A MULTICENTER EVALUATION OF THE REVISED 2015 WHO MINOR CRITERIA FOR PREFIBROTIC PRIMARY MYELOFIBROSIS
(Abstract release date: 05/19/16)
EHA Library. Jeryczynski G. 06/09/16; 132890; E1341
Dr. Georg Jeryczynski
Contributions
Contributions
Abstract
Abstract: E1341
Type: Eposter Presentation
Background
The gold standard for the diagnosis of prefibrotic primary myelofibrosis (prePMF) remains the histomorphologic assessment of bone marrow (BM) biopsy samples. Clinically, especially early cases are difficult to distinguish from other myeloproliferative neoplasm (MPN), in particular essential thrombocythemia (ET). Most prominently, thrombocyte counts are not different in those two entities. However, several clinical features are frequently encountered in prePMF, that are not routinely found in ET. The revised 2015 WHO diagnostic criteria recognize four of these features as minor criteria for prePMF, at least one of which must be present to establish the diagnosis: anemia, leukocyte counts ≥ 11 G/L, elevated lactate dehydrogenase levels (LDH) as well as palpable splenomegaly.
Aims
The aim was to evaluate the new WHO minor criteria for prefibrotic primary myelofibrosis on a cohort of histomorphologically characterized patients.
Methods
From a multicenter database, 161 cases of prePMF in which the diagnosis had been established through BM biopsy, were selected. The presence of the minor criteria was then assessed at diagnosis. The results were compared to 186 patients diagnosed with ET from the same database. A fiber grade of 1 was present In 54 of prePMF patients, while no ET patients showed increased fibrosis in the BM.
Results
One or more minor criteria could be found in 90.7% (146/161) patients with prePMF, while this was true for only 52.7% (98/186) of ET cases. In both entities elevated LDH levels were the most prevalent feature (77.0% vs. 29.0%, p<0.001), followed by high white blood cell counts. There were no significant differences between patients with fiber grade 0 or 1, except in splenomegaly (37.4% vs. 42.9%, p=0.030).
The number of minor criteria increased with age. While prePMF patients with one criterion were aged 60 years, patients that displayed all four symptoms were aged 74 years. Accordingly, overall survival was shorter in patients with a higher disease burden.
Conclusion
The revised minor criteria for prePMF could be well reproduced in this population. In combination with the histomorphologic assessment, they are suitable to correctly establish the diagnosis of prePMF. However, there is a small subgroup of prePMF patients that show no minor criteria at time of diagnosis. These patients should be monitored closely and their diagnosis should be re-evaluated if they should present with one of the features. The proportion of ET patients that also display one or more minor clinical symptoms underlines the central role of histomorphologic differentiation of MPN patients.
Session topic: E-poster
Keyword(s): Diagnosis, Myelofibrosis, Thrombocythemia
Type: Eposter Presentation
Background
The gold standard for the diagnosis of prefibrotic primary myelofibrosis (prePMF) remains the histomorphologic assessment of bone marrow (BM) biopsy samples. Clinically, especially early cases are difficult to distinguish from other myeloproliferative neoplasm (MPN), in particular essential thrombocythemia (ET). Most prominently, thrombocyte counts are not different in those two entities. However, several clinical features are frequently encountered in prePMF, that are not routinely found in ET. The revised 2015 WHO diagnostic criteria recognize four of these features as minor criteria for prePMF, at least one of which must be present to establish the diagnosis: anemia, leukocyte counts ≥ 11 G/L, elevated lactate dehydrogenase levels (LDH) as well as palpable splenomegaly.
Aims
The aim was to evaluate the new WHO minor criteria for prefibrotic primary myelofibrosis on a cohort of histomorphologically characterized patients.
Methods
From a multicenter database, 161 cases of prePMF in which the diagnosis had been established through BM biopsy, were selected. The presence of the minor criteria was then assessed at diagnosis. The results were compared to 186 patients diagnosed with ET from the same database. A fiber grade of 1 was present In 54 of prePMF patients, while no ET patients showed increased fibrosis in the BM.
Results
One or more minor criteria could be found in 90.7% (146/161) patients with prePMF, while this was true for only 52.7% (98/186) of ET cases. In both entities elevated LDH levels were the most prevalent feature (77.0% vs. 29.0%, p<0.001), followed by high white blood cell counts. There were no significant differences between patients with fiber grade 0 or 1, except in splenomegaly (37.4% vs. 42.9%, p=0.030).
| Parameter | prePMF | ET | p-value | ||
| % | n | % | n | ||
| Anemia (Hemoglobin m/f ≤ 13/12 g/dl) | 26.7 | 43 | 8.6 | 16 | <0.001 |
| Splenomegaly (palpable or increased length diameter by imaging analysis ≥13 cm) | 43.5 | 70 | 11.3 | 21 | <0.001 |
| LDH (above upper institutional limit) | 77.0 | 124 | 29.0 | 54 | <0.001 |
| WBC ≥ 11 x 109/L | 47.2 | 76 | 25.8 | 48 | <0.001 |
Conclusion
The revised minor criteria for prePMF could be well reproduced in this population. In combination with the histomorphologic assessment, they are suitable to correctly establish the diagnosis of prePMF. However, there is a small subgroup of prePMF patients that show no minor criteria at time of diagnosis. These patients should be monitored closely and their diagnosis should be re-evaluated if they should present with one of the features. The proportion of ET patients that also display one or more minor clinical symptoms underlines the central role of histomorphologic differentiation of MPN patients.
