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Pre‐fibrotic/early primary myelofibrosis vs. WHO‐defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease
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| Zeitschriftentitel: | American Journal of Hematology |
|---|---|
| Personen und Körperschaften: | , , , , , , , , , , , , , , , , , |
| In: | American Journal of Hematology, 92, 2017, 9, S. 885-891 |
| Format: | E-Article |
| Sprache: | Englisch |
| veröffentlicht: |
Wiley
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| Schlagwörter: |
| author_facet |
Jeryczynski, Georg Thiele, Jürgen Gisslinger, Bettina Wölfler, Albert Schalling, Martin Gleiß, Andreas Burgstaller, Sonja Buxhofer‐Ausch, Veronika Sliwa, Thamer Schlögl, Ernst Geissler, Klaus Krauth, Maria‐Theresa Nader, Alexander Vesely, Michael Simonitsch‐Klupp, Ingrid Müllauer, Leonhard Beham‐Schmid, Christine Gisslinger, Heinz Jeryczynski, Georg Thiele, Jürgen Gisslinger, Bettina Wölfler, Albert Schalling, Martin Gleiß, Andreas Burgstaller, Sonja Buxhofer‐Ausch, Veronika Sliwa, Thamer Schlögl, Ernst Geissler, Klaus Krauth, Maria‐Theresa Nader, Alexander Vesely, Michael Simonitsch‐Klupp, Ingrid Müllauer, Leonhard Beham‐Schmid, Christine Gisslinger, Heinz |
|---|---|
| author |
Jeryczynski, Georg Thiele, Jürgen Gisslinger, Bettina Wölfler, Albert Schalling, Martin Gleiß, Andreas Burgstaller, Sonja Buxhofer‐Ausch, Veronika Sliwa, Thamer Schlögl, Ernst Geissler, Klaus Krauth, Maria‐Theresa Nader, Alexander Vesely, Michael Simonitsch‐Klupp, Ingrid Müllauer, Leonhard Beham‐Schmid, Christine Gisslinger, Heinz |
| spellingShingle |
Jeryczynski, Georg Thiele, Jürgen Gisslinger, Bettina Wölfler, Albert Schalling, Martin Gleiß, Andreas Burgstaller, Sonja Buxhofer‐Ausch, Veronika Sliwa, Thamer Schlögl, Ernst Geissler, Klaus Krauth, Maria‐Theresa Nader, Alexander Vesely, Michael Simonitsch‐Klupp, Ingrid Müllauer, Leonhard Beham‐Schmid, Christine Gisslinger, Heinz American Journal of Hematology Pre‐fibrotic/early primary myelofibrosis vs. WHO‐defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease Hematology |
| author_sort |
jeryczynski, georg |
| spelling |
Jeryczynski, Georg Thiele, Jürgen Gisslinger, Bettina Wölfler, Albert Schalling, Martin Gleiß, Andreas Burgstaller, Sonja Buxhofer‐Ausch, Veronika Sliwa, Thamer Schlögl, Ernst Geissler, Klaus Krauth, Maria‐Theresa Nader, Alexander Vesely, Michael Simonitsch‐Klupp, Ingrid Müllauer, Leonhard Beham‐Schmid, Christine Gisslinger, Heinz 0361-8609 1096-8652 Wiley Hematology http://dx.doi.org/10.1002/ajh.24788 <jats:title>Abstract</jats:title><jats:p>The 2016 revised WHO criteria for the diagnosis of pre‐fibrotic/early primary myelofibrosis (pre‐PMF) require at least one of the following four borderline expressed minor clinical criteria: anemia, leukocytosis, elevated lactate dehydrogenase and splenomegaly. In this study, we evaluated the relative frequency of these four criteria in a group of 170 pre‐PMF patients and compared them to 225 ET cases. More than 91% of pre‐PMF cases showed one or more of these features required for diagnosis, by contrast with only 48% of ET patients. According to clinical data the cumulative risk of progression to advanced/overt PMF in pre‐PMF was 36.9% after 15 years. After fitting cox regression models to analyze the impact of the minor criteria on overall survival, only leukocytosis remained as a significant predictor of survival in both pre‐PMF and ET. Molecular characterization showed differences in survival in pre‐PMF but not ET, with <jats:italic>CALR</jats:italic> being a more favorable mutation than <jats:italic>JAK2</jats:italic>. The different outcome of pre‐PMF versus ET and associated molecular genetic data supports the concept of two different entities, rather than a continuum of the same disease. Although slightly less than 50% of ET patients also show one or more minor clinical criteria, accurate distinction between ET and pre‐PMF is possible by following an integrated approach including histomorphological diagnosis and presence of minor clinical criteria.</jats:p> Pre‐fibrotic/early primary myelofibrosis vs. WHO‐defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease American Journal of Hematology |
