenia. Splenomegaly is minimal or absent. The disease is rapidly progressive with poor therapy response. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAcute panmyelosis with myelofibrosis
Follow this link to review classifications for Acute panmyelosis with myelofibrosis in Orphanet.

Professional guidelines

PubMed

Bae E, Park CJ, Cho YU, Seo EJ, Chi HS, Jang S, Lee KH, Lee JH, Lee JH, Suh JJ, Im HJ
Int J Lab Hematol 2013 Dec;35(6):629-36. Epub 2013 May 22 doi: 10.1111/ijlh.12101. PMID: 23693053

Recent clinical studies

Etiology

Konuma T, Kondo T, Kawata T, Iwato K, Sato Y, Mori T, Ohashi K, Nakazawa H, Sugahara H, Ago H, Eto T, Imamura Y, Fukuda T, Kanda Y, Atsuta Y, Yano S; Adult Acute Myeloid Leukemia Working Group of the Japan Society for Hematopoietic Cell Transplantation
Biol Blood Marrow Transplant 2019 Jan;25(1):e23-e27. Epub 2018 Aug 10 doi: 10.1016/j.bbmt.2018.08.006. PMID: 30103016
Song X, Peng Y, Wang X, Chen Y, Jin L, Yang T, Qian M, Ni W, Tong X, Lan J
Acta Haematol 2018;139(2):115-127. Epub 2018 Feb 16 doi: 10.1159/000486228. PMID: 29455198
Orazi A
Pathobiology 2007;74(2):97-114. doi: 10.1159/000101709. PMID: 17587881
Ngirabacu MC, Ravoet C, Dargent JL, Meuleman N, Ahmad I, Ysebrant L, Bennani J, André M, Bron D
Haematologica 2006 Dec;91(12 Suppl):ECR53. PMID: 17194659
Thiele J, Kvasnicka HM, Zerhusen G, Vardiman J, Diehl V, Luebbert M, Schmitt-Graeff A
Ann Hematol 2004 Aug;83(8):513-21. Epub 2004 Jun 2 doi: 10.1007/s00277-004-0881-8. PMID: 15173958

Diagnosis

George TI, Bajel A
Pathology 2021 Apr;53(3):312-327. Epub 2021 Mar 4 doi: 10.1016/j.pathol.2021.02.001. PMID: 33676766
Orazi A, Czader MB
Am J Clin Pathol 2009 Aug;132(2):290-305. doi: 10.1309/AJCPRCXX4R0YHKWV. PMID: 19605823
Orazi A, O'Malley DP, Jiang J, Vance GH, Thomas J, Czader M, Fang W, An C, Banks PM
Mod Pathol 2005 May;18(5):603-14. doi: 10.1038/modpathol.3800348. PMID: 15578075
Thiele J, Kvasnicka HM, Zerhusen G, Vardiman J, Diehl V, Luebbert M, Schmitt-Graeff A
Ann Hematol 2004 Aug;83(8):513-21. Epub 2004 Jun 2 doi: 10.1007/s00277-004-0881-8. PMID: 15173958
Thiele J, Kvasnicka HM, Schmitt-Graeff A
Leuk Lymphoma 2004 Apr;45(4):681-7. doi: 10.1080/10428190310001625692. PMID: 15160939

Therapy

Orazi A
Pathobiology 2007;74(2):97-114. doi: 10.1159/000101709. PMID: 17587881
Ngirabacu MC, Ravoet C, Dargent JL, Meuleman N, Ahmad I, Ysebrant L, Bennani J, André M, Bron D
Haematologica 2006 Dec;91(12 Suppl):ECR53. PMID: 17194659
Español I, Romagosa V, Berlanga J, Domingo A, Losa F, Heras L, Janáriz J
Eur J Haematol 2004 Sep;73(3):215-8. doi: 10.1111/j.1600-0609.2004.00277.x. PMID: 15287920

Prognosis

George TI, Bajel A
Pathology 2021 Apr;53(3):312-327. Epub 2021 Mar 4 doi: 10.1016/j.pathol.2021.02.001. PMID: 33676766
Konuma T, Kondo T, Kawata T, Iwato K, Sato Y, Mori T, Ohashi K, Nakazawa H, Sugahara H, Ago H, Eto T, Imamura Y, Fukuda T, Kanda Y, Atsuta Y, Yano S; Adult Acute Myeloid Leukemia Working Group of the Japan Society for Hematopoietic Cell Transplantation
Biol Blood Marrow Transplant 2019 Jan;25(1):e23-e27. Epub 2018 Aug 10 doi: 10.1016/j.bbmt.2018.08.006. PMID: 30103016
Grygalewicz B, Woroniecka R, Pastwińska A, Rygier J, Krawczyk P, Borg K, Makuch-Łasica H, Patkowska E, Pieńkowska-Grela B
Cancer Genet 2012 May;205(5):255-60. doi: 10.1016/j.cancergen.2012.02.003. PMID: 22682625
Suvajdzic N, Marisavljevic D, Kraguljac N, Pantic M, Djordjevic V, Jankovic G, Cemerikic-Martinovic V, Colovic M
Leuk Lymphoma 2004 Sep;45(9):1873-9. doi: 10.1080/10428190410001683688. PMID: 15223649
Thiele J, Kvasnicka HM, Schmitt-Graeff A
Leuk Lymphoma 2004 Apr;45(4):681-7. doi: 10.1080/10428190310001625692. PMID: 15160939

Clinical prediction guides

Kim SY, Im K, Park SN, Kwon J, Kim JA, Lee DS
Am J Clin Pathol 2015 May;143(5):635-44. doi: 10.1309/AJCPUAAC16LIWZMM. PMID: 25873496
Orazi A
Pathobiology 2007;74(2):97-114. doi: 10.1159/000101709. PMID: 17587881
Español I, Romagosa V, Berlanga J, Domingo A, Losa F, Heras L, Janáriz J
Eur J Haematol 2004 Sep;73(3):215-8. doi: 10.1111/j.1600-0609.2004.00277.x. PMID: 15287920
Thiele J, Kvasnicka HM, Zerhusen G, Vardiman J, Diehl V, Luebbert M, Schmitt-Graeff A
Ann Hematol 2004 Aug;83(8):513-21. Epub 2004 Jun 2 doi: 10.1007/s00277-004-0881-8. PMID: 15173958

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...