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Myelofibrosis PowerPoint Presentation

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Description : myelofibrosis

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Published on : Nov 22, 2017 | Views : 3009 | Downloads : 21

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MYELOFIBROSIS Dr izyan syakirah Mohamed fauad
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PRIMARY MYELOFIBROSIS Also known as myelosclerosis, agnogenic myeloid metaplasia or idiopathic myelofibrosis. Is a clonal myeloproliferative disorder characterized by increase fibrosis within the marrow, splenomegaly and extramedullary haemopoiesis in the spleen, liver and at times in lymph node, kidneys and adrenals. Idiopathic myelofibrosis is indistinguishable from myelofibrosis secondary to polycythemia vera or essential thrombocythemia and the diagnostic criteria for these conditions are the same.
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Hoffbrand Essential Haematology 7Th Edition, Myeloproliferative Disease
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PATHOPHYSIOLOGY In myelofibrosis, proliferation of multiple cell lineages is accompanied by progressive bone marrow fibrosis. Marrow fibrosis is thought to be secondary to the release of proinflammatory cytokines and growth factors from abnormal clonal cells (primarily megakaryocytes), which act to stimulate fibroblast proliferation, fibrosis, angiogenesis and new bone formation. Transforming growth factor ß (TGF-ß) Basic fibroblast growth factors (bFGF) Vascular endothelial growth factor (VEGF) As with PV and ET, erythroid and megakaryocytic colonies can also be derived in the absence of exogenous growth factors.
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Clinical features Usually occurs in middle aged and elderly patients. Symptoms of anaemia like lassitude, fatigue, weakness and anorexia. Symptoms due to massive splenomegaly like abnormal distention, dyspnea and dragging discomfort in the left hypochondrium. Splenic infarcts are common sequelae of splenomegaly. Symptoms resulting from hypermetabolic state like fever, weight loss, sweating and heat intolerance. In late stages, bleeding tendencies occurs due to thrombocytopenia. Hepatomegaly with portal hypertension and esophageal varices, lymphadenopathy, ascites, cardiac failure, gout and jaundice also seen.
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Colour Atlas of Clinical Hematology 4th edition, Myelofibrosis
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Laboratory findings Peripheral blood film Anemia is usual but a normal or increased haemoglobin level maybe found in some patients. Leucocytosis and thrombocytosis at the time presentation. Later in the disease leucopenia and thrombocytopenia are common. A leucoerythroblastic blood film (simultaneous presence of erythroid precursors and granulocytic precursors are seen). Marked anisopoikilocytosis and the red cells show characteristic ‘tear-drop’ poikilocytes. Basophilic stippling, giant platelets and megarkayocyte fragments may also be present.
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Peripheral blood films showing leucoerythroblastic changes with red polychormasia, anisocytosis, poikilocytosis including “tear-drop” forms ( the nucleated cells are erythroblast and a late myelocte).
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Peripheral blood films showing myelocytes, erythroblast and megakaryocyte fragments (A) ; at higher magnification, megakaryocyte fragments (B).
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Laboratory findings Bone Marrow examination Attempts at bone marrow aspiration often yield a dry tap or hemodilute sample. Early stage or ‘cellular phase’ : the marrow is hypercellular with an increase in all three cell lines particularly megakaryocytes (abnormal large megakaryocytes in cluster). Fibrosis is minimal. Late stage or ‘hypocellular phase’ : the marrow is hypocellular with reduction in all cell lines. Marked increase in fibrosis. The degree of fibrosis is best demonstrated using silver impregnation which stains reticulin fibres and collagen fibres are best demonstrated using a trichrome stain. The degree of fibrosis can be graded from 0-4 (Bauermeister) or MF-0 to 3 (EU consensus) according to severity.
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British Journal of Haematology Volume 139, Issue 3, Version of Record online: 1 OCT 2007
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Trephine biopsies showing most of the intertrabecular space occupied by cellular loose connective tissue contains scattered hematopoietic cells, including prominent megakaryocyte, fat cells, making up less than 15% of the intertrabecular space and extensive deposition of loose connective tissue around hematopoietic cells (1) ; the prominence of megakaryocyte in the myelofibrotic tissue at higher magnification (2).
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Laboratory findings Genetic test Sufficient material obtained either from bone marrow or peripheral blood to assess the karyotype which can help to exclude diagnosis such as CML (Philadelphia chromosome is negative). The vast majority of patients with PMF demonstrate mutually exclusive mutations in JAK2, MPL, or CALR ●JAK2 mutation – 60 to 65 percent ●CALR mutation – 20 to 25 percent ●MPL mutation – 5 percent ●No JAK2, CALR, or MPL mutation ("triple negative") – 8 to 10 percent Abnormalities of chromosome 5 and 7 are usually found in patients with prior exposure to genotoxic agents and associated with a poor prognosis. Other test Leukocyte alkaline phosphatase (LAP) score is elevated. High serum urate LDH levels increased.
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Diagnostic criteria (The 2016 revision to the World Health Organization classification )
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http://www.bloodjournal.org/content/bloodjournal/127/20/2391.full.pdf?sso-checked=true
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Diagnostic criteria (BSH 2015) Postgraduate Hematology 7th edition, Myeloproliferative Neoplasm
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tREATMENTS Allogeneic stem cell transplantation JAK2 inhibitor : Ruxolitinib Antiangiogenic drugs: Thalidomide Cytoreductive therapy : hydroxycarbamide and anagrelide Splenectomy Splenic irradiation
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prognosis Has the poorest prognosis of the MPNs. The median survival is 3-5 years. Cause of death includes : Heart failure Infection Leukaemic transformation
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References Postgraduate Haematology 7th edition edited by Hoffbrand, Higgs, Keeling and Mehta Hoffbrand’s Essential Haematology 7th edition by A. Victor Hoffbrand , Paul A.H Moss Color Atlas of Clinical Hematology 4th edition by A. Victor Hoffbrand, John E. Pettit, Paresh Vyas Robbins and Cotran Pathologic Basis of Disease 9th edition by Kumas,Abbas, Aster
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Thank you!