Contributions
Abstract: PB2026
Type: Publication Only
Background
Castleman disease (CD) is a non-clonal lymphoproliferative disorder as a common cause of non-neoplastic lymphadenopathy. CD encompasses several distinct clinicopathological disorders at the intersection of haematology, immunology, oncology, rheumatology and virology that share a spectrum of histopathological features. An international collaborative working group has reached consensus definitions and classification, defining diagnostic criteria for CD which enables to clinicians reaching the proper diagnose.
Aims
The aim of this study is to review our CD patients from a single centre according to newly established diagnostic criterias.
Methods
All patients with a biopsy proven histopathological characteristics of CD diagnosed at Ege University Hospital between 2000-2017 years were reviewed for analysis. Clinical and laboratory datas were collected retrospectively. The patients were divided into two main groups based on the anatomical distribution of the disease: Unicentric CD (UCD) and multicentric CD (MCD). Also MCD were divided into two groups: HHV8 positive MCD and idiopathic MCD.
Results
A total of 64 patients were reviewed. Among the study group; 34 patients were excluded because two were diagnosed with synchronous Hodgkin lymphoma, one was diagnosed with POEMS, and the rest were unable to access all data. Detailed clinical and laboratory datas were summarized in Table 1. The mean age at diagnosis of 30 patients with adequate data was 48.8 (26-82). After histopathological evaluation, majority had hyaline vascular type (n=19), followed by mixed type (n=8) and plasma cell type (n=3). There were 16 patients in UCH group, none of them had HHV-8 and HIV positivity. After a median follow up of 54 months, the estimated 2-year OS was 87.7% [95% confidence interval (CI): 72.1–103.3]. There were 7 patients in the HHV-8+MCH group and two of them had Kaposi sarcoma. All of whom received chemotherapy and/or immunotherapy treatments. Clinical manifestations in the HHV-8+MCH group were; fever, splenomegaly, skin lesions, acute renal failure, oedema, effusion, respiratory symptoms, CRP/ferritin and LDH elevation. After a median follow up of 38.2 months, the estimated 2-year OS was 46% [95% confidence interval (CI): 17.1–74.9]. There are 7 patients in the iMCH group with HHV-8 negative and the clinical findings are very similar to the HHV-8+MCH group. Two-year overall survival in iMCH group was higher than HHV-8+MCH with 82.7% [95% confidence interval (CI): 58.6–106.8].
Conclusion
CH is a very rare lymphoproliferative disease which should be kept in mind in the differential diagnosis with asymptomatic and localized lymphadenopathies or widespread lymphadenopathies with severe systemic symptoms. Future studies should be multicentred and collaborative in order to evaluate significant numbers of patients and to establish up to date and effective treatment protocols for this rare but potentially life-threatening disorder.
Session topic: 20. Indolent Non-Hodgkin lymphoma – Clinical
Keyword(s): Castleman's disease, HIV related lymphoma
Abstract: PB2026
Type: Publication Only
Background
Castleman disease (CD) is a non-clonal lymphoproliferative disorder as a common cause of non-neoplastic lymphadenopathy. CD encompasses several distinct clinicopathological disorders at the intersection of haematology, immunology, oncology, rheumatology and virology that share a spectrum of histopathological features. An international collaborative working group has reached consensus definitions and classification, defining diagnostic criteria for CD which enables to clinicians reaching the proper diagnose.
Aims
The aim of this study is to review our CD patients from a single centre according to newly established diagnostic criterias.
Methods
All patients with a biopsy proven histopathological characteristics of CD diagnosed at Ege University Hospital between 2000-2017 years were reviewed for analysis. Clinical and laboratory datas were collected retrospectively. The patients were divided into two main groups based on the anatomical distribution of the disease: Unicentric CD (UCD) and multicentric CD (MCD). Also MCD were divided into two groups: HHV8 positive MCD and idiopathic MCD.
Results
A total of 64 patients were reviewed. Among the study group; 34 patients were excluded because two were diagnosed with synchronous Hodgkin lymphoma, one was diagnosed with POEMS, and the rest were unable to access all data. Detailed clinical and laboratory datas were summarized in Table 1. The mean age at diagnosis of 30 patients with adequate data was 48.8 (26-82). After histopathological evaluation, majority had hyaline vascular type (n=19), followed by mixed type (n=8) and plasma cell type (n=3). There were 16 patients in UCH group, none of them had HHV-8 and HIV positivity. After a median follow up of 54 months, the estimated 2-year OS was 87.7% [95% confidence interval (CI): 72.1–103.3]. There were 7 patients in the HHV-8+MCH group and two of them had Kaposi sarcoma. All of whom received chemotherapy and/or immunotherapy treatments. Clinical manifestations in the HHV-8+MCH group were; fever, splenomegaly, skin lesions, acute renal failure, oedema, effusion, respiratory symptoms, CRP/ferritin and LDH elevation. After a median follow up of 38.2 months, the estimated 2-year OS was 46% [95% confidence interval (CI): 17.1–74.9]. There are 7 patients in the iMCH group with HHV-8 negative and the clinical findings are very similar to the HHV-8+MCH group. Two-year overall survival in iMCH group was higher than HHV-8+MCH with 82.7% [95% confidence interval (CI): 58.6–106.8].
Conclusion
CH is a very rare lymphoproliferative disease which should be kept in mind in the differential diagnosis with asymptomatic and localized lymphadenopathies or widespread lymphadenopathies with severe systemic symptoms. Future studies should be multicentred and collaborative in order to evaluate significant numbers of patients and to establish up to date and effective treatment protocols for this rare but potentially life-threatening disorder.
Session topic: 20. Indolent Non-Hodgkin lymphoma – Clinical
Keyword(s): Castleman's disease, HIV related lymphoma