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Journal of Clinical Pathology logoLink to Journal of Clinical Pathology
. 1988 Jun;41(6):609–614. doi: 10.1136/jcp.41.6.609

Extramedullary haemopoietic tumours complicating polycythaemia vera.

P K MacCallum 1, M J Newbould 1, P S Sambrook 1, I E Burton 1
PMCID: PMC1141539  PMID: 3164326

Abstract

A 52 year old man with newly diagnosed polycythaemia vera in proliferative phase developed widespread extramedullary haemopoiesis (EMH), and died as a result of cervical cord compression. At necropsy, microscopic areas of primitive cells characteristic of granulocytic sarcoma were found within a large tumour of EMH in the right retroperitoneal region. Ultrastructural analysis showed unusual hexagonal inclusions within the cytoplasm of these primitive cells. Clinicians should be aware of the possibility of discrete haemopoietic tumours, whether EMH or granulocytic sarcoma, in patients with polycythaemia vera as well as in other myeloproliferative disorders.

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Selected References

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  1. Adamson J. W., Fialkow P. J., Murphy S., Prchal J. F., Steinmann L. Polycythemia vera: stem-cell and probable clonal origin of the disease. N Engl J Med. 1976 Oct 21;295(17):913–916. doi: 10.1056/NEJM197610212951702. [DOI] [PubMed] [Google Scholar]
  2. Berk P. D., Goldberg J. D., Donovan P. B., Fruchtman S. M., Berlin N. I., Wasserman L. R. Therapeutic recommendations in polycythemia vera based on Polycythemia Vera Study Group protocols. Semin Hematol. 1986 Apr;23(2):132–143. [PubMed] [Google Scholar]
  3. Brown J. A., Gomez-Leon G. Subdural hemorrhage secondary to extramedullary hematopoiesis in postpolycythemic myeloid metaplasia. Neurosurgery. 1984 May;14(5):588–591. doi: 10.1227/00006123-198405000-00013. [DOI] [PubMed] [Google Scholar]
  4. Cameron W. R., Ronnert M., Brun A. Extramedullary hematopoiesis of CNS in postpolycythemic myeloid metaplasia. N Engl J Med. 1981 Sep 24;305(13):765–765. doi: 10.1056/NEJM198109243051315. [DOI] [PubMed] [Google Scholar]
  5. Clausen K. P. Reticulosarcomatosis in primary myelofibrosis. Cancer. 1968 Jul;22(1):136–141. doi: 10.1002/1097-0142(196807)22:1<136::aid-cncr2820220116>3.0.co;2-x. [DOI] [PubMed] [Google Scholar]
  6. Cornfield D. B., Shipkin P., Alavi A., Becker J., Peyster R. Intracranial myeloid metaplasia: diagnosis by CT and Fe52 scans and treatment by cranial irradiation. Am J Hematol. 1983 Nov;15(3):273–278. doi: 10.1002/ajh.2830150308. [DOI] [PubMed] [Google Scholar]
  7. Crawford D. C., Nightingale S., Bates D., Tomlinson B. E. Spinal cord compression by extramedullary haematopoiesis in myelofibrosis. Postgrad Med J. 1984 Jan;60(699):62–63. doi: 10.1136/pgmj.60.699.62. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Cromwell L. D., Kerber C. Spinal cord compression by extramedullary hematopoiesis in agnogenic myeloid metaplasia. Radiology. 1978 Jul;128(1):118–118. doi: 10.1148/128.1.118. [DOI] [PubMed] [Google Scholar]
  9. Eshghabadi M., Shojania A. M., Carr I. Isolated granulocytic sarcoma: report of a case and review of the literature. J Clin Oncol. 1986 Jun;4(6):912–917. doi: 10.1200/JCO.1986.4.6.912. [DOI] [PubMed] [Google Scholar]
  10. Gomez G., Hossfeld D. K., Sokal J. E. Removal of abnormal clone of leukaemic cells by splenectomy. Br Med J. 1975 May 24;2(5968):421–423. doi: 10.1136/bmj.2.5968.421. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Heffez D. S., Sawaya R., Udvarhelyi G. B., Mann R. Spinal epidural extramedullary hematopoiesis with cord compression in a patient with refractory sideroblastic anemia. Case report. J Neurosurg. 1982 Sep;57(3):399–406. doi: 10.3171/jns.1982.57.3.0399. [DOI] [PubMed] [Google Scholar]
  12. Lewkow L. M., Shah I. Sickle cell anemia and epidural extramedullary hematopoiesis. Am J Med. 1984 Apr;76(4):748–751. doi: 10.1016/0002-9343(84)90310-3. [DOI] [PubMed] [Google Scholar]
