Myelofibrosis pathophysiology

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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, a myeloproliferative disorder, is characterized by the proliferation of megakaryocytes in the bone marrow, disrupted cytokine production, and reactive fibrosis resulting in bone marrow failure. The fibrosed and scarred bone marrow produces fewer and fewer normal functioning blood cells leading to pancytopenia and extramedullary hematopoiesis (EMH). It can mainly be associated with somatic mutation of the myeloproliferative leukemia virus (MPL) oncogene, the calreticulin (CALR) gene, or Janus kinase 2 (JAK2) gene but other genes can also be involved and it can also result in the setting of another primary insult. Associated conditions include malignancies, hematologic disorders, infections, autoimmune diseases, and endocrine disorders. Hepatomegaly, splenomegaly, and lymphadenopathy are the positive findings on gross pathology. On microscopic pathology, myelofibrosis is characterized by low RBC count, leukopenia - leukocytosis, basophilia, erythroblastosis, thrombocytopenia - thrombocytosis, micromegakaryocytes, splenic pulp changes, and abnormalities of platelets.

Pathophysiology

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Microscopic Pathology

On Light Microscopy

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On Electron Microscopy

  • Ultrastructural abnormalities in platelets such as:
  • Splenic findings can consist of the following:

Images

Histopathological image showing teardrop poikilocytes (yellow arrows) [100]
Histopathological image showing essential thrombocytosis (yellow arrows) [101]


