Hostname: page-component-8448b6f56d-t5pn6 Total loading time: 0 Render date: 2024-04-23T15:57:41.779Z Has data issue: false hasContentIssue false

Myeloproliferative disorders in older people

Published online by Cambridge University Press:  03 January 2012

Wendy Osborne
Affiliation:
Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust, UK
Gail L Jones*
Affiliation:
Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust, UK
Graham H Jackson
Affiliation:
Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust, UK
Helen Marr
Affiliation:
Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust, UK
*
Address for correspondence: Dr Gail L Jones, Department of Haematology, Freeman Hospital, Newcastle upon Tyne NE7 7DN. Email: gail.jones@nuth.nhs.uk

Summary

The term myeloproliferative disorders (MPDs) describes a group of conditions in which an intrinsic stem cell defect is present in the bone marrow. This leads to hypercellularity in one or more of the myeloid cell lines (red blood cells, platelets, granulocytes and their precursors), or increased bone marrow fibrosis. MPDs are rare; combined annual incidence is 6 per 100,000.

MPDs may present in asymptomatic patients as an incidental finding, or may be associated with constitutional features such as weight loss, sweats or lethargy. Splenomegaly is common but lymphadenopathy is not a feature. The most significant complications are: arterial thrombosis, venous thrombosis, secondary marrow fibrosis and transformation to acute myeloblastic leukaemia (AML). Treatment aims to reduce the risk of such complications.

Recent advances in the understanding of the pathogenesis of these disorders have radically changed the approach to the diagnosis of MPD and are the focus of new treatment developments.

