Making The Diagnosis

The clinical diagnosis of AMM is highly unlikely in the absence of a palpable spleen. Examination of the peripheral blood smear is very important, as typical teardrop RBCs, immature granulocytes, nucleated ery-throid cells and abnormalities of platelet and megakary-ocytic morphology are seen in the blood film. The diagnosis of early myelofibrosis requires an experienced hematopathologist, but as the disease advances, the degree of fibrosis and osteosclerosis makes the disease readily apparent. As in ET, about 50% of patients express abnormality of JAK2 in the periferal blood or marrow.23 In about 20% of patients with CML, significant fibrosis is noted when the patient is first encountered.2 Therefore, appropriate cytogenetic and molecular tests to exclude CML should be performed in patients with AGM.

13. Gruppo Igaliano Studio Polycythemia. Polycythemia vera: the natural history of 1213 patients followed for 20 years. Ann Intern Med 123:656, 1995.

14. Kralovics R, Passamonti F, Buser AS, Teoss, Tiedt R Passweg JR, Tichelli A, Cazzola M, Skoda R. New Eng J Med 352:1779-1790, 2005.

15. Najfeld V, Montella L, Scalise A, et al.: Exploring poly-cythemia vera with FISH: additional cryptic 9p is the most frequent abnormality detected. Br J Haematol 119(2):558, 2002.

16. Moliterno AR, Hankins WD, Spivak JL: Impaired expression of the thrombopoietin receptor by platelets from patients with polycythemia vera. N Engl J Med 338(9):572, 1998.

17. Horikawa Y, Matsumura I, Hashimoto K, et al.: Markedly reduced expression of platelet c-mpl receptor in essential thrombocythemia. Blood 90(10):4031.

18. Temerinac S, Klippel S, Strunck E, et al.: Cloning of PRV-1, a novel member of the uPAR receptor superfamily, which is overexpressed in polycythemia rubra vera. Blood 95(8):2569, 2000.

19. Jones AV, Kreil S, Zoi K, et al.: Widespread occurence of the JAK2 V617F mutation in chronic myeloproliferative disorders. Blood. 106(6):2162-8, Sep 15 2005.

20. Michiels JJ: Bone marrow histopathology and biological markers as specific clues to the differential diagnosis of essential thrombocythemia, polycythemia vera and pre-fibrotic agnogenic myeloid metaplasia. Hematol J 5(2): 93, 2004.

21. Thiele J, Kvasnicka HM: Chronic myeloproliferative disorders with thrombocythemia: a comparative study of two classifications systems (VSG, WHO) on 839 patients. Ann Hematol 82: 148, 2003.

22. Silverstein MN, Wollaeger EE, Baggenstoss AH: Gastrointestinal and abdominal manifestations of agnogenic myeloid metaplasia. Arch Intern Med 131:532, 1973.

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Chapter 48

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