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| title |
Pre‐fibrotic/early primary myelofibrosis vs. WHO‐defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease |
| title_unstemmed |
Pre‐fibrotic/early primary myelofibrosis vs. WHO‐defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease |
| title_full |
Pre‐fibrotic/early primary myelofibrosis vs. WHO‐defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease |
| title_fullStr |
Pre‐fibrotic/early primary myelofibrosis vs. WHO‐defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease |
| title_full_unstemmed |
Pre‐fibrotic/early primary myelofibrosis vs. WHO‐defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease |
| title_short |
Pre‐fibrotic/early primary myelofibrosis vs. WHO‐defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease |
| title_sort |
pre‐fibrotic/early primary myelofibrosis vs. who‐defined essential thrombocythemia: the impact of minor clinical diagnostic criteria on the outcome of the disease |
| topic |
Hematology |
| url |
http://dx.doi.org/10.1002/ajh.24788 |
| publishDate |
2017 |
| physical |
885-891 |
| description |
<jats:title>Abstract</jats:title><jats:p>The 2016 revised WHO criteria for the diagnosis of pre‐fibrotic/early primary myelofibrosis (pre‐PMF) require at least one of the following four borderline expressed minor clinical criteria: anemia, leukocytosis, elevated lactate dehydrogenase and splenomegaly. In this study, we evaluated the relative frequency of these four criteria in a group of 170 pre‐PMF patients and compared them to 225 ET cases. More than 91% of pre‐PMF cases showed one or more of these features required for diagnosis, by contrast with only 48% of ET patients. According to clinical data the cumulative risk of progression to advanced/overt PMF in pre‐PMF was 36.9% after 15 years. After fitting cox regression models to analyze the impact of the minor criteria on overall survival, only leukocytosis remained as a significant predictor of survival in both pre‐PMF and ET. Molecular characterization showed differences in survival in pre‐PMF but not ET, with <jats:italic>CALR</jats:italic> being a more favorable mutation than <jats:italic>JAK2</jats:italic>. The different outcome of pre‐PMF versus ET and associated molecular genetic data supports the concept of two different entities, rather than a continuum of the same disease. Although slightly less than 50% of ET patients also show one or more minor clinical criteria, accurate distinction between ET and pre‐PMF is possible by following an integrated approach including histomorphological diagnosis and presence of minor clinical criteria.</jats:p> |
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| description | <jats:title>Abstract</jats:title><jats:p>The 2016 revised WHO criteria for the diagnosis of pre‐fibrotic/early primary myelofibrosis (pre‐PMF) require at least one of the following four borderline expressed minor clinical criteria: anemia, leukocytosis, elevated lactate dehydrogenase and splenomegaly. In this study, we evaluated the relative frequency of these four criteria in a group of 170 pre‐PMF patients and compared them to 225 ET cases. More than 91% of pre‐PMF cases showed one or more of these features required for diagnosis, by contrast with only 48% of ET patients. According to clinical data the cumulative risk of progression to advanced/overt PMF in pre‐PMF was 36.9% after 15 years. After fitting cox regression models to analyze the impact of the minor criteria on overall survival, only leukocytosis remained as a significant predictor of survival in both pre‐PMF and ET. Molecular characterization showed differences in survival in pre‐PMF but not ET, with <jats:italic>CALR</jats:italic> being a more favorable mutation than <jats:italic>JAK2</jats:italic>. The different outcome of pre‐PMF versus ET and associated molecular genetic data supports the concept of two different entities, rather than a continuum of the same disease. Although slightly less than 50% of ET patients also show one or more minor clinical criteria, accurate distinction between ET and pre‐PMF is possible by following an integrated approach including histomorphological diagnosis and presence of minor clinical criteria.</jats:p> |
