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PRIMARY BONE LYMPHOMA (PBL) - ?REAL WORLD? EXPERIENCE OF A SINGLE CANCER HOSPITAL. CLINICAL CHARACTERISTICS, PROGNOSTIC FACTORS AND MANAGEMENT OF TWELVE PATIENTS.
Author(s): ,
Dionisios Stoumbos
Affiliations:
Hematology,Metaxa Cancer Hospital,Peiraias,Greece
,
Ioannis Patsias
Affiliations:
Hematology,Metaxa Cancer Hospital,Peiraias,Greece
,
Georgia Papageorgiou
Affiliations:
Hematology,Metaxa Cancer Hospital,Peiraias,Greece
,
Evangelos Fergadis
Affiliations:
Hematology,Metaxa Cancer Hospital,Peiraias,Greece
,
Christos Gkekas
Affiliations:
Hematology,Metaxa Cancer Hospital,Peiraias,Greece
,
Evangelia Eleni Christaki
Affiliations:
Hematology,Metaxa Cancer Hospital,Peiraias,Greece
,
Evangelos Asmanis
Affiliations:
Hematology,Metaxa Cancer Hospital,Peiraias,Greece
,
Polyxeni Lampropoulou
Affiliations:
Hematology,Metaxa Cancer Hospital,Peiraias,Greece
,
Anastasia Tsakiridou
Affiliations:
Hematology,Metaxa Cancer Hospital,Peiraias,Greece
,
Panagiotis Repousis
Affiliations:
Hematology,Metaxa Cancer Hospital,Peiraias,Greece
,
Aikaterini Megalakaki
Affiliations:
Hematology,Metaxa Cancer Hospital,Peiraias,Greece
,
Ioannis Skouras
Affiliations:
Radiology,Metaxa Cancer Hospital,Peiraias,Greece
,
Aris Petridis
Affiliations:
Radiation Oncology,Metaxa Cancer Hospital,Peiraias,Greece
Maria Kotsopoulou
Affiliations:
Hematology,Metaxa Cancer Hospital,Peiraias,Greece
(Abstract release date: 05/19/16) EHA Library. Stoumpos D. 06/09/16; 134635; PB1735
Dr. Dionysios Stoumpos
Dr. Dionysios Stoumpos
Contributions
Abstract
Abstract: PB1735

Type: Publication Only

Background
PBL was first described as a distinct clinopathological entity on ‘reticulum cell sarcoma of bone’ in 1939 by Parker and Jackson. It is a very rare condition which constitutes less than 1% of all malignant lymphomas, less than 2% of all bone tumors and less than 5% of extra-nodal lymphomas. A number of studies have been reported, but most with a limited number of patients.Being an uncommon entity, there is a lack of experience in paramaters such as clinical characteristics and optimal management.

Aims
To determine patient characteristics, prognostic and treatment factors that could affect outcome measured by overall survival (OS) and complete remission (CR).

Methods
Herein, we retrospectively review 12 patients diagnosed with PBL and treated at our institution from 2001 to present. 

Results
The demographic and clinical characteristics of the 12 patients at the time of diagnosis are summarized in Table 1. The median age of the patients was 49 years old (range, 31-81 years). Eight (70%) patients were female and 4 (30%) male. The histological type of all 12 patients (100%) was Diffuse Large B-Cell Lymphoma. Ten patients (83%) were presented with Ann Arbor Stage I or II disease. Two patients (17%) had high stage disease. The median follow-up after achieving CR (without relapse) to date was approximately 80 months (range, 0-170 months). Our analysis demonstrated that age less than 47 years normal LDH level, Ann Arbor Stage I or II and female gender were found to be favorable prognostic factors for achieving CR and OS. Notably, the pelvis was the most frequently primary involved site of the bone (5; 41.5%). All twelve patients underwent chemotherapy with most of them (10; 83%) receiving six to eight cycles of the RCHOP regimen. The majority (11; 92%) of them received radiotherapy. After front line chemotherapy followed by radiotherapy most patients (9; 75%) achieved CR. Among the remaining three patients, the one died before completing first line chemotherapy due to an infection while in chemotherapy-induced neutropenia. The other  patient is undergoing autologous stem-cell transplantation due to refractory PBL. The third one achieved CR after he underwent autologous stem-cell transplantation due to refractory PBL. Both patients that had refractory disease were male and initially diagnosed with high stage PBL. The median OS was 89,5 months (range, 6-178 months).
Table 1. Patient demographic and clinical characteristics
 MedianRange
Age49 years31 - 81
Gender M/F4 (30%) / 8 (70%)
Serum LDH250 IU/L137 - 857
Clinical Stage I & II10 (83%)
Pelvis as primary involved site of the bone5 (41,5%)
Initial CTx: RCHOP10 (83%)
Response to initial treatment CTx+RT: CR9 (75%)
ASCT2 (17%)
Follow up after CR (without relapse)80 months0 - 170
OS89.5 months6 - 178


