Abstract 5065

The Latium is a region of central Italy that counts approximatively 5.600.000 of residents. We reported herein a preliminary analysis of 1572 patients affected by MPN diagnosed in the last 20 years and treated in 9 of the Centers belonging to our group. Our centers are mainly located in Rome (7 centers) and neighboring districts (2 centers, Latina and Viterbo). The diagnosis was performed according to PVSG criteria until 2001 and then according to the WHO criteria: the majority of the patients underwent bone marrow biopsy. 218 patients were affected by primary myelofibrosis (PMF), 779 by Essential thrombocytemia (ET) and 575 by Policytemia Vera (PV). Epidemiological and clinical findings of all patients at diagnosis as well as thrombotic complications and evolution are reported in the table:

ET (779 pts)PV (575 pts)PMF (218 pts)
M/F 288/491 350/225 134/84 
Age (yrs) 59 (r: 20-93) 60 (r: 19-91) 67 (r: 30-86) 
WBC (x109/L) 9192 (+/− 3483 SD) 10423 (+/− SD 4307) 13313 (+/−5220) 
Hb (g/dL) 13, 9 (+/− 1,8) 18,1 (+/− 2,3) 11,7 (+/− 2,8) 
Plts (x 109/L) 877 (+/− 353) 445 (+/−247) 457 (+/− 358) 
Splenomegaly (%) 19 35 79 
Bone marrow biopsy (performed/total)) 557/779 (71,5%) 254/575 (44,2%) 207/218 (95%) 
JAK-2 V617F mut (%) 284/484 (59%) 256/369 (69,4%) 69/116 (59,5 %) 
Jak-2 allele burden Performed in 188/284 cases 26,49% (SD +/− 24) Performed in 199/256 cases: 59,4 % (SD +/−30,9) Performed in 51/69 cases 57,5% (SD +/− 29,4) 
Median Follow-up (years) 7,15 7,86 3,89 
Thrombosis pre-diagnosis of MPN N. of patients (%) 108/779 (13,8%) 100/575 (17,4%) 29/218 (13,3%) 
 (arterial+venous) 74+34 76+24 24+5 
Thrombosis post diagnosis of MPN N. of patients (%) 71/779 (9,1%) 66/575 (11,5%) 20/218(9,17%) 
 (arterial+venous) 42+29 42+24 12+8 
Evolution Myelofibrosis 9/779 (1,1%) 24/575 (4,1%)  
 AML 9/779 (1,1%) 13/575 (2,3%) 17/143 (11,8%) 
ET (779 pts)PV (575 pts)PMF (218 pts)
M/F 288/491 350/225 134/84 
Age (yrs) 59 (r: 20-93) 60 (r: 19-91) 67 (r: 30-86) 
WBC (x109/L) 9192 (+/− 3483 SD) 10423 (+/− SD 4307) 13313 (+/−5220) 
Hb (g/dL) 13, 9 (+/− 1,8) 18,1 (+/− 2,3) 11,7 (+/− 2,8) 
Plts (x 109/L) 877 (+/− 353) 445 (+/−247) 457 (+/− 358) 
Splenomegaly (%) 19 35 79 
Bone marrow biopsy (performed/total)) 557/779 (71,5%) 254/575 (44,2%) 207/218 (95%) 
JAK-2 V617F mut (%) 284/484 (59%) 256/369 (69,4%) 69/116 (59,5 %) 
Jak-2 allele burden Performed in 188/284 cases 26,49% (SD +/− 24) Performed in 199/256 cases: 59,4 % (SD +/−30,9) Performed in 51/69 cases 57,5% (SD +/− 29,4) 
Median Follow-up (years) 7,15 7,86 3,89 
Thrombosis pre-diagnosis of MPN N. of patients (%) 108/779 (13,8%) 100/575 (17,4%) 29/218 (13,3%) 
 (arterial+venous) 74+34 76+24 24+5 
Thrombosis post diagnosis of MPN N. of patients (%) 71/779 (9,1%) 66/575 (11,5%) 20/218(9,17%) 
 (arterial+venous) 42+29 42+24 12+8 
Evolution Myelofibrosis 9/779 (1,1%) 24/575 (4,1%)  
 AML 9/779 (1,1%) 13/575 (2,3%) 17/143 (11,8%) 
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Comments:

In our opinion, this casistic of patients with different types of MPNs reflects clinical presentation and evolution of three variants of the same disease. Many clinical findings in our unselected cohort of patients are similar to those reported in literature; in particular, thrombotic events were seen in about 13 – 17% of patients, without any correlation with Jak-2 status in both pre-diagnosis and follow-up. However, 2 main differences were noted: a lower incidence of JAK-2 V617F mutation among our PV patients and a lower rate of evolution in myelofibrosis and AML among our ET and PV patients. Both these features warrant further insights to be fully elucidated.

Disclosures:

No relevant conflicts of interest to declare.

Topics:
bone marrow biopsy, brachial plexus neuritis, epidemiology, follow-up, myelofibrosis, myelofibrosis, idiopathic, chronic, neoplasms, splenomegaly, thrombocythemia, hemorrhagic, thrombosis

Author notes

*

Asterisk with author names denotes non-ASH members.

© 2010 by The American Society of Hematology
2010
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