Primary Pulmonary Peripheral T-cell Lymphoma,not Otherwise Specified Lymphoma:A Case Report And Literature Review

Objective:To investigate the clinical characteristics,diagnosis and therapy of the primary pulmonary peripheral T-cell lymphoma,not otherwise specified(PTCL-NOS)disease and decrease the misdiagnosis.Method : Retrospectively review of a case of primary pulmonary PTCL-NOS diagnosed in the first affiliated hospital of Guangxi Medical Uniwersity in 2018.Retrieveing relevant pulmonary PTCL-NOS literatures to analyzed its clinical characteristics.Results:1.Data in this case:(1)General information: The patient was a male staff member,26 years old;(2)Clinical manifestations: Start with respiratory symptoms such as cough,hemoptysis,and difficulty breathing,progressive hemoptysis,difficulty breathing and weight loss.Physical examination: Cachexia,both lungs have weak breath sounds,a small amount of wet rales appeared in the two middle and lower lungs,dry voice appeared in the right lower lung,and the pleural friction sound is not appeared.(3)Imagingexamination: Chest CT showed large patchy,mass-like density of both lungs,cross-leaf span,accompany air bronchograms,cavities,tracheal gathering or cystic dilatation in one or both lung fields,pleural effusions formation.(4)Ultrasound-guided percutaneous lung biopsy pathological examination: Positive for CD2?CD3?CD4?CD7?TIA-1?CD43?CD56?Bcl-2?CD21.negative for CD8 ? GB ? CD5 expression.EBERs(-).(5)The external hospital was misdiagnosed as pneumonia,bronchiectasis,tuberculosis and pulmonary fungal disease.The primary pulmonary PTCL-NOS was considered by our hospital for diagnosis.(6)The anti-tuberculosis,anti-bacterial and fungal treatments in the outer hospital are not effective,and the condition continues to progress.Eventually,he died of respiratory failure after half a month of diagnosis in our hospital.2.Systematic literature review results: 9 cases of primary pulmonary PTCL-NOS reported from 1991 to 2019,including this case are totally 10 cases,secondary pulmonary PTCL-NOS reported from 2000 to 2019 there were 9cases,all of which was case report.(1)Primary pulmonary PTCL-NOS clinical respiratory symptoms as the main symptoms,manifested as cough,fever,shortness of breath,hemoptysis,etc,cachexia.Secondary pulmonary PTCL-NOS with systemic symptoms as the main symptoms,manifested as fever,dry cough,shortness of breath,night sweats,fatigue,more with superficial lymphadenopathy,liver and spleen swollen.(2)Imaging findings primary pulmonary PTCL-NOS is more common with nodular mass and pneumonia,mainly as diffuse lesions of both lungs,multiple plaques,solid images,cross-leaf span,or manifested as a mass shadow,with or without cavity necrosis,air bronchograms.Secondary pulmonary PTCL-NOS is more common in interstitial and miliary types,showing multiple small nodules in both lungs,ground glass,interstitial inflammation,with interlobular thickening,crazypaving pattern,submucosal masses,etc.Hilar,mediastinal lymphadenopathy are common.(3)Pathologically all cases showed CD3 positive expression.(4)Primary and secondary pulmonary PTCL-NOS treatment is mainly based on CHOP chemotherapy,primary pulmonary PTCL-NOS in 10 cases,8 patients treated with chemotherapy(3 patients were stable after treatment,followed up for 14 months;5 patients died,the survival time was 1 to 10 months,and the average survival time was 3.9 months).2 cases were not treated(1 case was unknown,1 case died after half a month of diagnosis).The total death rate was6 cases,accounting for 60.0%.The survival time was 0.5 months to 10 months,and the average survival time was 3.4 months.9 cases of secondary pulmonary PTCL-NOS,8 patients were treated with chemotherapy(4 cases were stable after treatment,follow-up for 14 months;4 cases died,survival time was 2.3 to10 months,average survival time was 6.2 months).1 patient had not been treated for death on the day of diagnosis.The total death rate was 5 cases,accounting for 55.6%.The survival time was 1 day to 10 months,and the average survival time was 5.0 months.Conclusion : 1.Peripheral T-cell lymphoma,not otherwise specified is highly invasive,with high mortality and poor prognosis.Especially,primary pulmonary PTCL-NOS has a shorter mean survival time than secondary pulmonary PTCL-NOS.2.Primary pulmonary PTCL-NOS chest imaging lung exudation,consolidation changes more significant.Secondary pulmonary PTCL-NOS hilar and mediastinal lymphadenopathy are more common.3.Positive for CD3 is the most specific in PTCL-NOS.4.First-line treatment of CHOP regimen is not effective,so far there is no uniform treatment standard,hematopoietic stem cell transplantation has some value.New drugs and new drugs combined with chemotherapy are hot topics in future research.