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Primary pulmonary lymphoma in children
Xiaohui Wu1, Chunju Zhou2, Ling Jin3
1Department of Respiratory Medicine, Beijing Children's Hospital, Capital Medical University, Nanlishi Road 56, Xicheng District, Beijing, China.
Insights
Primary pulmonary lymphoma (PPL) is rare in children. This study analyzes four pediatric PPL cases, highlighting clinical features and diagnostic considerations for this uncommon condition.
Area of Science:
- Pediatric Oncology
- Hematology
- Pulmonology
Background:
- Primary pulmonary lymphoma (PPL) is an exceptionally rare malignancy, particularly in pediatric populations.
- Understanding the clinical presentation and diagnostic markers of PPL in children is crucial due to its rarity.
Purpose of the Study:
- To analyze the clinical features of PPL in four pediatric patients.
- To enhance the understanding and diagnostic approach to PPL in children.
Main Methods:
- Retrospective case series analysis of four pediatric patients diagnosed with PPL.
- Review of clinical presentations, laboratory findings, and imaging results (chest computed tomography).
- Genetic analysis for primary immunodeficiency in relevant cases.
Main Results:
- Four pediatric PPL cases were identified: three diffuse large B-cell lymphomas and one natural killer-T cell lymphoma.
- Common symptoms included prolonged fever, cough, weight loss, and fatigue.
- Radiological findings consistently showed pulmonary nodules with halo signs and air bronchograms on CT scans.
- Two patients were diagnosed with primary immunodeficiency, suggesting a potential link.
Conclusions:
- PPL should be considered in pediatric patients presenting with persistent fever, elevated C-reactive protein, altered white blood cell counts, and characteristic pulmonary nodules on CT.
- The co-existence of primary immunodeficiency warrants further investigation in children diagnosed with PPL.
Background:
Primary pulmonary lymphoma (PPL) is a rare disease, especially in children. We analyse the clinical features of PPL in 4 children to strengthen a understanding of it.
Results:
We reported a case series of 4 pediatric patients with PPLs including three diffuse large B-cell lymphomas and one natural killer-T cell lymphoma. All patients presented with unknown fever and cough as well as weight loss and fatigue. The white blood cell count was reduced in three patients and increased in the other one. The level of C-reactive protein was increased in all patients. The procalcitonin concentrations and bone marrow specimens were normal. Multiple or single pulmonary nodules with halo signs were found in all patients and air bronchograms found in 3 of them on chest computed tomography scan. Primary immunodeficiency was diagnosed in two patients who was performed genetic analysis.
Conclusions:
When a patient presents with long-term fever, high C-reactive protein level, leukopenia/leukocytosis, and multiple or single pulmonary nodules with a "halo sign" and air bronchogram on computed tomography, a possibility of PPL should be considered. A co-existance of immunodeficiency needs to be further investigated in patients with PPL.
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