Session topic: E-poster
Keyword(s): Diagnosis, Myelofibrosis, Thrombocythemia
Abstract: E1341
Type: Eposter Presentation
Background
The gold standard for the diagnosis of prefibrotic primary myelofibrosis (prePMF) remains the histomorphologic assessment of bone marrow (BM) biopsy samples. Clinically, especially early cases are difficult to distinguish from other myeloproliferative neoplasm (MPN), in particular essential thrombocythemia (ET). Most prominently, thrombocyte counts are not different in those two entities. However, several clinical features are frequently encountered in prePMF, that are not routinely found in ET. The revised 2015 WHO diagnostic criteria recognize four of these features as minor criteria for prePMF, at least one of which must be present to establish the diagnosis: anemia, leukocyte counts ≥ 11 G/L, elevated lactate dehydrogenase levels (LDH) as well as palpable splenomegaly.
Aims
The aim was to evaluate the new WHO minor criteria for prefibrotic primary myelofibrosis on a cohort of histomorphologically characterized patients.
Methods
From a multicenter database, 161 cases of prePMF in which the diagnosis had been established through BM biopsy, were selected. The presence of the minor criteria was then assessed at diagnosis. The results were compared to 186 patients diagnosed with ET from the same database. A fiber grade of 1 was present In 54 of prePMF patients, while no ET patients showed increased fibrosis in the BM.
Results
One or more minor criteria could be found in 90.7% (146/161) patients with prePMF, while this was true for only 52.7% (98/186) of ET cases. In both entities elevated LDH levels were the most prevalent feature (77.0% vs. 29.0%, p<0.001), followed by high white blood cell counts. There were no significant differences between patients with fiber grade 0 or 1, except in splenomegaly (37.4% vs. 42.9%, p=0.030).
The number of minor criteria increased with age. While prePMF patients with one criterion were aged 60 years, patients that displayed all four symptoms were aged 74 years. Accordingly, overall survival was shorter in patients with a higher disease burden.
Conclusion
The revised minor criteria for prePMF could be well reproduced in this population. In combination with the histomorphologic assessment, they are suitable to correctly establish the diagnosis of prePMF. However, there is a small subgroup of prePMF patients that show no minor criteria at time of diagnosis. These patients should be monitored closely and their diagnosis should be re-evaluated if they should present with one of the features. The proportion of ET patients that also display one or more minor clinical symptoms underlines the central role of histomorphologic differentiation of MPN patients.
Session topic: E-poster
Keyword(s): Diagnosis, Myelofibrosis, Thrombocythemia
Type: Eposter Presentation
Background
The gold standard for the diagnosis of prefibrotic primary myelofibrosis (prePMF) remains the histomorphologic assessment of bone marrow (BM) biopsy samples. Clinically, especially early cases are difficult to distinguish from other myeloproliferative neoplasm (MPN), in particular essential thrombocythemia (ET). Most prominently, thrombocyte counts are not different in those two entities. However, several clinical features are frequently encountered in prePMF, that are not routinely found in ET. The revised 2015 WHO diagnostic criteria recognize four of these features as minor criteria for prePMF, at least one of which must be present to establish the diagnosis: anemia, leukocyte counts ≥ 11 G/L, elevated lactate dehydrogenase levels (LDH) as well as palpable splenomegaly.
Aims
The aim was to evaluate the new WHO minor criteria for prefibrotic primary myelofibrosis on a cohort of histomorphologically characterized patients.
Methods
From a multicenter database, 161 cases of prePMF in which the diagnosis had been established through BM biopsy, were selected. The presence of the minor criteria was then assessed at diagnosis. The results were compared to 186 patients diagnosed with ET from the same database. A fiber grade of 1 was present In 54 of prePMF patients, while no ET patients showed increased fibrosis in the BM.
Results
One or more minor criteria could be found in 90.7% (146/161) patients with prePMF, while this was true for only 52.7% (98/186) of ET cases. In both entities elevated LDH levels were the most prevalent feature (77.0% vs. 29.0%, p<0.001), followed by high white blood cell counts. There were no significant differences between patients with fiber grade 0 or 1, except in splenomegaly (37.4% vs. 42.9%, p=0.030).
| Parameter | prePMF | ET | p-value | ||
| % | n | % | n | ||
| Anemia (Hemoglobin m/f ≤ 13/12 g/dl) | 26.7 | 43 | 8.6 | 16 | <0.001 |
| Splenomegaly (palpable or increased length diameter by imaging analysis ≥13 cm) | 43.5 | 70 | 11.3 | 21 | <0.001 |
| LDH (above upper institutional limit) | 77.0 | 124 | 29.0 | 54 | <0.001 |
| WBC ≥ 11 x 109/L | 47.2 | 76 | 25.8 | 48 | <0.001 |
Conclusion
The revised minor criteria for prePMF could be well reproduced in this population. In combination with the histomorphologic assessment, they are suitable to correctly establish the diagnosis of prePMF. However, there is a small subgroup of prePMF patients that show no minor criteria at time of diagnosis. These patients should be monitored closely and their diagnosis should be re-evaluated if they should present with one of the features. The proportion of ET patients that also display one or more minor clinical symptoms underlines the central role of histomorphologic differentiation of MPN patients.
Session topic: E-poster
Keyword(s): Diagnosis, Myelofibrosis, Thrombocythemia
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