Table 1 Summary of retrospective studies on patients with blast phase myeloproliferative neoplasms (some studies have included patients with accelerated phase disease) (References cited in the text).
From: Blast phase myeloproliferative neoplasm: contemporary review and 2024 treatment algorithm
Study | MPN-BP type | Driver mutation | Karyotype | Treatment N (%) | Response | AHSCT N (%) | Survival | Risk factors | Impact of treatment on survival |
---|---|---|---|---|---|---|---|---|---|
Tefferi et al. Leukemia 2018 Mayo cohort N = 248 Median age 67 years | Post-PMF (N = 118) Post-ET/PV (N = 130) | JAK2 (68%) | Abnormal (81%) Adverse (23%) | Intensive chemo 69 (28) HMA 26 (11) Other agents 30 (12) | Intensive chemo 59% CR/CRi HMA 3% CR/CRi | 24 (10) | Median 3.6 months 1/3/5-year 17/6/4% | Karyotype Thrombocytopenia age >65 years Transfusions | 1/3/5-year: AHSCT 66/32/10% CR/CRi without AHSCT 37/19/13% No CR/CRi No AHSCT 8/1/1% |
Tefferi et al. Leukemia 2018 Florence cohort N = 162 Median age 69 years | Post-PMF (N = 70) Post-ET/PV (N = 92) | JAK2 (61%) | Abnormal (61%) | Intensive chemo 48 (30) | Intensive chemo 35.4% CR/CRi | 25 (15) | Median 3.6 months 1/3-year 25/11% | - | 1/3-year: AHSCT in CR/CRi 69/30% AHSCT without CR/CRi 62/38% |
Abdelmagid et al. Haematologica 2023 N = 103 Median age 70 years | Post-PMF (N = 35) Post-ET/PV (N = 64) | JAK2 (67%) | Abnormal (83%) | Intensive chemo 35 (35) HMA 21 (21) HMA+venetoclax 12 (12) | Intensive chemo 15% CR/CRi | 11 (11) | Median 6.7 months No ruxolitinib exposure Median 3.7 months with ruxolitinib exposure | Age >65 years, Karyotype Thrombocytopenia Ruxolitinib | 3-year/Median: Intensive chemo without AHSCT 3%/4.7 months Less intensive chemo without AHSCT 3%/5.4 months |
Mesa et al. Blood 2005 N = 91 Median age 66 years | Post-PMF (N = 49) Post-ET/PV (N = 42) | - | Abnormal (91%) Adverse (61%) | Intensive chemo 24 (26) Less intensive chemo 19 (21) | Intensive chemo 0% CR 41% reverted to Chronic phase | - | Median 2.7 months | - | Median: Intensive chemo 3.9 months Less intensive chemo 2.9 months |
Tokumori et al. Clinical Lymphoma Myeloma & Leukemia 2022 N = 75 Median age 66 years | - | JAK2 (71%) MPL (14%) | - | Intensive chemo 28 (37) HMA based, 28 (37) | Intensive chemo 70% CR/CRi HMA based 29% CR/CRi | 15 (20) | Median 4.8 months | No CR/CRi No AHSCT | Median: Intensive chemo 11.4 months Less intensive chemo HMA 4.7 months AHSCT not reached |
Mollard et al. Leuk Lymphoma 2018 N = 122 Median age 66 years AP-MPN, N = 48 BP-MPN, N = 74 | Post-PMF (N = 30) Post-ET/PV (N = 92) | JAK2 (61%) CALR (7%) MPL (3%) | Adverse (61%) | Intensive chemo 13 (11) HMA 24 (20) | Intensive chemo 62% CR/CRi HMA 33% CR/CRi | 11 (15) | Median 4 months | - | Median: Intensive chemo 10.2 months HMA 9 months AHSCT 19.4 months |
Tam et al. Blood 2008 N = 74 Median age 64 years | Post-PMF (N = 36) Post-ET/PV (N = 32) Post-MPN-U (N = 6) | JAK2 (43%) | Abnormal (72%) Adverse (48%) | Intensive chemo 41 (55) Less intensive chemo 12 (16) | Intensive chemo 46% CR/CRi Less intensive chemo 0% CR/CRi | 11 (15) | Median 5 months | PS Splenectomy Karyotype | Median: Intensive chemo without AHSCT 6 months Less intensive chemo without AHSCT 7 months CR/CRi Without AHSCT 13 months |
Patel et al. Blood 2022 N = 80 Median age 69 years AP-MPN, N = 16 BP-MPN, N = 64 | Post-PMF (N = 16) Post-ET/PV (N = 33) Post-MPN-U (N = 21) | JAK2 (66%) CALR (14%) MPL (10%) | - | Intensive chemo 32 (40) HMA 16 (20) HMA+venetoclax 23 (29) IDH inhibitor 3 (4) | Intensive chemo 28% CR/CRi HMA 19% CR/CRi HMA+venetoclax 30% CR/CRi IDH inhibitor 33% CR/CRi | 21 (26) | Median 8.8 months | - | Median: Intensive chemo 7 months HMA+ venetoclax 7.2 months HMA 13.2 months AHSCT 16 months |
Lancman et al. Leuk Res 2018 N = 57 Median age 68 years | Post-PMF (N = 13) Post-ET/PV (N = 39) | JAK2 (64%) | Adverse (63%) | Intensive chemo 12 (21) HMA 27 (47) Other agents 6 (11) | Intensive chemo 67% CR/CRi HMA 15% CR/CRi | 19 (33) | Median 5.8 months 2-year 28% | Transfusions | Median: Intensive chemo Not reached HMA 6.7 months AHSCT Not reached |