Hematology
Contributions
Type: Publication Only
Background
ET is not such an innocent disease in young adults. Most thrombotic events occur during diagnosis and almost half of the initially asymptomatic patients will have an event during the course of their disease
Aims
Herein, we analyzed retrospectively the medical records of 33 ET patients diagnosed below the age of 45 years and followed in a single institution from 1992 to 2014.
Methods
Management was left at the discretion of the treating physician but antithrombotic agents were used from disease diagnosis in all patients. Among 33 young adult patients 11 were males and 22 females. 18 patients were followed for a median period of 94,5 (range 45-178) months and left untreated and they were included in group A. While cytoreductive treatment was introduced in 15 patients included in group B. Among 15 treated patients cytoreduction was introduced from disease diagnosis in seven patients and in eight during the course of their disease after a median period on observation of 36 months (ranging from 6 to 168).
Results
We separated our patients into two groups according to the need to start any cytoreductive treatment and try to discover patient’s characteristics at diagnosis that can predict the early use of cytoreductive treatment. Splenomegaly of any grade was palpated in 3/18 (16.5%) patients in group A and in 2/15(13%) in group B, p=0,796. Although hematocrit levels were equal between the two groups [42,7%(34,7-47,6) in group A and 40,7%(37,3-46) in group B, p=0,84] as were also and the leucocyte counts [ 7,85 (5,6-13,2) X 103 pcm in group A and 9,73 (4,7-11,7) X 103 in group B p=0,6], the median values of platelet counts at diagnosis was significantly higher in group B compared to group A [805(490-2300) X 109 pcm vs 604,5(490-921) X 109 pcm, p=0,012]. Fibrosis (grade I) at the initially performed bone marrow biopsy was met at higher percentages in patients allocated in group B 40% (6/15) in group B and11% (2/18) at group A, p=0,03. JAK2V617F was detected in 40% (6/15) in group B and 50% (9/18) in group A, p=1. According to the IPSET-thrombosis scoring system in group A most patients had low thrombotic risk 10/18, 7/18 had intermediate thrombotic risk and 1/18 had high thrombotic risk. In group B 7/15 had low thrombotic risk, 3/15 intermediate and 5/15 had high thrombotic risk.
In the total cohort of 33 young ET patients platelet counts at disease diagnosis can predict reduced Event free Survival (EFS). As an event were considered thrombohemorhagic complications, development of myelofibrosis and the need to start cytoreductive treatment. 10 year EFS was for patients with platelet counts < 600 x 109 pcm 89% for platelet counts between 600-800 X 109 pcm 66% and for platelet counts> 800 X 109 pcm was 22%, p=0.003, Kaplan-Mayer, LogRANK test. On the contrary the presence of grade-I fibrosis was not associated with reduced EFS.
Summary
In young ET patients increased platelet counts at disease diagnosis can predict an eventful disease journey with early need for the use of cytoreductive treatment and reduced EFS.
Keyword(s): Essential Thrombocytemia, Young adult
Session topic: Publication Only
Type: Publication Only
Background
ET is not such an innocent disease in young adults. Most thrombotic events occur during diagnosis and almost half of the initially asymptomatic patients will have an event during the course of their disease
Aims
Herein, we analyzed retrospectively the medical records of 33 ET patients diagnosed below the age of 45 years and followed in a single institution from 1992 to 2014.
Methods
Management was left at the discretion of the treating physician but antithrombotic agents were used from disease diagnosis in all patients. Among 33 young adult patients 11 were males and 22 females. 18 patients were followed for a median period of 94,5 (range 45-178) months and left untreated and they were included in group A. While cytoreductive treatment was introduced in 15 patients included in group B. Among 15 treated patients cytoreduction was introduced from disease diagnosis in seven patients and in eight during the course of their disease after a median period on observation of 36 months (ranging from 6 to 168).
Results
We separated our patients into two groups according to the need to start any cytoreductive treatment and try to discover patient’s characteristics at diagnosis that can predict the early use of cytoreductive treatment. Splenomegaly of any grade was palpated in 3/18 (16.5%) patients in group A and in 2/15(13%) in group B, p=0,796. Although hematocrit levels were equal between the two groups [42,7%(34,7-47,6) in group A and 40,7%(37,3-46) in group B, p=0,84] as were also and the leucocyte counts [ 7,85 (5,6-13,2) X 103 pcm in group A and 9,73 (4,7-11,7) X 103 in group B p=0,6], the median values of platelet counts at diagnosis was significantly higher in group B compared to group A [805(490-2300) X 109 pcm vs 604,5(490-921) X 109 pcm, p=0,012]. Fibrosis (grade I) at the initially performed bone marrow biopsy was met at higher percentages in patients allocated in group B 40% (6/15) in group B and11% (2/18) at group A, p=0,03. JAK2V617F was detected in 40% (6/15) in group B and 50% (9/18) in group A, p=1. According to the IPSET-thrombosis scoring system in group A most patients had low thrombotic risk 10/18, 7/18 had intermediate thrombotic risk and 1/18 had high thrombotic risk. In group B 7/15 had low thrombotic risk, 3/15 intermediate and 5/15 had high thrombotic risk.
In the total cohort of 33 young ET patients platelet counts at disease diagnosis can predict reduced Event free Survival (EFS). As an event were considered thrombohemorhagic complications, development of myelofibrosis and the need to start cytoreductive treatment. 10 year EFS was for patients with platelet counts < 600 x 109 pcm 89% for platelet counts between 600-800 X 109 pcm 66% and for platelet counts> 800 X 109 pcm was 22%, p=0.003, Kaplan-Mayer, LogRANK test. On the contrary the presence of grade-I fibrosis was not associated with reduced EFS.
Summary
In young ET patients increased platelet counts at disease diagnosis can predict an eventful disease journey with early need for the use of cytoreductive treatment and reduced EFS.
Keyword(s): Essential Thrombocytemia, Young adult
Session topic: Publication Only