Myelofibrosis classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, M.D.[2]
Overview
Myelofibrosis is subclassified into primary and secondary types with the primary type being more common and a high proportion of the cases resulting from mutations in the Janus kinase 2 (JAK2) gene. It can be secondary to a variety of malignant, non-malignant, and hematologic conditions. It can also be secondary to malignancies, infections, toxins, autoimmune, and endocrine diseases.
Classification
- Myelofibrosis may be classified according to etiology into two types:
- Primary
- Secondary
Primary Myelofibrosis
- The primary type is associated with:[1][2][3]
- Stem cell-derived clonal myeloproliferation
- Mutations of the Myeloproliferative leukemia virus (MPL) oncogene, the calreticulin (CALR) gene, or Janus kinase 2 (JAK2) gene in 90% of patients
- "Triple-negative" in 10% of patients
- The 2016 World Health Organization (WHO) revised classification of myeloproliferative neoplasms (MPNs) defines 2 stages of primary myelofibrosis (PMF):
- Prefibrotic/early (pre-primary myelofibrosis) phase, characterized by granulocytic/megakaryocytic proliferation and lack of reticulin bone marrow fibrosis.[4][5]
- Overtly fibrotic (overt primary myelofibrosis) phase, characterized by bone marrow fibrosis, pancytopenia, higher blast count, extramedullary hematopoiesis (EMH), and unfavorable karyotype with a significantly shortened median survival.[4]
Secondary Myelofibrosis
- Myelofibrosis can be secondary to toxins exposure and multiple primary conditions such as:
- Malignancies and hematologic disorders (Hodgkin lymphoma, non-Hodgkin lymphoma, essential thrombocythemia (ET), polycythemia vera (PV), multiple myeloma (MM), thrombotic thrombocytopenic purpura [TTP], and malignancies with metastases to the bone)[6][7][8][9][10][11][12][13][14][15][6][16]
- Toxins (Benzene, thorium dioxide, nitrosourea, and x- or γ-radiation)[17][18][19][20][21][22][23][24]
- Infections (tuberculosis [TB], HIV infection, and dengue fever)[25][26][27][28]
- Autoimmune diseases (systemic lupus erythematosus [SLE], multiple sclerosis [MS], and juvenile idiopathic arthritis)[29][30][31]
- Endocrine disorders (primary hyperparathyroidism)[32]
References
- ↑ Tefferi A (December 2016). "Primary myelofibrosis: 2017 update on diagnosis, risk-stratification, and management". Am. J. Hematol. 91 (12): 1262–1271. doi:10.1002/ajh.24592. PMID 27870387.
- ↑ Barbui T, Thiele J, Gisslinger H, Kvasnicka HM, Vannucchi AM, Guglielmelli P, Orazi A, Tefferi A (February 2018). "The 2016 WHO classification and diagnostic criteria for myeloproliferative neoplasms: document summary and in-depth discussion". Blood Cancer J. 8 (2): 15. doi:10.1038/s41408-018-0054-y. PMC 5807384. PMID 29426921.
- ↑ Abaza Y, Yin CC, Bueso-Ramos CE, Wang SA, Verstovsek S (April 2017). "Primary autoimmune myelofibrosis: a case report and review of the literature". Int. J. Hematol. 105 (4): 536–539. doi:10.1007/s12185-016-2129-5. PMID 27830539.
- ↑ 4.0 4.1 Guglielmelli P, Pacilli A, Rotunno G, Rumi E, Rosti V, Delaini F, Maffioli M, Fanelli T, Pancrazzi A, Pietra D, Salmoiraghi S, Mannarelli C, Franci A, Paoli C, Rambaldi A, Passamonti F, Barosi G, Barbui T, Cazzola M, Vannucchi AM (June 2017). "Presentation and outcome of patients with 2016 WHO diagnosis of prefibrotic and overt primary myelofibrosis". Blood. 129 (24): 3227–3236. doi:10.1182/blood-2017-01-761999. PMID 28351937.
- ↑ Rumi E, Cazzola M (February 2017). "Diagnosis, risk stratification, and response evaluation in classical myeloproliferative neoplasms". Blood. 129 (6): 680–692. doi:10.1182/blood-2016-10-695957. PMC 5335805. PMID 28028026.
