Myelodysplastic/Myeloproliferative Disorders. MDS/MPD Unclassified Provisional: CMML JMML acml RARS T. K. Foucar November 2007 SH/EAHP Workshop
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1 Myelodysplastic/Myeloproliferative Disorders CMML JMML acml MDS/MPD Unclassified Provisional: RARS T K. Foucar November 2007 SH/EAHP Workshop
2 MDS/MPD: General Features Blood: Cytosis and cytopenia(s) Dysplasia BM: Both dysplastic and proliferative features at presentation. DeNovo hybrid Not progression of CMPD or MDS. disorder:
3 MDS/MPD: Cases Submitted: 28 Cases Moved to Other Category: 5 Submitter/Panel Agreement: 15 Submitter/ Panel Discordance: 11 Missing Critical Slides to Reach Dx: 2
4 MDS/MPD: Key Problem Areas RARS T vs CMPD with RS vs CMML vs MDS/MPD U CMML vs AMML acml vs MDS/MPD U Does prior therapy mean therapyrelated? Where do DS related disorders belong?
5 MDS/MPD Case Presentations Presenter Case # Dx L. Contis 038 RARS T vs MPD A. demascarel 150 CMML 2 T. Molina 154 RARS T A. Schmitt Graeff 202 RARS T F. Fend 237 acml A. Orazi 238 MDS/MPD U
6 CASE PRESENTATIONS
7 Case # Summary: CMML Submitter Proposed Dx Panel Dx Comments 072 Roullet RARS T CMML AMC Hanson CMML CMML Borderline AMML 138 Bot CMML > AMML MDS >AML Hx of MDS 139 Yared MDS/MPD U CMML AMC demascarel CMML CMML2 Promonocyte def n 198 Gratzinger CMML, eos CMML, eos 7, t(12;14), (PDGFRβ)
8 Blood: CMML Case # 139, Yared
9 Monocytes Vs Promonocytes Case# 150, demascarel Case# 198, Gratzinger
10 Promonocytes
11 BM Aspirate: CMML Case # 139, Yared
12 CMML: Cytochemical Stains BMA: CMML Case # 111, Hanson
13 CMML: PDGFRβ tx Case# 198, Gratzinger
14 CMML: Strategies Absolute monocytosis is key factor even when WBC not significantly elevated. May see ringed sideroblasts, eosinophilia Delineation of promonocytes critical (these are blast equivalents and may lead to dx of AMML)
15 Summary: JMML Case # Submitter Proposed Dx Panel Dx Comment 016 Dunphy JMML JMML Nl CC; hypersen. GM CSF 205 Wang JMML JMML Nl CC; hypersen. GM CSF
16 JMML: Strategies Majority (75%) of patients <3 years of age. Increase in granulocytic and monocytic cells Exclude AMML promonocyte ID Exclude CML Mono7, inc Hgb F, hypersensitivity to GM CSF
17 Blood: JMML Courtesy W. Finn
18 BM Aspirate: JMML ANBE Case 016, Dunphy
19 Flow Cytometry: JMML Case # 016, Dunphy
20 Summary: Atypical CML Case # Submitter Proposed Dx Panel DX 015 Dunphy acml MDS/MPD U (1900 AMC) 237 Fend acml acml
21 Blood: Atypical CML 68 y. female, WBC 116, RBC, Plt
22 BM Atypical CML CD34 68 y. female, WBC 116, RBC, Plt BCR/ABL-, JAK2-
23 BM: Atypical CML Case # 237, Fend CD34 MPO
24 Atypical CML: Strategies Blood is distinctive Prominent granulocytic dysplasia No basophilia No monocytosis BM Should mimic CML in that mature granulocytic cells predominate Exclude CML, AML, CMML
25 Summary: Provisional RARS T Case # Submitter Proposed Dx Panel Dx Comments 038 Jacob RARS T CIMF with RS (split) 126 Orduz RARS T MDS/MPD U (Split) 128 McClune MDS/MPD with Plts CIMF with RS 154 Molina RARS T RARS T vs CIMF (split) 168 Kelley RARS T MDS/MPD U (split) Megas MPD like JAK2 positive Inv(11),?JAK2 JAK Gratzinger RARS T Unclassified Hx of MDS 202 Schmtt Graeff RARS T α Thal RARS T
26 RARS T vs MPD Case # 128, McClure
27 BMA: RARS-T vs CIMF vs MDS/MPD-U 126, Orduz 195, Gratzinger 168, Kelley 168, Kelley
28 BM Bx: RARS T vs CIMF vs MDS/MPD U 126, Orduz 128, McClure 128, Kelley 195, Gratzinger
29 RARS T: Strategies/Issues Too much emphasis on ringed sideroblasts Also need to exhibit increased erythroid cells, dysplastic RBC s If megas have MPD features, favors CMPD If monocytosis present, favors CMML
30 Summary: MDS/MPD U Case # Submitter Proposed Dx Panel Dx Comments 015 Dunphy acml MDS/MPD U Monocytosis; basophilia 031 Zhao MDS/MPD U MDS/MPD U Neg BCR/ABL, JAK2, Flt3 098 Rao MDS/MPD U MDS/MPD U Del(20)(q11.2), monocytosis 126 Orduz RARS T MDS/MPD U 155 Nguyen MDS/MPD U MDS/MPD U Tri8, neg PDGFRα,β 189 Alobeid MDS/MPD U MDS/MPD U Marked fibrosis 193 Bhagat MDS/MPD U MDS/MPD U Cardiac tx, later T LL, del 5(q15q31)
31 Blood: MDS/MPD-U Case 015, Dunphy Case 098, Rao
32 BM Aspirate: MDS/MPD-U Case # 015, Dunphy Case # 155, Nguyen
33 BM bx: MDS/MPD-U Case # 031, Zhao Case # 015, Dunphy
34 BM bx: MDS/MPD-U Case # 098, Rao
35 BM bx: MDS/MPD-U Retic Case # 189, Alobeid
36 MDS/MPD: Strategies Must be denovo hybrid dysplastic/proliferative picture (no prior MDS or MPD) Megakaryocyte morphology (if MPD like, favors MPD) Prior chemotherapy suggests is therapyrelated
37 MDS/MPD: Unresolved Issues Lack of consensus MDS/MPD vs MDS MDS/MPD vs MPD MDS/MPD vs AML Role of Genetics in Classification JAK2 mutations t(5;12) (q31;p12) t(5;10) (q33;q22) NF1 mutations Other
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