Subcutaneous Panniculitis-Like T Cell Lymphomas With Lung Involvement:a Case Report And Literature Review

Objective: To study the clinical features,diagnosis and treatments of subcutaneous panniculitis-like T cell lymphomas(SPTCL)with Lung involvement,aiming to improve the clinician’s understanding of SPTCL with lung involvement.Methods: This study retrospectively reviewed the clinical data of a case of subcutaneous panniculitis-like T-cell lymphoma with lung involvement in the first affiliated hospital of Guangxi Medical University in December 2018,and to search the databases,such as China national knowledge internet,Wanfang and Pubmed,systematic analysis the relevant literature and summarize the clinical characteristics.Results: 1.Data of this patient:(1)General information: Male,65 years old,freelancer;(2)Clinical manifestations: The symptoms include repeated high fever,cough,expectoration and dyspnea,and the whole body was scattered in some painless red rashes,mainly in the trunk.Physical examination: The whole body was scattered in some red rashes,mainly in the trunk,the diameter of skin rashes were about 1-4 cm,some of the rashes ulcers,necrosis,or forms a scab.wheezy phlegm was sounded in the double lung.(3)Imaging examination:Chest CT: we can see multiple patchy shadows in two lungs,there was a large patchy shadow in the lingual segment of upper lobe of left lung,close to the pleura,and pleural cavity effusion.(4)Pathology: the pathology of skin biopsy:the epidermis was slightly atrophied and thinned,a large number of lymphoid cells infiltrated around the superficial vessels of the dermis,some of the nucleus were large and deep staining,a small number of cells moved into the epidermis,and PAS staining,anti-acid staining was all negative.Immunohistochemistry:CD45RO(++),CD20(-).It was diagnosed as subcutaneous paniculitis-like T-cell lymphoma.The pathology of the left lung biopsy: a large number of foamy cells were found in the partial region of alveolar tissue and filled with the alveolar lumen,the alveolar walls was fibration and hyalinization.we can see coagulation necrosis in the other part of lung tissue,PAS staining,anti-acid staining was all negative.(5)The treatment and prognosis: the patient had been treated with anti-infection therapy,but it was not effective,and he was diagnosed as subcutaneous paniculitis-like T-cell lymphoma with lung involvement after the skin and lung biopsy.Because of the dysfunction of multiple organs,the patient died finally.2.The results of systematic literature review: From 1991 to 2019,12 cases of subcutaneous paniculitis-like T-cell lymphoma with lung involvement were reported,including 7 cases(58.3%)in our country and 5 cases(41.7%)in foreign countries.(1)The general situation of the patients: The disease more occurred in young and middle-aged men,the onset age is 19 ~ 82 years old,the average age is 45 years old,median age is 46 years old;(2)Clinical manifestations: Fever is common,skin lesions are mainly painless subcutaneous nodules,ecchymosis or plaques,and some of the lesions accompanied by ulceration and necrosis;Cough is the main respiratory symptom,patients could be accompanied with expectoration,dyspnea,chest pain and hemoptysis.(3)Imaging: the image of the lung lesions can be single or multiple nodules,patchy shadows and solids,close to the pleura,most of them were accompanied with mediastinal lymph node enlargement or pleural effusions,other can be irregular masses,changes of interstitial pneumonia,thickening of pleura,and so on.(4)All the 12 patients(100%)were diagnosed as SPTCL by skin biopsy,which was characterized by cytotoxic T lymphocytes infiltrating subcutaneous adipose tissue and the tumor cells characterized by rosette-like changes around a single adipocyte.2 cases(16.7%)showed atypical lymphocytic infiltration around the pulmonary blood vessels and interstitial by lung biopsy pathology,1 case(8.3%)showed CD8+ lymphocytics infiltration in the pulmonary parenchyma,and 1 case(8.3%)tumor cells was found in the pleural effusion,the rest were diagnosed by observing the clinical characteristics and the effect of anti-tumor treatment.The pathology of lung lesions is an important evidence for the diagnosis of lymphoma lung invasion.(5)After 12 patients were diagnosied,10 cases(83.3%)were treated with CHOP chemotherapy regimen,but 3 cases died,1 case gave up treatment,1 case was relieved for about 30 months after treated with second-line regimen(etoposide)and 1 case treated with hematopoietic stem cell transplantation(HSCT),the patient has been in remission for 4 years,the rest had no follow-up data after being discharged from hospital.1 case(8.3%)was treated with FMD regimen,1 case(8.3%)was treated with immunosuppressant,and they were all died;The time from clinical symptoms occurred to death in 5 patients was 5 to 22 months,with an average of 14 months.Compared with the type of skin involvement only,this disease progresses faster,was more prone to occurred hemophagocytic syndrome,and the overall prognosis was worse.Conclusion: 1.Subcutaneous panniculitis-like T cell lymphomas with pulmonary involvement is rare and characterized by fever,rashes and cough,dyspnea,pulmonary speckle shadow and progressive multiple organs failure.2.The diagnosis required the pathology of skin and lung lesions,the pathological features of skin biopsy are cytotoxic T lymphocytes infiltrating subcutaneous adipose tissue,tumor cells around a single adipocyte presented a rosette-like change;atypical lymphocyte infiltrate the lung tissue in lung lesions.3.At present,systemic chemotherapy(such as CHOP regimen)is the main treatment,immunosuppressive agent,local radiotherapy and hematopoietic stem cell transplantation may also effective,but the overall prognosis is poor.