- ↑ 6.0 6.1 Chang JC, Naqvi T (2003). "Thrombotic thrombocytopenic purpura associated with bone marrow metastasis and secondary myelofibrosis in cancer". Oncologist. 8 (4): 375–80. PMID 12897334.
- ↑ Boiocchi L, Mathew S, Gianelli U, Iurlo A, Radice T, Barouk-Fox S, Knowles DM, Orazi A (December 2013). "Morphologic and cytogenetic differences between post-polycythemic myelofibrosis and primary myelofibrosis in fibrotic stage". Mod. Pathol. 26 (12): 1577–85. doi:10.1038/modpathol.2013.109. PMID 23787440.
- ↑ Sakatoku K, Takeoka Y, Araki T, Miura A, Fujitani Y, Yamamura R, Miyagi Y, Senzaki H, Ohta K (2017). "Lymphocyte-depleted classical Hodgkin lymphoma accompanied by myelofibrosis". Rinsho Ketsueki (in Japanese). 58 (7): 772–775. doi:10.11406/rinketsu.58.772. PMID 28781273.
- ↑ Fu R, Yu H, Wu YH, Liu H, Shao ZH (September 2015). "Hodgkin's lymphoma associated with myelofibrosis: A case report". Oncol Lett. 10 (3): 1551–1554. doi:10.3892/ol.2015.3438. PMC 4533276. PMID 26622707.
- ↑ Liu YL, Wang WJ, Wang XN (June 2015). "[Pathological Characteristics of Bone Marrow in Non-Hodgkin's Lymphoma Patients with Secondary Myelofibrosis and Their Relationship with Prognosis]". Zhongguo Shi Yan Xue Ye Xue Za Zhi (in Chinese). 23 (3): 674–8. doi:10.7534/j.issn.1009-2137.2015.03.014. PMID 26117015.
- ↑ Dolgikh TY, Domnikova NP, Tornuev YV, Vinogradova EV, Krinitsyna YM (February 2017). "Incidence of Myelofibrosis in Chronic Myeloid Leukemia, Multiple Myeloma, and Chronic Lymphoid Leukemia during Various Phases of Diseases". Bull. Exp. Biol. Med. 162 (4): 483–487. doi:10.1007/s10517-017-3645-x. PMID 28239786.
- ↑ Zhao J, Ma L, Guan JH (August 2017). "[Pathological Characteristics of Bone Marrow in Multiple Myeloma Patients with Secondary Myelofibrosis and Their Relationship with Prognosis]". Zhongguo Shi Yan Xue Ye Xue Za Zhi (in Chinese). 25 (4): 1080–1085. doi:10.7534/j.issn.1009-2137.2017.04.021. PMID 28823272.
- ↑ Passamonti F, Giorgino T, Mora B, Guglielmelli P, Rumi E, Maffioli M, Rambaldi A, Caramella M, Komrokji R, Gotlib J, Kiladjian JJ, Cervantes F, Devos T, Palandri F, De Stefano V, Ruggeri M, Silver RT, Benevolo G, Albano F, Caramazza D, Merli M, Pietra D, Casalone R, Rotunno G, Barbui T, Cazzola M, Vannucchi AM (December 2017). "A clinical-molecular prognostic model to predict survival in patients with post polycythemia vera and post essential thrombocythemia myelofibrosis". Leukemia. 31 (12): 2726–2731. doi:10.1038/leu.2017.169. PMID 28561069.
- ↑ Masarova L, Bose P, Daver N, Pemmaraju N, Newberry KJ, Manshouri T, Cortes J, Kantarjian HM, Verstovsek S (August 2017). "Patients with post-essential thrombocythemia and post-polycythemia vera differ from patients with primary myelofibrosis". Leuk. Res. 59: 110–116. doi:10.1016/j.leukres.2017.06.001. PMC 5573611. PMID 28601551.
- ↑ MARKAND ON (May 1965). "SECONDARY MARBLE BONE DISEASE: GENERALISED OSTEOSCLEROSIS AND MYELOFIBROSIS IN CARCINOMA OF PROSTATE WITH A CASE REPORT". J Assoc Physicians India. 13: 349–55. PMID 14302719.
- ↑ Hiwada K, Sera Y, Nishimura M (July 1970). "[Autopsy case of secondary myelofibrosis due to bone marrow metastasis of stomach cancer]". Iryo (in Japanese). 24 (7): 585–90. PMID 5458299.
