Primary pulmonary lymphoma in children
Abstract 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 lymphoma...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2019-02-01
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| Series: | Orphanet Journal of Rare Diseases |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s13023-019-1009-5 |
| _version_ | 1818015068762144768 |
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| author | Xiaohui Wu Chunju Zhou Ling Jin Hui Liu Jinrong Liu Shunying Zhao |
| author_facet | Xiaohui Wu Chunju Zhou Ling Jin Hui Liu Jinrong Liu Shunying Zhao |
| author_sort | Xiaohui Wu |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-04-14T06:52:25Z |
| format | Article |
| id | doaj.art-6baf28ac53cb49b78e3da5a5df9146d8 |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-04-14T06:52:25Z |
| publishDate | 2019-02-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-6baf28ac53cb49b78e3da5a5df9146d82022-12-22T02:06:59ZengBMCOrphanet Journal of Rare Diseases1750-11722019-02-011411510.1186/s13023-019-1009-5Primary pulmonary lymphoma in childrenXiaohui Wu0Chunju Zhou1Ling Jin2Hui Liu3Jinrong Liu4Shunying Zhao5Department of Respiratory Medicine, Beijing Children’s Hospital, Capital Medical UniversityDepartment of Pathology, Beijing Children’s Hospital, Capital Medical UniversityHematology Oncology Center, Beijing Children’s Hospital, Capital Medical UniversityDepartment of Respiratory Medicine, Beijing Children’s Hospital, Capital Medical UniversityDepartment of Respiratory Medicine, Beijing Children’s Hospital, Capital Medical UniversityDepartment of Respiratory Medicine, Beijing Children’s Hospital, Capital Medical UniversityAbstract 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.http://link.springer.com/article/10.1186/s13023-019-1009-5Primary pulmonary lymphomaChildrenImmunodeficiency |
| spellingShingle | Xiaohui Wu Chunju Zhou Ling Jin Hui Liu Jinrong Liu Shunying Zhao Primary pulmonary lymphoma in children Orphanet Journal of Rare Diseases Primary pulmonary lymphoma Children Immunodeficiency |
| title | Primary pulmonary lymphoma in children |
| title_full | Primary pulmonary lymphoma in children |
| title_fullStr | Primary pulmonary lymphoma in children |
| title_full_unstemmed | Primary pulmonary lymphoma in children |
| title_short | Primary pulmonary lymphoma in children |
| title_sort | primary pulmonary lymphoma in children |
| topic | Primary pulmonary lymphoma Children Immunodeficiency |
| url | http://link.springer.com/article/10.1186/s13023-019-1009-5 |
| work_keys_str_mv | AT xiaohuiwu primarypulmonarylymphomainchildren AT chunjuzhou primarypulmonarylymphomainchildren AT lingjin primarypulmonarylymphomainchildren AT huiliu primarypulmonarylymphomainchildren AT jinrongliu primarypulmonarylymphomainchildren AT shunyingzhao primarypulmonarylymphomainchildren |