Primary pulmonary lymphoma in children

Abstract

BackgroundPrimary 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.ResultsWe 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.ConclusionsWhen 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.

Primary Pulmonary B-Cell Lymphoma: A Review and Update

“…Male and female patients are equally affected, and median age at presentation is around 60 years (range 30–80 years) [ 4 , 28 ], but it may occur at a younger age in immunosuppressed patients. Although rarely, PP-DLBCL has been described in children as well [ 89 ]. In a recent small series of pediatric primary pulmonary lymphomas, three out of four cases were DLBCL, and two of these patients had an underlying immunodeficiency [ 89 ].…”

“…Although rarely, PP-DLBCL has been described in children as well [ 89 ]. In a recent small series of pediatric primary pulmonary lymphomas, three out of four cases were DLBCL, and two of these patients had an underlying immunodeficiency [ 89 ].…”

“…Hilar adenopathy may be seen. Imaging findings of consolidation of multiple pulmonary nodules with air bronchograms and a halo of ground-glass shadowing at lesion margins (so-called ‘halo sign’) [ 92 ] have been described in pediatric cases as well [ 89 ]; such halo sign may be due to peritumoral extension of lymphoma and/or bleeding. FDG-PET usually demonstrates metabolic activity [ 93 ].…”

A decade's experience of pediatric lung abscess and empyema at a community hospital

“…Case 2 had a prolonged workup that lead to a late diagnosis of a primary pulmonary lymphoma (PPL). PPL in pediatrics is rare with only 11 reported cases 22–26 . Patients present with cough and fever and are treated for pneumonia causing an average delay in diagnosis of 8 weeks.…”

Primary pulmonary diffuse large B-cell lymphoma with multiple ground-glass nodules as the primary manifestation

“…Although the mechanisms of the increased incidence of lymphoproliferative diseases in immunodeficient patients are not fully understood, deterioration in immunoregulation, chronic antigenic stimulation, and tumor suppressor system dysregulation are thought to be the main reasons for it. [ 10 ]…”