Thrombotic events are major complications in patients (pts) affected by Essential Thrombocytemia (ET) and Polycytemia Vera (PV). To compare thrombotic risk in these 2 groups, we evaluated retrospectively our database of 1249 ET and 623 PV pts diagnosed and followed in 11 hematological centers in the Latium region between 1/1980 and 12/2010: the diagnosis was done according to PVSG, WHO 2001 and 2008criteria based on the time of first observation. Baseline features of ET pts: 797F/452M,median age 62.9 yrs (range 19-96),median WBC count 8.8 x 109/L (range 1.2-57.7), median PLT count 812 x 109/L (range 457-3582), median Hb level 14.0 g/dl (range 6-20.5), JAK-2V617F positivity 59.7% with a median allele burden of 19,6% (range 0.2- 99.9), spleen enlargement in 18.7% of pts, previous thrombosis223/1239 evaluable pts (17.9%) [arterial 176/223 (14.1%), venous 47/223 (3.8%)]. Baseline features of PV pts: 289F/334M, median age 63.0yrs (range 21-91), median WBC count 10.1 x 109/L (range 3.5-37.6), median PLT count 457 x 109/L (range 169-1790), median Hb level 18.2 g/dl (range 10.5-24.8), JAK-2V617F positivity 94.3% with a median allele burden of 59.1% (range 0.3-99.9), spleen enlargement in 42% of patients, previous thrombosis 146/617 evaluable pts (23.7%)[arterial 114/617 (18.5%), venous 32/617 (5,2%)].in the ET cohort, after a median follow-up of 7.7 yrs, thrombotic complications were seen in 107/1141 evaluable pts (9.4%) [arterial60 (5.25%), venous 47 (4.11%)]; in the PV cohort, after a median follow-up of 8.5 yrs, thrombotic complications were seen in 107/623pts (17.2%) [arterial 67 (10.8%),venous 40 (6.4%)].All common risk factors for thrombosis were evaluated in multivariate analysis, searching the cut-off number for continuous variables with ROC curves. The significant variables at multivariate analysis for ET and PV pts are shown in the table; age, previous thromboses and spleen enlargement were risk factors in ET pts, while previous thromboses and JAK-2V617F allele burden were risk factors in PV pts. PLT count above ROC value seemed to be a protective factor in both cohorts. In conclusion, in contrast with the tendency to evaluate in a similar manner the thrombotic risk of PV and ET, data from our retrospective database showed that these 2 groups should be considered populations with different risk factors for thrombosis.

Table 1.
Putative prognostic factorsPolycythemia VeraEssential Thrombocythemia
HR95% C.I.pHR95% C.I .p
Previous thromboses 2,31 1,13 - 4,74 0,02 1,87 1,08 -3,23 0,026 
Age ≥ 60 y 1,54 0,79 - 2,99 0,21 1,90 1,18 - 3,06 0,009 
JAK2V617F PV: allelic burden ≥ 81%
ET: pos 
1,95 1,03 - 3,71 0,04 0,76 0,48 - 1,21 0,25 
Plt count PV ≥ 452.109/L
ET ≥ 944.109/L 
0,49 0,25 - 0,95 0,04 0,52 0,31 - 0,89 0,017 
Spleen enlargement 0,67 0,34 -1,31 0,24 1,71 1,02 - 2,89 0,04 
CV risk factors (at least 1) 0,92 0,41 - 2,03 0,83 0,87 0,51 - 1,49 0,62 
WBC PV ≥ 10,175.109/L
ET ≥ 9,630.109/L 
1,09 0,57 - 2,08 0,80 1,41 0,89 -2,26 0,15 
Putative prognostic factorsPolycythemia VeraEssential Thrombocythemia
HR95% C.I.pHR95% C.I .p
Previous thromboses 2,31 1,13 - 4,74 0,02 1,87 1,08 -3,23 0,026 
Age ≥ 60 y 1,54 0,79 - 2,99 0,21 1,90 1,18 - 3,06 0,009 
JAK2V617F PV: allelic burden ≥ 81%
ET: pos 
1,95 1,03 - 3,71 0,04 0,76 0,48 - 1,21 0,25 
Plt count PV ≥ 452.109/L
ET ≥ 944.109/L 
0,49 0,25 - 0,95 0,04 0,52 0,31 - 0,89 0,017 
Spleen enlargement 0,67 0,34 -1,31 0,24 1,71 1,02 - 2,89 0,04 
CV risk factors (at least 1) 0,92 0,41 - 2,03 0,83 0,87 0,51 - 1,49 0,62 
WBC PV ≥ 10,175.109/L
ET ≥ 9,630.109/L 
1,09 0,57 - 2,08 0,80 1,41 0,89 -2,26 0,15 

Disclosures

No relevant conflicts of interest to declare.

Author notes

*

Asterisk with author names denotes non-ASH members.

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