Bone marrow neoplastic
Bone marrow - neoplastic myeloid
MDS / MPN
MDS / MPN - unclassifiable
Author: Nikhil Sangle, M.D.
Last author update: 1 August 2011
Last staff update: 20 July 2022
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: MDS/MPN unclassifiable
Cite this page: Sangle N. MDS / MPN - unclassifiable. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/myeloproliferativemdsmpnunclassified.html. Accessed April 24th, 2024.
Definition / general
- This is a provisional myelodysplasia / myeloproliferative disorder, with JAK2 V617F mutation present in 48 - 67% (Haematologica 2008;93:34, Blood 2006;108:2173)
- 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
- 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)
