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Chronic Myeloid Neoplasms

MDS/MPN - unclassifiable

Reviewer: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 12 August 2011, last major update August 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● This is a provisional myelodysplasia/myeloproliferative disorder (Haematologica 2008;93:34, Blood 2006;108:2173), with JAK2-V617F mutation present in 48-67%
● Diagnostic criteria: features of one of the myelodysplasia / myeloproliferative categories, but no history of MDS/MPN, no history of growth factor or cytotoxic therapy, no BCR-ABL1 gene fusion, no rearrangement of PDGFRA, PDGFRB or FGFR1, no del(5q), no inv(3)(q21;q26) OR de novo disease with MPN and MDS features which cannot be assigned to any specific category of MDS / MPN
● Includes refractory anemia with ringed sideroblasts (RARS) with dyserythropoiesis and marked thrombocytosis (RARS-T) with megakaryocytes similar to those in ET, PV or primary myelofibrosis

Case reports
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● Essential thrombocythemia with ringed sideroblasts (Arch Pathol Lab Med 2004;128:815)
● Prominent erythroid dysplasia and t(8;9) progressing to acute erythroid leukemia (Hum Pathol 2005;36:1148)
● Unclassified disorder with thrombocytosis and splenomegaly (Ann Hematol 2002;81:308)

Micro images
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Peripheral blood:

Essential thrombocythemia with ringed sideroblasts
Fig 1: peripheral blood shows occasional giant platelets, fig 2: hypercellular (70%) marrow
Fig 3: clusters of megakaryocytes with hyperlobulation; fig 4: iron stain shows ringed sideroblasts

End of Chronic Myeloid Neoplasms > MDS/MPN - unclassifiable

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