Contributions
Abstract: EP1143
Type: E-Poster Presentation
Session title: Platelet disorders
Background
The incidence of immune thrombocytopenia (ITP) is around 3/100,000 person-years. ITP is associated to another disease in 15-20% of the cases, mostly systemic auto-immune diseases, or hematological malignancies. For the latter, the association is principally reported with chronic lymphoid leukemia (CLL), large granular leukemia (LGL) and Hodgkin lymphoma (HL). As data have been derived from cohorts from tertiary centers with a possible selection bias, the accurate incidence rate of lymphoid malignancies (LM)-related ITP remains elusive.
Aims
Determination of the frequency, clinical characteristics and evolution of LM-related ITP based on the prospective data collected in the register of hematological malignancies of Côte d’Or (RHEMCO), a population-based register recording all incident hematological malignancies occurring in a restricted geographical area of 532,886 inhabitants.
Methods
Patients included in RHEMCO between January 1995 and December 2015 (n=3499) were analyzed to calculate incidences. The medical records of ITP patients referred to our tertiary hospital during the same period were also considered for the clinical description and the evolution of LM-related ITP. ITP was defined as a thrombocytopenia <100 G/L after exclusion of other causes. Response was defined as a platelet count >30 G/L with at least a 2-fold increase of baseline level.
Results
Based on RHEMCO, the frequency of ITP during LM was 1.06% (n=37/3499): 3.46% for T cell lymphoma, 1.21% for CLL, 0.82% for B cell lymphoma and 0.36% for HL. The frequency of Evans’ syndrome (ES) was 0.29% (n=10/3499) in the whole cohort: 0.44% during CLL, 0.43% for T cell lymphoma, and 0.24% for B cell lymphoma.
From RHEMCO and medical records, 76 patients with secondary ITP were identified, among which 17 with ES. For ITP, the median age at diagnosis was 69.5 [interquartile range (IQR): 19-94] years and 59% were male. For ES, the median age was 62 [IQR: 29-85] and 76% were male. Among all LM-related ITP, CLL and indolent B cell lymphoma were the most represented (29% each), followed by T cell lymphoma (20%), aggressive B cell lymphoma (18.5%) and HL (3.5%). ES was mostly associated with indolent B cell lymphoma (47%) or CLL (41%), T cell lymphoma or HL representing only 6% each. LM occurred before ITP in 46% of the cases, with a median delay of 3 [IQR: 0.5-18.4] years and in 65% for ES with a delay of 5.1 [IQR: 0.3-10.1] years. LM and ITP were simultaneous (≤ 3 months) in 44% for ITP and 29% for ES. Bleedings were reported in 45% and splenomegaly in 29% of the cases. The median platelet count at diagnosis was 30 [IQR: 1-96] G/L.
All the patients were treated: 84% received specific medications for ITP and 91.5% for LM. Responses to ITP treatments were 63% for steroids and IVIg, 79% for rituximab and 80% for thrombopoietin receptor agonists. After 4.4-year follow-up, ITP was considered in remission in 53%.
Conclusion
LM-related ITP is a rare condition, with an incidence of 0.073/100,000 person-years for CLL, 0.072/100,000 for B cell lymphoma and 0.059/100,000 for T cell lymphoma. The diagnosis of LM precedes or is concomitant of ITP in most of the cases (90%). Responses rates to usual ITP therapies seem to be lower than in primary ITP, with a remission rate of 50%.
Keyword(s): ITP, Lymphoid malignancy
Abstract: EP1143
Type: E-Poster Presentation
Session title: Platelet disorders
Background
The incidence of immune thrombocytopenia (ITP) is around 3/100,000 person-years. ITP is associated to another disease in 15-20% of the cases, mostly systemic auto-immune diseases, or hematological malignancies. For the latter, the association is principally reported with chronic lymphoid leukemia (CLL), large granular leukemia (LGL) and Hodgkin lymphoma (HL). As data have been derived from cohorts from tertiary centers with a possible selection bias, the accurate incidence rate of lymphoid malignancies (LM)-related ITP remains elusive.
Aims
Determination of the frequency, clinical characteristics and evolution of LM-related ITP based on the prospective data collected in the register of hematological malignancies of Côte d’Or (RHEMCO), a population-based register recording all incident hematological malignancies occurring in a restricted geographical area of 532,886 inhabitants.
Methods
Patients included in RHEMCO between January 1995 and December 2015 (n=3499) were analyzed to calculate incidences. The medical records of ITP patients referred to our tertiary hospital during the same period were also considered for the clinical description and the evolution of LM-related ITP. ITP was defined as a thrombocytopenia <100 G/L after exclusion of other causes. Response was defined as a platelet count >30 G/L with at least a 2-fold increase of baseline level.
Results
Based on RHEMCO, the frequency of ITP during LM was 1.06% (n=37/3499): 3.46% for T cell lymphoma, 1.21% for CLL, 0.82% for B cell lymphoma and 0.36% for HL. The frequency of Evans’ syndrome (ES) was 0.29% (n=10/3499) in the whole cohort: 0.44% during CLL, 0.43% for T cell lymphoma, and 0.24% for B cell lymphoma.
From RHEMCO and medical records, 76 patients with secondary ITP were identified, among which 17 with ES. For ITP, the median age at diagnosis was 69.5 [interquartile range (IQR): 19-94] years and 59% were male. For ES, the median age was 62 [IQR: 29-85] and 76% were male. Among all LM-related ITP, CLL and indolent B cell lymphoma were the most represented (29% each), followed by T cell lymphoma (20%), aggressive B cell lymphoma (18.5%) and HL (3.5%). ES was mostly associated with indolent B cell lymphoma (47%) or CLL (41%), T cell lymphoma or HL representing only 6% each. LM occurred before ITP in 46% of the cases, with a median delay of 3 [IQR: 0.5-18.4] years and in 65% for ES with a delay of 5.1 [IQR: 0.3-10.1] years. LM and ITP were simultaneous (≤ 3 months) in 44% for ITP and 29% for ES. Bleedings were reported in 45% and splenomegaly in 29% of the cases. The median platelet count at diagnosis was 30 [IQR: 1-96] G/L.
All the patients were treated: 84% received specific medications for ITP and 91.5% for LM. Responses to ITP treatments were 63% for steroids and IVIg, 79% for rituximab and 80% for thrombopoietin receptor agonists. After 4.4-year follow-up, ITP was considered in remission in 53%.
Conclusion
LM-related ITP is a rare condition, with an incidence of 0.073/100,000 person-years for CLL, 0.072/100,000 for B cell lymphoma and 0.059/100,000 for T cell lymphoma. The diagnosis of LM precedes or is concomitant of ITP in most of the cases (90%). Responses rates to usual ITP therapies seem to be lower than in primary ITP, with a remission rate of 50%.
Keyword(s): ITP, Lymphoid malignancy