The Etiology and Diagnosis of Primary Myelofibrosis: Case Study
Asian Journal of Case Reports in Medicine and Health,
Page 11-15
Abstract
Primary myelofibrosis is a myeloproliferative neoplasm. It is the rarest among the group of myeloproliferative neoplasms and the incidence is 0.1-1 per 1,00,000 per year. This is characterised by the replacement of normal marrow by fibrous tissue. Patients may present with hepatosplenomegaly due to extramedullary erythropoiesis. A high index of suspicion is needed to diagnose the same. This study describes a case that was diagnosed to have myelofibrosis when he presented with splenomegaly as a main symptom.
Keywords:
- Primary Myelofibrosis
- splenomegaly
- extramedullary erythropoiesis
- myeloproliferative disorder
How to Cite
Indu, M. B., Khan, S., Sharma, B., & Jyotsana, P. L. (2022). The Etiology and Diagnosis of Primary Myelofibrosis: Case Study. Asian Journal of Case Reports in Medicine and Health, 7(2), 11-15. Retrieved from https://journalajcrmh.com/index.php/AJCRMH/article/view/30205
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Francisco Cervantes: How I treat myelofibrosis?; Blood. 2014;124(17):2635-2642
Claire Harrison MD; When to initiate treatment in myelofibrosis?; Clinical advances in hematology and oncology. 2016;14(11):934-937.
TizianoBarbui, Jurgen Thiele, Heinz Gisslinger, Hans Michael Kvasnicka, Alessandro M. Vannucchi, Paola Guglielmelli, Attilioorazi and AyalewTefferi; The 2016 WHO classification and diagnostic criteria for myeloproliferative neoplasms: document summary and in depth discussion; blood Cancer J. 2018;8(2): 15
Vakil E, Tefferi A. BCR-ABL1—negative myeloproliferative neoplasms: a review of molecular biology, diagnosis, and treatment. Clin Lymphoma Myeloma Leuk. 2011;11Suppl 1:S37-45.
Baxter EJ, Scott LM, Campbell PJ, et al. Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. Lancet. 2005;365:1054-6
T, Bhattacharya S, Braun-stein J, Schindler CW. Signalling through JAK STAT pathwy, recent advances and future challenges. Gene. 2002;285:1-24
Pikman Y, Lee BH, Mercher T , McDowell E, Ebert BL, Gozo M, Cuker A, Wernig G, Moore S et al. MPLW515L is a novel somatic activating mutation in myelofibrosis with myeloid metaplasia. PLoS Med. 2006;3(7):e270.
Campbell PJ, Green AR. The myeloproliferative disorders. N Engl J Med. 2006;355(23):2452-66.
Robbins SL, Kumar V, Cotran RS. Robbins and Cotrans Pathologic basis of disease eighth edition: saunders/ Elseiver. 2010;1164-1167.
Francisco Cervantes: How I treat myelofibrosis?; Blood. 2014;124(17):2635-2642
Claire Harrison MD; When to initiate treatment in myelofibrosis?; Clinical advances in hematology and oncology. 2016;14(11):934-937.
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