References

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  2. ↑ Vainchenker W, Kralovics R (February 2017). "Genetic basis and molecular pathophysiology of classical myeloproliferative neoplasms". Blood. 129 (6): 667–679. doi:10.1182/blood-2016-10-695940. PMID 28028029.
  3. ↑ 3.0 3.1 3.2 Alshemmari SH, Rajan R, Emadi A (2016). "Molecular Pathogenesis and Clinical Significance of Driver Mutations in Primary Myelofibrosis: A Review". Med Princ Pract. 25 (6): 501–509. doi:10.1159/000450956. PMC 5588514. PMID 27756071.
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  7. ↑ 7.0 7.1 7.2 Reilly JT, Barnett D, Dolan G, Forrest P, Eastham J, Smith A (January 1993). "Characterization of an acute micromegakaryocytic leukaemia: evidence for the pathogenesis of myelofibrosis". Br. J. Haematol. 83 (1): 58–62. PMID 8435338.
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  73. ↑ Thiele J, Kvasnicka HM (August 2005). "Hematopathologic findings in chronic idiopathic myelofibrosis". Semin. Oncol. 32 (4): 380–94. doi:10.1053/j.seminoncol.2005.04.010. PMID 16202684.
  74. ↑ Hernández-Boluda JC, Correa JG, García-Delgado R, Martínez-López J, Alvarez-Larrán A, Fox ML, García-Gutiérrez V, Pérez-Encinas M, Ferrer-Marín F, Mata-Vázquez MI, Raya JM, Estrada N, García S, Kerguelen A, Durán MA, Albors M, Cervantes F (April 2017). "Predictive factors for anemia response to erythropoiesis-stimulating agents in myelofibrosis". Eur. J. Haematol. 98 (4): 407–414. doi:10.1111/ejh.12846. PMID 28009442.
  75. ↑ Tóth P, Tóth Z (2002). "Idiopathic myelofibrosis with prominent postsplenectomy erythroblastosis terminating in acute myeloid transformation". Haematologia (Budap). 32 (2): 155–61. PMID 12412736.
  76. ↑ Osman Y, Kishi K, Narita M, Saito H, Masuko M, Koike T, Shibata A (June 1996). "Idiopathic myelofibrosis with unusually high erythroblastosis in the peripheral blood". Am. J. Hematol. 52 (2): 122–3. doi:10.1002/(SICI)1096-8652(199606)52:2<122::AID-AJH12>3.0.CO;2-J. PMID 8638637.
  77. ↑ Robier C, Körber C, Quehenberger F, Neubauer M, Wölfler A (January 2018). "The frequency of occurrence of fish-shaped red blood cells in different haematologic disorders". Clin. Chem. Lab. Med. 56 (2): 323–326. doi:10.1515/cclm-2017-0378. PMID 28731851.
  78. ↑ Biagini G, Severi B, Govoni E, Preda P, Pileri S, Martinelli G, Visani G, Finelli C, Castaldini C (1985). "Stromal cells in primary myelofibrosis: ultrastructural observations". Virchows Arch., B, Cell Pathol. 48 (1): 1–8. PMID 2580387.
  79. ↑ Egelé A, van Gelder W, Riedl J (December 2015). "Automated detection and classification of teardrop cells by a novel RBC module using digital imaging/microscopy". Int J Lab Hematol. 37 (6): e153–6. doi:10.1111/ijlh.12399. PMID 26118701.
  80. ↑ Madelung A, Bzorek M, Bondo H, Zetterberg E, Bjerrum OW, Hasselbalch HC, Scheding S, Ralfkiaer E (March 2012). "A novel immunohistochemical sequential multi-labelling and erasing technique enables epitope characterization of bone marrow pericytes in primary myelofibrosis". Histopathology. 60 (4): 554–60. doi:10.1111/j.1365-2559.2011.04104.x. PMID 22250648.
  81. ↑ 81.0 81.1 Lundberg LG, Lerner R, Sundelin P, Rogers R, Folkman J, Palmblad J (July 2000). "Bone marrow in polycythemia vera, chronic myelocytic leukemia, and myelofibrosis has an increased vascularity". Am. J. Pathol. 157 (1): 15–9. doi:10.1016/S0002-9440(10)64511-7. PMC 1850191. PMID 10880370.
  82. ↑ 82.0 82.1 Magyari F, Bedekovics J, Décsy J, Ilonczai P, Illés Á, Simon Z (April 2018). "[Investigation and treatment of prefibrotic/early primary myelofibrosis. A case study]". Orv Hetil (in Hungarian). 159 (15): 603–609. doi:10.1556/650.2018.30995. PMID 29631428.
  83. ↑ 83.0 83.1 Barraco D, Lasho TL, Gangat N, Finke C, Elala YC, Pardanani A, Tefferi A (June 2016). "Leukocytosis and presence of CALR mutation is associated with non-hepatosplenic extramedullary hematopoiesis in primary myelofibrosis". Blood Cancer J. 6: e436. doi:10.1038/bcj.2016.44. PMC 5141359. PMID 27315113.
  84. ↑ 84.0 84.1 Beauverd Y, Alimam S, McLornan DP, Radia DH, Harrison CN (October 2016). "Disease characteristics and outcomes in younger adults with primary and secondary myelofibrosis". Br. J. Haematol. 175 (1): 37–42. doi:10.1111/bjh.14173. PMID 27293069.
  85. ↑ 85.0 85.1 Ota S, Hiramatsu Y, Kondo E, Kasahara A, Takada S, Umena S, Noguchi T, Tanimoto M, Matsumura T (December 2014). "Severe case of peripheral leukocytosis initially diagnosed as myelodysplastic syndrome/myeloproliferative neoplasm, unclassifiable, but possibly prefibrotic primary myelofibrosis". Acta Med. Okayama. 68 (6): 363–8. doi:10.18926/AMO/53025. PMID 25519030.
