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Treatment and prognosis of primary pulmonary lymphoma: A long-term follow-up study
Michelina Santopietro1, Sofia Kovalchuk2, Roberta Battistini1
1Haematology and Haematopoietic Stem Cells Transplant Unit, AO San Camillo-Forlanini, Rome, Italy.
Abstract:
Primary pulmonary lymphoma (PPL) is a rare disease with not well-defined optimal treatment. Outcomes and follow-up are variable in published data.
Objectives:
To define the outcome and optimal treatment strategies in PPL.
Methods:
We reviewed the medical records of 49 patients with PPL treated in three Italian Hematological Institutions between 2002 and 2018.
Results:
Thirty-eight (77.5%) cases were indolent PPL, and 11 (22.5%) cases were aggressive PPL. The majority of patients were asymptomatic at diagnosis, early stages (stages IE-IIE), normal serum LDH, no bone marrow involvement, and low or low-intermediate risks of IPI. Local therapy ± immunotherapy or immuno-chemotherapy was possible in 18/49 (37%) patients. Twenty-eight (57%) patients were treated with immuno-chemotherapy after biopsy. Waiting and watching were reported in 3 (6%) patients. Overall, the CR and ORR were 83.7% and 95.9%. With a median follow-up of 62.5 months (range 0.8-199 months), the estimated 5- and 10-year OS rates were 85% and 72.3% for all patients, 89.2% and 80.3% for indolent PPL, and 70.7% and 47.1% for aggressive PPL. Aggressive PPL tended to have a high risk of progression in the first months (P = .056). No advantages were found for indolent PPL who received immuno-chemotherapy or more conservative approaches.
Conclusion:
Our studies confirm the epidemiological and favorable survival of patients with PPL, suggesting a very conservative approach, particularly in indolent subtypes.
Insights
Primary pulmonary lymphoma (PPL) is rare, but survival is favorable. A conservative treatment approach is recommended, especially for indolent PPL, as aggressive PPL has a higher risk of early progression.
Area of Science:
- Hematology
- Oncology
- Pulmonary Medicine
Background:
- Primary pulmonary lymphoma (PPL) is a rare neoplastic condition.
- Optimal treatment strategies and patient outcomes for PPL remain incompletely defined.
- Published data on PPL outcomes and follow-up show significant variability.
Purpose of the Study:
- To define the outcomes of patients diagnosed with PPL.
- To identify optimal treatment strategies for PPL.
- To analyze survival rates and prognostic factors in PPL.
Main Methods:
- Retrospective review of medical records for 49 PPL patients.
- Data collected from three Italian Hematological Institutions between 2002 and 2018.
- Analysis of treatment approaches, including local therapy, immunotherapy, immuno-chemotherapy, and watchful waiting.
Main Results:
- 38 (77.5%) cases were indolent PPL, 11 (22.5%) were aggressive PPL.
- High overall response rates (ORR) of 95.9% and complete remission (CR) of 83.7% were observed.
- Estimated 5- and 10-year overall survival (OS) rates were 85% and 72.3% for all patients, with better outcomes for indolent PPL.
Conclusions:
- PPL demonstrates favorable survival statistics, consistent with epidemiological data.
- A conservative management approach is suggested for PPL, particularly for indolent subtypes.
- Aggressive PPL subtypes showed a trend towards higher early progression risk.
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