Type
Clinical geriatrics
Copyright
Copyright © Cambridge University Press 2012

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1Dameshek, W. Some speculations on the myeloproliferative syndromes. Blood 1951; 6: 372–75.CrossRefGoogle ScholarPubMed
2Swerdlow, H, Campo, E, Harris, NL et al. (eds). WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (4th edn). Lyon: International Agency for Research on Cancer, 2008.Google Scholar
3Rollison, DE, Howlader, N, Smith, MT, Strom, SS, Merritt, WD, Ries, LA, Edwards, BK, List, AF. Epidemiology of myelodysplastic syndromes and chronic myeloproliferative disorders in the United States, 2001–2004, using data from the NAACCR and SEER programs. Blood 2008; 112: 4552.CrossRefGoogle ScholarPubMed
4Wolanskyj, AP, Schwager, SM, McClure, RF, Larson, DR, Tefferi, A. Essential thrombocythaemia beyond the first decade: life expectancy, long-term complication rates and prognostic factors. Mayo Clin Proc 2006; 81: 159–66.CrossRefGoogle ScholarPubMed
5Cervantes, F, Passamonti, F, Barosi, G. Life expectancy and prognostic factors in the classic BCR/ABL negative myeloproliferative disorders. Leukemia 2008; 22: 905–14.CrossRefGoogle ScholarPubMed
6Nowell, PC, Hungerford, DA. Chromosome studies on normal and leukaemic human leukocytes. J Natl Cancer Inst 1960; 25: 85109.Google Scholar
7Sawyers, CL. Chronic myeloid leukaemia review. N Engl J Med 1999; 340: 1330–40.CrossRefGoogle Scholar
8Vannucchi, AM, Guglielmelli, P, Tefferi, A. Advances in the understanding and management of myeloproliferative neoplasms. CA Cancer J Clin 2009; 59: 171–91.CrossRefGoogle ScholarPubMed
9Scott, LM, Beer, PA, Bench, AJ, Erber, WN, Green, AR. Prevalence of JAK2 V617F and exon 12 mutations in polycythaemia vera. Br J Haematol 2007; 139: 511–12.CrossRefGoogle ScholarPubMed
10Pardanani, AD, Levine, RL, Lasho, T, Pikman, Y, Mesa, RA, Wadleigh, M, Steensma, DP, Elliott, MA, Wolanskyj, AP, Hogan, WJ, McClure, RF, Litzow, MR, Gilliland, DG, Tefferi, A. (2006). MPL515 mutations in myeloproliferative and other myeloid disorders: a study of 1182 patients. Blood 2006; 108: 3472–76.CrossRefGoogle ScholarPubMed
11McMullin, MF, Reilly, JT, Campbell, P, Bareford, D, Green, AR, Harrison, CN, Conneally, E; National Cancer Research Institute, Myeloproliferative Disorder Subgroup, Ryan, K; British Committee for Standards in Haematology. Amendment to the guideline for diagnosis and investigation of polycythaemia/erythrocytosis. Br J Haematol 2007; 138: 2122.CrossRefGoogle Scholar
12McMullin, MF. The classification and diagnosis of erythrocytosis. Int J Lab Hematol 2008; 30: 447–59.CrossRefGoogle ScholarPubMed
13Landolfi, R, Marchioli, R, Kutti, J, Gisslinger, H, Tognoni, G, Patrono, C, Barbui, T; European Collaboration on Low-Dose Aspirin in Polycythemia Vera Investigators. Efficacy and safety of low-dose aspirin in polycythaemia vera. N Engl J Med 2004; 350: 114–24.CrossRefGoogle Scholar
14McMullin, MF, Bareford, D, Campbell, P. Guidelines for the diagnosis, investigation and management of polycythaemia/erythrocytosis. Br J Haematol 2005; 130: 174–95.CrossRefGoogle ScholarPubMed
15Pearson, TC, Wetherley-Main, G. Vascular occlusive episodes and venous haematocrit in primary proliferative polycythaemia. Lancet 1978; 312: 1219–22.CrossRefGoogle Scholar
16Passamonti, F, Rumi, E, Pungolino, E, Malabarba, L, Bertazzoni, P, Valentini, M, Orlandi, E, Arcaini, L, Brusamolino, E, Pascutto, C, Cazzola, M, Morra, E, Lazzarino, M. Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia. Am J Med 2004; 117: 755–61.CrossRefGoogle ScholarPubMed
17Marchioli, R, Finazzi, G, Landolfi, R, Kutti, J, Gisslinger, H, Patrono, C, Marilus, R, Villegas, A, Tognoni, G, Barbui, T. Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. J Clin Oncol 2005; 23: 2224–32.CrossRefGoogle Scholar
18Beer, PA, Erber, WN, Campbell, PJ, Green, AR. How I treat essential thrombocythemia. Blood 2011; 117: 1472–82.CrossRefGoogle Scholar
19Harrison, CN, Bareford, D, Butt, N, Campbell, P, Conneally, E, Drummond, M, Erber, W, Everington, T, Green, AR, Hall, GW, Hunt, BJ, Ludlam, CA, Murrin, R, Nelson-Piercy, C, Radia, DH, Reilly, JT, Van der Walt, J, Wilkins, B, McMullin, MF; British Committee for Standards in Haematology. Guideline for investigation and management of adults and children presenting with a thrombocytosis. Br J Haematol 2010; 149: 352–75.CrossRefGoogle ScholarPubMed