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| spelling | Jeryczynski, Georg Thiele, Jürgen Gisslinger, Bettina Wölfler, Albert Schalling, Martin Gleiß, Andreas Burgstaller, Sonja Buxhofer‐Ausch, Veronika Sliwa, Thamer Schlögl, Ernst Geissler, Klaus Krauth, Maria‐Theresa Nader, Alexander Vesely, Michael Simonitsch‐Klupp, Ingrid Müllauer, Leonhard Beham‐Schmid, Christine Gisslinger, Heinz 0361-8609 1096-8652 Wiley Hematology http://dx.doi.org/10.1002/ajh.24788 <jats:title>Abstract</jats:title><jats:p>The 2016 revised WHO criteria for the diagnosis of pre‐fibrotic/early primary myelofibrosis (pre‐PMF) require at least one of the following four borderline expressed minor clinical criteria: anemia, leukocytosis, elevated lactate dehydrogenase and splenomegaly. In this study, we evaluated the relative frequency of these four criteria in a group of 170 pre‐PMF patients and compared them to 225 ET cases. More than 91% of pre‐PMF cases showed one or more of these features required for diagnosis, by contrast with only 48% of ET patients. According to clinical data the cumulative risk of progression to advanced/overt PMF in pre‐PMF was 36.9% after 15 years. After fitting cox regression models to analyze the impact of the minor criteria on overall survival, only leukocytosis remained as a significant predictor of survival in both pre‐PMF and ET. Molecular characterization showed differences in survival in pre‐PMF but not ET, with <jats:italic>CALR</jats:italic> being a more favorable mutation than <jats:italic>JAK2</jats:italic>. The different outcome of pre‐PMF versus ET and associated molecular genetic data supports the concept of two different entities, rather than a continuum of the same disease. Although slightly less than 50% of ET patients also show one or more minor clinical criteria, accurate distinction between ET and pre‐PMF is possible by following an integrated approach including histomorphological diagnosis and presence of minor clinical criteria.</jats:p> Pre‐fibrotic/early primary myelofibrosis vs. WHO‐defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease American Journal of Hematology |
| spellingShingle | Jeryczynski, Georg, Thiele, Jürgen, Gisslinger, Bettina, Wölfler, Albert, Schalling, Martin, Gleiß, Andreas, Burgstaller, Sonja, Buxhofer‐Ausch, Veronika, Sliwa, Thamer, Schlögl, Ernst, Geissler, Klaus, Krauth, Maria‐Theresa, Nader, Alexander, Vesely, Michael, Simonitsch‐Klupp, Ingrid, Müllauer, Leonhard, Beham‐Schmid, Christine, Gisslinger, Heinz, American Journal of Hematology, Pre‐fibrotic/early primary myelofibrosis vs. WHO‐defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease, Hematology |
| title | Pre‐fibrotic/early primary myelofibrosis vs. WHO‐defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease |
| title_full | Pre‐fibrotic/early primary myelofibrosis vs. WHO‐defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease |
| title_fullStr | Pre‐fibrotic/early primary myelofibrosis vs. WHO‐defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease |
| title_full_unstemmed | Pre‐fibrotic/early primary myelofibrosis vs. WHO‐defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease |
| title_short | Pre‐fibrotic/early primary myelofibrosis vs. WHO‐defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease |
| title_sort | pre‐fibrotic/early primary myelofibrosis vs. who‐defined essential thrombocythemia: the impact of minor clinical diagnostic criteria on the outcome of the disease |
| title_unstemmed | Pre‐fibrotic/early primary myelofibrosis vs. WHO‐defined essential thrombocythemia: The impact of minor clinical diagnostic criteria on the outcome of the disease |
| topic | Hematology |
| url | http://dx.doi.org/10.1002/ajh.24788 |