medigraphic.com
SPANISH

Revista Cubana de Medicina

ISSN 1561-302X (Print)
  • Contents
  • View Archive
  • Information
    • General Information        
    • Directory
  • Publish
    • Instructions for authors        
  • medigraphic.com
    • Home
    • Journals index            
    • Register / Login
  • Mi perfil

2021, Number 1

<< Back Next >>

Rev cubana med 2021; 60 (1)

Acute panmyelosis with myelofibrosis

Rodriguez FY
Full text How to cite this article

Language: Spanish
References: 13
Page: 1-5
PDF size: 238.99 Kb.


Key words:

acute panmyelosis with myelofibrosis, AML, myeloproliferative diseases, myelofibrosis.

ABSTRACT

Acute panmyelosis with myelofibrosis (PMAF) is a rare hematologic disorder, defined as a subtype of acute leukemia. A 31-year-old male patient with a history of marked decay, evening fever, and gingivorrhagia is reported. The physical examination showed mucous skin paleness, slight splenomegaly and the complementary examinations showed pancytopenia with 5% blasts. In the medullogram no material was obtained and the imprint suggests non-promyelocytic acute myeloid leukemia. Immunophenotype study by flow cytometry confirmed an increase in myeloblasts positive for (CD34, CD13, CD17, CD117, CD38) and decreased in (CD11c and HLA-DR), with marked myelofibrosis in bone marrow biopsy without the presence of blasts. He received induction treatment with a 3 + 7 scheme (Citosar + Rubidomycin) after which the patient was in hematological remission with persistence of spinal fibrosis. Later, he started treatment with lenalidomide, bisphosphonates (Zoledronic Acid) and was in hematological remission 11 months after diagnosis, until March 2020. Histocompatibility studies (HLA) were performed for allogeneic transplantation.


REFERENCES

  1. Arber D, Oraza A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision tothe World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127:2391-405.

  2. Miller KB, Pihan G. Chap. 60. Clinical manifestations of Acute Myeloid Leukemia. En: HoffmanR, Benz EJ, Sanford SJ, et al, editors. Hoffman: Hematology basic principles and practice.5th ed.Philadelphia: Churchill Livingstone Elsevier, Epub; 2009.

  3. Zhaodong Xu. AML with myelodysplasia-related changes masquerades as acute panmyelosis withmyelofibrosis. Blood, The Journal of American society of Hematology. 2017;130(15);1775.

  4. Tathagata Ch, Srishti G, Ajay Sh, Sanjeevan Sh, Devika G. Acute Panmyelosis with Myelofibrosis:A Rare Cause of Pancytopenia. Mediterr J Hematol Infect Dis. 2013[acceso: 15/08/2019];5(1).Disponible en: http://www.mjhid.org/article/view/11647

  5. Orazi A, O’Malley DP, Jiang J. Acute panmyelosis with myelofibrosis: an entity distinct fromacute megakaryoblastic leukemia Department of Pathology and Laboratory Medicine, IndianaUniversity School of Medicine. Modern Pathology. 2005;(18):603-14.

  6. Thiele H. M, Kvasnicka. G. Zerhusen. J. Vardiman. V. Diehl M. Luebbert. A, et al. Acutepanmyelosis with myelofibrosis: a clinicopathological study on 46 patients including histochemistryof bone marrow biopsies and follow-up. Ann Hematol. 2004;83:513-21.

  7. Jurguen T, Hans M. Kvasnicka and Annette S-G. Acute Panmyelosis with Myelofibrosis. Leukemia& Lymphoma. 2004;45(4):681-7.

  8. Rumi E, Cazzola M. Diagnosis, risk stratification, and response evaluation in classicalmyeloproliferative neoplasms. Blood. 2017;129(6):680-92.

  9. Tathagata Ch, Srishti G, Ajay Sh, Sanjeevan Sh. Acute Panmyelosis with Myelofibrosis - A RareSubtype of Acute Myeloid Leukemia. Mediterr J Hematol Infect Dis. 2013[acceso:15/08/2019];5(1). Disponible en: http://www.mjhid.org/article/view/11647

  10. Konuma T, Kondo T, Kawata T, Iwato K, Sato Y, Mori T, et al. Hematopoietic celltransplantation for acute panmyelosis with myelofibrosis: a retrospective study in Japan, Biologyof Blood and Marrow Transplantation Adult Acute Myeloid Leukemia Working Group of the JapanSociety for Hematopoietic Cell Transplantation. 2018.https://doi.org/10.1016/j.bbmt.2018.08.006

  11. Carnot Uria J, Hernández Cruz C, Muñío Perurena J, Torres Yribar W, Diego de la Campa J, DelCastillo Carrillo C, et al. Bone Marrow Transplantation in Patients with Acute Leukemia In Cuba:Results from the Last 30 Years and New Opportunities Through International Collaboration. Journalof Global Oncology. 2018:4:1-7.

  12. Vassilopoulos G, Palassopoulou M, Zisaki K, Befani M, Bouronikou E, Giannakoulas N, et al.Successful Control of Acute Myelofibrosis with Lenalidomide. Case Reports in Medicine. 2010.http://doi.org/10.1155/2010/421239

  13. Español I, Romagosa V, Berlanga J. Zoledronate-induced remission of acute panmyelosis withmyelofibrosis. Eur J Hematol. 2004:73:1-4.




2020     |     www.medigraphic.com

Mi perfil

C?MO CITAR (Vancouver)

Rev cubana med. 2021;60