- ↑ Bausà R, Navarro L, Cortès-Franch I (2017). "[Myelofibrosis in a benzene-exposed cleaning worker]". Arch Prev Riesgos Labor (in Spanish; Castilian). 20 (3): 167–169. doi:10.12961/aprl.2017.20.3.03. PMID 28715625.
- ↑ Hu H (January 1987). "Benzene-associated myelofibrosis". Ann. Intern. Med. 106 (1): 171–2. PMID 3789571.
- ↑ Tondel M, Persson B, Carstensen J (February 1995). "Myelofibrosis and benzene exposure". Occup Med (Lond). 45 (1): 51–2. PMID 7703476.
- ↑ Visfeldt J, Andersson M (January 1995). "Pathoanatomical aspects of malignant haematological disorders among Danish patients exposed to thorium dioxide". APMIS. 103 (1): 29–36. PMID 7695889.
- ↑ Brandt L, Emanuelsson H, Mitelman F, Stenstam M, Söderström N (1977). "Pronounced deficiency in T-cells and lymphocyte chromosomal aberrations in a patient with sarcoidosis, myelofibrosis and acute leukaemia following thorotrast angiography". Acta Med Scand. 201 (5): 487–9. PMID 302634.
- ↑ Arnold AG, Oelbaum MH (February 1980). "Thorotrast administration followed by myelofibrosis". Postgrad Med J. 56 (652): 124–7. PMC 2425512. PMID 7393792.
- ↑ Jennings RC, Priestley SE (December 1978). "Haemangioendothelioma (Kupffer cell angiosarcoma), myelofibrosis, splenic atrophy, and myeloma paraproteinaemia after parenteral thorotrast administration". J. Clin. Pathol. 31 (12): 1125–32. PMC 1145517. PMID 748384.
- ↑ McKenney SA, Fehir KM (October 1986). "Myelofibrosis following treatment with a nitrosourea for malignant glioma". Cancer. 58 (7): 1426–7. PMID 3742462.
- ↑ Qing X, Sun N, Yeh J, Yue C, Cai J (October 2014). "Dengue fever and bone marrow myelofibrosis". Exp. Mol. Pathol. 97 (2): 208–10. doi:10.1016/j.yexmp.2014.07.004. PMID 25016180.
- ↑ Lee AC, Fong CM (May 2012). "Autoimmune myelofibrosis as the first manifestation of human immunodeficiency virus infection in an infant". Ann. Hematol. 91 (5): 809–810. doi:10.1007/s00277-011-1329-6. PMID 21894472.
- ↑ Hashim MS, Kordofani AY, el Dabi MA (March 1997). "Tuberculosis and myelofibrosis in children: a report". Ann Trop Paediatr. 17 (1): 61–5. PMID 9176580.
- ↑ Viallard JF, Parrens M, Boiron JM, Texier J, Mercie P, Pellegrin JL (June 2002). "Reversible myelofibrosis induced by tuberculosis". Clin. Infect. Dis. 34 (12): 1641–3. doi:10.1086/340524. PMID 12032901.
- ↑ Jain N, Sinha R, Sengupta J, Chakrabartty J (June 2016). "A rare case of myelofibrosis secondary to juvenile idiopathic arthritis". Br. J. Haematol. 173 (6): 819. doi:10.1111/bjh.14106. PMID 27102067.
- ↑ Cansu DÜ, Teke HÜ, Korkmaz C (March 2017). "A rare cause of cytopenia in a patient with systemic lupus erythematosus: Autoimmune myelofibrosis". Eur J Rheumatol. 4 (1): 76–78. doi:10.5152/eurjrheum.2016.011. PMC 5335895. PMID 28293461.
- ↑ Thorsteinsdottir S, Bjerrum OW, Hasselbalch HC (2013). "Myeloproliferative neoplasms in five multiple sclerosis patients". Leuk Res Rep. 2 (2): 61–3. doi:10.1016/j.lrr.2013.06.004. PMC 3850374. PMID 24371783.
- ↑ Lim DJ, Oh EJ, Park CW, Kwon HS, Hong EJ, Yoon KH, Kang MI, Cha BY, Lee KW, Son HY, Kang SK (December 2007). "Pancytopenia and secondary myelofibrosis could be induced by primary hyperparathyroidism". Int J Lab Hematol. 29 (6): 464–8. doi:10.1111/j.1365-2257.2006.00877.x. PMID 17988303.