  86. ↑ 86.0 86.1 Scotch AH, Kosiorek H, Scherber R, Dueck AC, Slot S, Zweegman S, Boekhorst P, Commandeur S, Schouten H, Sackmann F, Fuentes AK, Hernández-Maraver D, Pahl HL, Griesshammer M, Stegelmann F, Döhner K, Lehmann T, Bonatz K, Reiter A, Boyer F, Etienne G, Ianotto JC, Ranta D, Roy L, Cahn JY, Harrison CN, Radia D, Muxi P, Maldonado N, Besses C, Cervantes F, Johansson PL, Barbui T, Barosi G, Vannucchi AM, Paoli C, Passamonti F, Andreasson B, Ferrari ML, Rambaldi A, Samuelsson J, Birgegard G, Xiao Z, Xu Z, Zhang Y, Sun X, Xu J, Kiladjian JJ, Zhang P, Gale RP, Mesa RA, Geyer HL (December 2017). "Symptom burden profile in myelofibrosis patients with thrombocytopenia: Lessons and unmet needs". Leuk. Res. 63: 34–40. doi:10.1016/j.leukres.2017.10.002. PMID 29096334. Vancouver style error: initials (help)
  87. ↑ 87.0 87.1 Wassie E, Finke C, Gangat N, Lasho TL, Pardanani A, Hanson CA, Ketterling RP, Tefferi A (April 2015). "A compendium of cytogenetic abnormalities in myelofibrosis: molecular and phenotypic correlates in 826 patients". Br. J. Haematol. 169 (1): 71–6. doi:10.1111/bjh.13260. PMID 25521305.
  88. ↑ 88.0 88.1 Guglielmelli P, Rotunno G, Pacilli A, Rumi E, Rosti V, Delaini F, Maffioli M, Fanelli T, Pancrazzi A, Pieri L, Fjerza R, Pietra D, Cilloni D, Sant'Antonio E, Salmoiraghi S, Passamonti F, Rambaldi A, Barosi G, Barbui T, Cazzola M, Vannucchi AM (September 2016). "Prognostic impact of bone marrow fibrosis in primary myelofibrosis. A study of the AGIMM group on 490 patients". Am. J. Hematol. 91 (9): 918–22. doi:10.1002/ajh.24442. PMID 27264006.
  89. ↑ 89.0 89.1 Xu Z (February 2017). "MDS/MPN with ring sideroblasts and thrombocytosis masquerading as prefibrotic/early primary myelofibrosis". Blood. 129 (5): 657. doi:10.1182/blood-2016-11-749937. PMID 28153839.
  90. ↑ 90.0 90.1 Cheminant M, Delarue R (August 2013). "[Investigation and management of patients presenting with thrombocytosis]". Rev Med Interne (in French). 34 (8): 465–71. doi:10.1016/j.revmed.2013.02.020. PMID 23498669.
  91. ↑ Koumas S, Prokopiou C, Lerni M, Seimeni O, Neokleous N (August 2015). "Isochromosome 17q10 associated with basophilia in primary myelofibrosis while with JAK2 inhibitor". Ann. Hematol. 94 (8): 1421–2. doi:10.1007/s00277-015-2380-5. PMID 25900789.
  92. ↑ Rautenbach Y, Goddard A, Clift SJ (March 2017). "Idiopathic myelofibrosis accompanied by peritoneal extramedullary hematopoiesis presenting as refractory ascites in a dog". Vet Clin Pathol. 46 (1): 46–53. doi:10.1111/vcp.12430. PMID 27874969.
  93. ↑ Takimoto Y, Imanaka F, Hayashi Y, Shindo H (1997). "A patient with basophilic-eosinophilic myeloproliferative disorder showing monosomy 7 and hyperhistaminemia". Acta Haematol. 98 (1): 37–41. doi:10.1159/000203559. PMID 9210912.
  94. ↑ Muth M, Büsche G, Bock O, Hussein K, Kreipe H (November 2010). "Aberrant proplatelet formation in chronic myeloproliferative neoplasms". Leuk. Res. 34 (11): 1424–9. doi:10.1016/j.leukres.2010.03.040. PMID 20430444.
  95. ↑ Kreipe H, Büsche G, Bock O, Hussein K (2012). "Myelofibrosis: molecular and cell biological aspects". Fibrogenesis Tissue Repair. 5 (Suppl 1): S21. doi:10.1186/1755-1536-5-S1-S21. PMC 3368793. PMID 23259436.
  96. ↑ Shimizu S, Onodera Y, Nakamura Y, Ide K, Ayabe T, Isobe H, Nagoshi H, Someya K (December 1989). "[Megakaryocyte proportion versus nucleated cells in the peripheral blood showing leukoerythroblastosis]". Rinsho Ketsueki (in Japanese). 30 (12): 2141–7. PMID 2621794.
  97. ↑ Thiele J, Vykoupil KF, Georgii A (1980). "Ultrastructure of blastic crisis in osteomyelofibrosis. A report of 2 cases with some unusual features". Virchows Arch A Pathol Anat Histol. 389 (3): 287–305. PMID 6935866.
  98. ↑ Hattori A, Koike K, Ito S, Matsuoka M (1975). "Static and functional morphology of the pathological platelets in primary myelofibrosis and myeloproliferative syndrome". Ser Haematol. 8 (1): 126–50. PMID 1129598.
  99. ↑ Tavassoli M, Weiss L (August 1973). "An electron microscopic study of spleen in myelofibrosis with myeloid metaplasia". Blood. 42 (2): 267–79. PMID 4793115.
  100. ↑ By Osaretin - Own work, CC BY-SA 4.0, https://commons.wikimedia.org/w/index.php?curid=36814333
  101. ↑ By Erhabor Osaro (Associate Professor) - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=32131622
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