Conclusion
Although the total number of patients was relatively small, the data from our study supports that most PBL patients have had early stage disease (IE-IIE) on diagnosis which also was the most important favorable prognostic factor. Overall, patients with primary lymphoma of the bone with DLBCL have an excellent prognosis and only a limited number of patients underwent autologous stem-cell transplantation in order to achieve CR.

Session topic: E-poster

Keyword(s): Bone disease, DLBCL, NHL, Radiotherapy
Abstract: PB1735

Type: Publication Only

Background
PBL was first described as a distinct clinopathological entity on ‘reticulum cell sarcoma of bone’ in 1939 by Parker and Jackson. It is a very rare condition which constitutes less than 1% of all malignant lymphomas, less than 2% of all bone tumors and less than 5% of extra-nodal lymphomas. A number of studies have been reported, but most with a limited number of patients.Being an uncommon entity, there is a lack of experience in paramaters such as clinical characteristics and optimal management.

Aims
To determine patient characteristics, prognostic and treatment factors that could affect outcome measured by overall survival (OS) and complete remission (CR).

Methods
Herein, we retrospectively review 12 patients diagnosed with PBL and treated at our institution from 2001 to present. 

Results
The demographic and clinical characteristics of the 12 patients at the time of diagnosis are summarized in Table 1. The median age of the patients was 49 years old (range, 31-81 years). Eight (70%) patients were female and 4 (30%) male. The histological type of all 12 patients (100%) was Diffuse Large B-Cell Lymphoma. Ten patients (83%) were presented with Ann Arbor Stage I or II disease. Two patients (17%) had high stage disease. The median follow-up after achieving CR (without relapse) to date was approximately 80 months (range, 0-170 months). Our analysis demonstrated that age less than 47 years normal LDH level, Ann Arbor Stage I or II and female gender were found to be favorable prognostic factors for achieving CR and OS. Notably, the pelvis was the most frequently primary involved site of the bone (5; 41.5%). All twelve patients underwent chemotherapy with most of them (10; 83%) receiving six to eight cycles of the RCHOP regimen. The majority (11; 92%) of them received radiotherapy. After front line chemotherapy followed by radiotherapy most patients (9; 75%) achieved CR. Among the remaining three patients, the one died before completing first line chemotherapy due to an infection while in chemotherapy-induced neutropenia. The other  patient is undergoing autologous stem-cell transplantation due to refractory PBL. The third one achieved CR after he underwent autologous stem-cell transplantation due to refractory PBL. Both patients that had refractory disease were male and initially diagnosed with high stage PBL. The median OS was 89,5 months (range, 6-178 months).
Table 1. Patient demographic and clinical characteristics
 MedianRange
Age49 years31 - 81
Gender M/F4 (30%) / 8 (70%)
Serum LDH250 IU/L137 - 857
Clinical Stage I & II10 (83%)
Pelvis as primary involved site of the bone5 (41,5%)
Initial CTx: RCHOP10 (83%)
Response to initial treatment CTx+RT: CR9 (75%)
ASCT2 (17%)
Follow up after CR (without relapse)80 months0 - 170
OS89.5 months6 - 178


Conclusion
Although the total number of patients was relatively small, the data from our study supports that most PBL patients have had early stage disease (IE-IIE) on diagnosis which also was the most important favorable prognostic factor. Overall, patients with primary lymphoma of the bone with DLBCL have an excellent prognosis and only a limited number of patients underwent autologous stem-cell transplantation in order to achieve CR.

Session topic: E-poster

Keyword(s): Bone disease, DLBCL, NHL, Radiotherapy

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