Contributions
Abstract: PB1563
Type: Publication Only
Session title: Hodgkin lymphoma - Clinical
Background
malignant lymphoid hemopathies represent a heterogeneous group, many authors have reported a history of familial neoplasia in these patients, in particular, in case of Hodgkin lymphoma, non-Hodgkin lymphoma , chronic lymphocytic leukemia and multiple myeloma. The pathophysiology involves genetic factors since a close link with certain HLA groups is found. The data in the literature are contradictory, however, many studies indicate that these clinical forms have certain peculiarities: an older age at diagnosis compared to the sporadic forms, a predominance of the male sex, of the scleronodular type and Poppema and Lennert's paragranuloma, extensive stages and extra nodal involvement.
Aims
to analyze the clinical and progressive characteristics of patients with Hodgkin lymphoma and with a history of familial neoplasia (hematological or extra-hematological) and to seek a correlation between the observed data and this parameter over a period of 4 years.
Methods
we conducted a retrospective, single-center and descriptive study on 98 patients with Hodgkin lymphoma enrolled in the Hematology department of Centra Hospital during the period (2016-2019), a history of familial neoplasia is found. in 08 cases (8%).
Results
in 8/98 patients with HL with a history of familial neoplasia, we found a history of Hodgkin lymphoma in 1 case (in the brother), digestive cancer in 4 cases (cousins), breast cancer in 1 case (2 sisters), uterine neoplasia in 1 case and lung carcinoma in 1 case (father). The mean age at diagnosis is 35 years in the sporadic forms and 37.5 years in the forms with a history of familial neoplasia, p = 0.12 (ns). Three out of 5 patients are male in the group with familial neoplasia vs 53/90 in the sporadic group, p = 0.22 (ns).The scleronodular type is found in the 8 cases of LH with familial neoplasia vs 74/90 (in the sporadic forms), p = 0.09 (ns), only one patient in our cohort presents a Poppema and Lennert paragranuloma, it belongs to in the 1st group (with familial neoplasia), p = 0.0004 (s). Four out of 8 patients are classified as localized stages (I-II) versus 30/90 in the sporadic forms, p = 0.38 (ns), a bulky mass (diameter greater than 7cm and / or IMT> 0.35) is found in 6 out of 8 cases versus 34/90 in the 2nd group, p = 0.02 (s). Extra nodal location (contiguous to a lymph node involvement) is noted in 3 out of 8 cases in the forms with familial neoplasia vs. 18/90 in the sporadic forms, p = 0.12 (ns)
By applying the prognostic classification of the EORTC (European Organization for Research and Treatment of Cancer) in the localized stages and the IPS (international Prognostic Score) in the extended stages, all patients with HL with familial neoplasia are classified in the unfavorable group vs 58/90 in sporadic forms: p = 0.01 (s).We recorded, over a period of 4 years, 8 deaths in the sporadic forms (no deaths were noted in the group with familial neoplasia), p = 0.21 (ns). The 5-year overall survival (January 2016 to January 2021) is estimated at 91% all forms combined, this factor is not influenced by the family history of the patients (p = 0.32)
Conclusion
our results agree with the data in the literature: the family history of neoplasia gives patients with HL certain clinical characteristics, however, the impact of this factor on the evolution of patients is not confirmed in our study series.
Keyword(s): Genetic, Hematological malignancy, Hodgkin's disease, Hodgkin's lymphoma
Abstract: PB1563
Type: Publication Only
Session title: Hodgkin lymphoma - Clinical
Background
malignant lymphoid hemopathies represent a heterogeneous group, many authors have reported a history of familial neoplasia in these patients, in particular, in case of Hodgkin lymphoma, non-Hodgkin lymphoma , chronic lymphocytic leukemia and multiple myeloma. The pathophysiology involves genetic factors since a close link with certain HLA groups is found. The data in the literature are contradictory, however, many studies indicate that these clinical forms have certain peculiarities: an older age at diagnosis compared to the sporadic forms, a predominance of the male sex, of the scleronodular type and Poppema and Lennert's paragranuloma, extensive stages and extra nodal involvement.
Aims
to analyze the clinical and progressive characteristics of patients with Hodgkin lymphoma and with a history of familial neoplasia (hematological or extra-hematological) and to seek a correlation between the observed data and this parameter over a period of 4 years.
Methods
we conducted a retrospective, single-center and descriptive study on 98 patients with Hodgkin lymphoma enrolled in the Hematology department of Centra Hospital during the period (2016-2019), a history of familial neoplasia is found. in 08 cases (8%).
Results
in 8/98 patients with HL with a history of familial neoplasia, we found a history of Hodgkin lymphoma in 1 case (in the brother), digestive cancer in 4 cases (cousins), breast cancer in 1 case (2 sisters), uterine neoplasia in 1 case and lung carcinoma in 1 case (father). The mean age at diagnosis is 35 years in the sporadic forms and 37.5 years in the forms with a history of familial neoplasia, p = 0.12 (ns). Three out of 5 patients are male in the group with familial neoplasia vs 53/90 in the sporadic group, p = 0.22 (ns).The scleronodular type is found in the 8 cases of LH with familial neoplasia vs 74/90 (in the sporadic forms), p = 0.09 (ns), only one patient in our cohort presents a Poppema and Lennert paragranuloma, it belongs to in the 1st group (with familial neoplasia), p = 0.0004 (s). Four out of 8 patients are classified as localized stages (I-II) versus 30/90 in the sporadic forms, p = 0.38 (ns), a bulky mass (diameter greater than 7cm and / or IMT> 0.35) is found in 6 out of 8 cases versus 34/90 in the 2nd group, p = 0.02 (s). Extra nodal location (contiguous to a lymph node involvement) is noted in 3 out of 8 cases in the forms with familial neoplasia vs. 18/90 in the sporadic forms, p = 0.12 (ns)
By applying the prognostic classification of the EORTC (European Organization for Research and Treatment of Cancer) in the localized stages and the IPS (international Prognostic Score) in the extended stages, all patients with HL with familial neoplasia are classified in the unfavorable group vs 58/90 in sporadic forms: p = 0.01 (s).We recorded, over a period of 4 years, 8 deaths in the sporadic forms (no deaths were noted in the group with familial neoplasia), p = 0.21 (ns). The 5-year overall survival (January 2016 to January 2021) is estimated at 91% all forms combined, this factor is not influenced by the family history of the patients (p = 0.32)
Conclusion
our results agree with the data in the literature: the family history of neoplasia gives patients with HL certain clinical characteristics, however, the impact of this factor on the evolution of patients is not confirmed in our study series.
Keyword(s): Genetic, Hematological malignancy, Hodgkin's disease, Hodgkin's lymphoma