Abstract Topic: 18. Indolent and mantle-cell non-Hodgkin lymphoma - Clinical
Background: Ocular adnexal Non Hodgkin Lymphomas (NHL) consist 2% of all NHL. The most frequent hystological sybtipe is ocular adnexal MALT (Mucosa-Associated Lymphoid Tissue) Lymphoma (OAML). Autoimmune inflamatory disorders and chronic infections can be important ethiological factors. A broad spectrum of various local signs and simptoms of disease can much before diagnosis is established. Various therapy options are available for this lymphoma treatment.
Aims: Goal of this research is twenty-year period retrospective analysis of our patients (pts) diagnosed with primary OAML. We researched epidemiologic-demographic, clinical and laboratory characterstics on the presentation. Effectiveness of various therapy approaches was also compared, as well as disease outcome.
Methods: Overall, 32 pts with primary OAML were inrolled. All pts were diagnosed and staged following physical examination, laboratory findings, contrast computerised tomography (CT) of neck, chest, abdomen and pelvis, bonne marrow biopsy as well as eye’s ultrasound or CT with ophtalmological examination. Remissions, relapses and lymphoma transformations were confirmed via laboratory and radiography check-ups, with bonne marrow rebiopsies if systemic involevement was suspected. Methods of parametric and non-parametric statistics were used wih accent on descriptive statistical analyses.
Results: In our group the highest incidence for this lymphoma occurement was in 8th decade, with greater incidence in males. Mostly heterogeneous and variuos initial local signs and symptoms differ, depending of eye structure involvement and can occur long period of time prior to diagnosis (median 9 months). Laboratory parameters of disease activity were in average normal or slightly increased. In subgroup where it was inveastigated, a presence of Hp was recognized in 66.67% of pts. No predictive significance of sialophorin was confirmed. All pts had localised disease (B symptoms in 12.5% of cases), with most frequent orbital soft tissue involvement (46.87%, followed by conjuctival involvement in 34.37%), being classified in low or intermediate low risk group. In our group best disease control in initial, as in diasease progression treatment, was eastablished following chemo or radio therapy. Half of our pts had systemic or local disease progression (relapse). The most frequent relapses occured in subgroup of our patients initially treated with surgical approach (71.42% relapased), whereas best intial response was registered in subgroups of pts treated initially with radiotherapy (20% relapsed) and chemotherapy (44.4%). Mean time from remission to relapse was 33 months (2-256). Five year progression free survival, (5-Y PFS) was 65% and 10-Y PFS was 48%. We concluded good disease control in OAML progression treatment with chemo or radiotherapy. Five year overal survival (5-Y OS) in our group of pts was 100%, and 10-Y OS was 88%. In 6.25% of case we detected disease transformation in “high grade“, agressive diffuse large B cell lymphoma. In our group five pts died, two in relation with lymphoma progression.
Summary/Conclusion: Our results confirm that OAML is and indolent lymphoma with good overall therapy response. Nevertheless it aquires regular check-ups of pts after initial remission acheivement, because of significant disease progression rate, as well as “high grade“ transformation possibility, with primary goal of proper re-diagnostics and treatment, as well as overall provision of good quality of life.
Keywords: Marginal zone, Radiotherapy, Extranodal lymphoma, Chemotherapy