pei lin professor of pathology department of hematopathology md anderson cancer center...
TRANSCRIPT
Clinical History
• 80-year-old man with a history of HTN, neuropathy and chronic anemia
• Came to MDACC to see Dr. Garcia-Manero on 8/2/2007 for an outside diagnosis of Refractory anemia or CMML one year ago
• Transfusion independent and untreated. • Chronic fatigue, dizziness • PE: o hepatosplenomegaly or peripheral adenopathy
Outside BM biopsy (3/7/2007)
WBC 4.7, Hgb 11.1, MCV 93, Platelet 341, Neutrophils 41.3, Lymphs 32.3, Monos 25.3 (abs: 1189), Eos 0.9, Basos 0.2,
• Hypercellular bone marrow (70-90%) with left-shifted granulopoiesis, megakaryocytic hyperplasia, dyserythropoiesis and 3% blasts (see comment)
Outside BM (comment):
• Flow cytometry immunophenotypic analysis of a sample from the bone marrow aspirate at Dianon Systems, Stratford, Connecticut demonstrated non-specific findings including left-shifted granulopoiesis with decreased expression of CD16.
Lab (8/2007 MDACC)
• WBC: 5.7, hemoglobin 12, and platelet 219• Neutrophils 67%, lymphocytes 17%, monocytes
16% (abs: 912)• Ferritin level is 915, Epo: 45.8.
RAS mutation negative
5/11/2010
• WBC: 7.6, HGB: 9.0, MCV: 94, Platelet: 273, Neutrophils: 68.0, Lymphs: 11.0, Monos: 16.0, Eos: 2.0, Metas: 3.0
JAK2 V617F PCR: 41%RAS mutation: neg
Pomalidomide 5/21/2010 Danazol 1/7/2011JAK2 inhibitor 4/2011
Risk Stratification
• IPSS, International Prognostic Score System
• DIPSS, dynamic IPSS• DIPSS, DIPSS plus • MIPSS, Molecular IPS • GPSS, Genetics-Based
PSSMascarenhas J Looking forward: novel therapeutic approaches in chronic and advanced phases of myelofibrosis in ASH education book: 2015
Monocytosis is an adverse prognostic factor for survival in younger patients with primary myelofibrosis
• 129 patients PMF ≤ 60 yrs. • Range: 18–60, median 52)• WBC: 30 k/uL• Hb: 10 g/dL• PLT 100 k/uL
W, H, P, M
W, H, P
WH
M.A. Elliott, S. Verstovsek, D. Dingli, S.M. Schwager, R.A. Mesa, C.Y. Li, A. Tefferi Leuk Res 2007;31:1503-9.
Proposed Diagnosis and Take home points
Dx: Primary myelofibrosis presenting with persistent monocytosis, JAK2 V617F +
• Monocytosis may occur as initial presentation of PMF or during the disease course
• Persistent monocytosis is associated with an adverse prognosis
• May not be associated with RAS mutations or cytogenetic aberrations
• Megakaryocytic dysplasia may be diagnostic clue