  13. Ligumski M., Polliack A., Benbassat J. Myeloid metaplasia of the central nervous system in patients with myelofibrosis and agnogenic myeloid metaplasia. Report of 3 cases and review of the literature. Am J Med Sci. 1978 Jan-Feb;275(1):99–103. doi: 10.1097/00000441-197801000-00012. [DOI] [PubMed] [Google Scholar]
  14. Lundh B., Brandt L., Cronqvist S., Eyrich R. Intracranial myeloid metaplasia in myelofibrosis. Scand J Haematol. 1982 Jan;28(1):91–94. doi: 10.1111/j.1600-0609.1982.tb02126.x. [DOI] [PubMed] [Google Scholar]
  15. McCarty K. S., Jr, Wortman J., Daly J., Rundles R. W., Hanker J. S. Chloroma (granulocytic sarcoma) without evidence of leukemia: facilitated light microscopic diagnosis. Blood. 1980 Jul;56(1):104–108. [PubMed] [Google Scholar]
  16. Meis J. M., Butler J. J., Osborne B. M., Manning J. T. Granulocytic sarcoma in nonleukemic patients. Cancer. 1986 Dec 15;58(12):2697–2709. doi: 10.1002/1097-0142(19861215)58:12<2697::aid-cncr2820581225>3.0.co;2-r. [DOI] [PubMed] [Google Scholar]
  17. Mitelman F., Brandt L., Nilsson P. G. Cytogenetic evidence for splenic origin of blastic transformation in chronic myeloid leukaemia. Scand J Haematol. 1974;13(2):87–92. doi: 10.1111/j.1600-0609.1974.tb00240.x. [DOI] [PubMed] [Google Scholar]
  18. Muss H. B., Moloney W. C. Chloroma and other myeloblastic tumors. Blood. 1973 Nov;42(5):721–728. [PubMed] [Google Scholar]
  19. Neiman R. S., Barcos M., Berard C., Bonner H., Mann R., Rydell R. E., Bennett J. M. Granulocytic sarcoma: a clinicopathologic study of 61 biopsied cases. Cancer. 1981 Sep 15;48(6):1426–1437. doi: 10.1002/1097-0142(19810915)48:6<1426::aid-cncr2820480626>3.0.co;2-g. [DOI] [PubMed] [Google Scholar]
  20. Nesland J. M., Langholm R., Marton P. F. Hexagonal crystals in the bone marrow in patients with myeloproliferative disease and preleukaemia. Scand J Haematol. 1984 May;32(5):552–558. doi: 10.1111/j.1600-0609.1984.tb02199.x. [DOI] [PubMed] [Google Scholar]
  21. Page B. M., Watt J. L., Reid I. N., Davidson R. J., Walker W. Clonal evolution of marker chromosomes in a case of myelofibrosis with myeloid metaplasia and myeloblastic transformation. Acta Haematol. 1979;61(6):301–309. doi: 10.1159/000207678. [DOI] [PubMed] [Google Scholar]
  22. Pettit J. E., Lewis S. M., Nicholas A. W. Transitional myeloproliferative disorder. Br J Haematol. 1979 Oct;43(2):167–184. doi: 10.1111/j.1365-2141.1979.tb03739.x. [DOI] [PubMed] [Google Scholar]
  23. Price F., Bell H. Spinal cord compression due to extramedullary hematopoiesis. Successful treatment in a patient with long-standing myelofibrosis. JAMA. 1985 May 17;253(19):2876–2877. [PubMed] [Google Scholar]
  24. Rice G. P., Assis L. J., Barr R. M., Ebers G. C. Extramedullary hematopoiesis and spinal cord compression complicating polycythemia rubra vera. Ann Neurol. 1980 Jan;7(1):81–84. doi: 10.1002/ana.410070115. [DOI] [PubMed] [Google Scholar]
  25. Robitaille G. A., Eisenberg M., Lehman R. Intracranial extramedullary hematopoiesis in polycythemia vera. Conn Med. 1985 Mar;49(3):149–151. [PubMed] [Google Scholar]
  26. Stavem P., Ly B., Bjorneklett A. Light green crystals in May-Grünwald and Giemsa-stained bone marrow macrophages in patients with myeloid leukaemia. Scand J Haematol. 1977 Jan;18(1):67–72. doi: 10.1111/j.1600-0609.1977.tb01479.x. [DOI] [PubMed] [Google Scholar]
  27. Ward H. P., Block M. H. The natural history of agnogenic myeloid metaplasia (AMM) and a critical evaluation of its relationship with the myeloproliferative syndrome. Medicine (Baltimore) 1971 Sep;50(5):357–420. doi: 10.1097/00005792-197109000-00001. [DOI] [PubMed] [Google Scholar]
  28. Wolf D. J., Fialk M. A., Mouradian J., Gottfried E. L., Pasmantier M. W. Unusual intracytoplasmic inclusions in acute myeloblastic leukemia. Am J Hematol. 1980;9(4):413–420. doi: 10.1002/ajh.2830090408. [DOI] [PubMed] [Google Scholar]

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