20Mesa, RA, Silverstein, MN, Jacobsen, SJWollan, PC, Tefferi, A. Population-based incidence and survival figures in essential thrombocythemia and agnogenic myeloid metaplasia: an Olmsted County Study, 1976–1995. Am J Hematol 1999; 61: 1015.3.0.CO;2-I>CrossRefGoogle ScholarPubMed
21Osborne, WL, Wilkins, B, Lennard, AL. Cutaneous extramedullary hemopoiesis in myelofibrosis. Available at: http://www.bloodmed.com/education/case1.asp?id=127 (accessed 21 October 2011).Google Scholar
22Barbui, T, Carobbio, A, Cervantes, F, Vannucchi, AM, Guglielmelli, P, Antonioli, E, Alvarez-Larrán, A, Rambaldi, A, Finazzi, G, Barosi, G. Thrombosis in primary myelofibrosis: incidence and risk factors. Blood 2010; 115: 778–82.CrossRefGoogle ScholarPubMed
23Visani, G, Finelli, C, Castelli, U et al. Myelofibrosis with myeloid metaplasia: clinical and haematological parameters predicting survival in a series of 133 patients. Br J Haematol 1990; 75: 49.CrossRefGoogle Scholar
24Sirhan, S, Lasho, TL, Hanson, CA, Mesa, RA, Pardanai, A, Tefferi, A. The presence of JAK2V617F in primary myelofibrosis or its allele burden in polycythemia vera predicts chemosensitivity to hydroxyurea. Am J Hematol 2008; 83: 363–65.CrossRefGoogle ScholarPubMed
25Besa, EC, Nowell, PC, Gella, NL, Gardner, FH. Analysis of the androgen response of 23 patients with agnogenic myeloid metaplasia: the value of chromosomal studies in predicting response and survival. Cancer 1982; 49: 308–13.3.0.CO;2-4>CrossRefGoogle ScholarPubMed
26Mesa, RA, Nagorney, DS, Schwager, S, Allred, J, Tefferi, A. Palliative goals, patient selection, and pre-operative platelet management: outcomes and lessons from 3 decades of splenectomy for myelofibrosis with myeloid metaplasia at the Mayo Clinic. Cancer 2006; 107: 361–70.CrossRefGoogle Scholar
27Cervantes, F, Dupriez, B, Pereira, A, Passamonti, F, Reilly, JT, Morra, E, Vannucchi, AM, Mesa, RA, Demory, JL, Barosi, G, Rumi, E, Tefferi, A. New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment. Blood 2009; 113: 2895–901.CrossRefGoogle Scholar
28Mesa, RA, Li, CY, Ketterling, RP, Schroeder, GS, Knudson, RA, Tefferi, A. Leukaemic transformation in myelofibrosis with myeloid metaplasia: a single institution experience with 91 cases. Blood 2005; 105: 973–77.CrossRefGoogle ScholarPubMed
29British Committee for Standards in Haematology 2007. Recommendations for the management of BCR-ABL positive chronic myeloid leukaemia. Available at: http://www.bcshguidelines.com/documents/CML_BCR-ABL_270707.pdf (accessed 21 October 2011).Google Scholar
30O'Brien, SG, Guilhot, F, Larson, RA, Gathmann, I, Baccarani, M, Cervantes, F, Cornelissen, JJ, Fischer, T, Hochhaus, A, Hughes, T, Lechner, K, Nielsen, JL, Rousselot, P, Reiffers, J, Saglio, G, Shepherd, J, Simonsson, B, Gratwohl, A, Goldman, JM, Kantarjian, H, Taylor, K, Verhoef, G, Bolton, AE, Capdeville, R, Druker, BJ; IRIS Investigators. Imatinib compared with interferon and low-dose cytarabine for newly diagnosed chronic-phase chronic myeloid leukemia. N Engl J Med 2003; 348: 9941004.CrossRefGoogle ScholarPubMed
31Druker, BJ, Guilhot, F, O'Brien, SG, Gathmann, I, Kantarjian, H, Gattermann, N, Deininger, MW, Silver, RT, Goldman, JM, Stone, RM, Cervantes, F, Hochhaus, A, Powell, BL, Gabrilove, JL, Rousselot, P, Reiffers, J, Cornelissen, JJ, Hughes, T, Agis, H, Fischer, T, Verhoef, G, Shepherd, J, Saglio, G, Gratwohl, A, Nielsen, JL, Radich, JP, Simonsson, B, Taylor, K, Baccarani, M, So, C, Letvak, L, Larson, RA; IRIS Investigators. Five year follow-up of patients receiving imatanib for CML. N Engl J Med 2006; 355: 2408–17.CrossRefGoogle Scholar
32Sokal, JE, Cox, EB, Baccarani, M et al. Prognostic discrimination in ‘good-risk’ chronic granulocytic leukemia. Blood 1984; 63: 789–99.CrossRefGoogle ScholarPubMed
33Goldman, G. How I treat chronic myeloid leukaemia in the imatinib era. Blood 2007; 110: 2828–37.CrossRefGoogle Scholar
34Chen, AT, Prchal, JT. JAK 2 kinase inhibitors and myeloproliferative disorders. Curr Opin Hematol 2010; 17: 110–16.CrossRefGoogle Scholar
35Stewart, WA, Pearce, R, Kirkland, KE, Bloor, A, Thomson, K, Apperley, J, McQuaker, G, Marks, DI, Craddock, C, McCann, S, Russell, N, Cook, G, Kottaridis, PD; British Society for Blood and Marrow Transplantation. The role of allogeneic SCT in primary myelofibrosis: a British Society for Blood and Marrow Transplantation study. Bone Marrow Transplant 2010; 45: 1587–93.CrossRefGoogle ScholarPubMed