Clinic And Pathologic Analysis Of 72 Patients With Primary Cutaneous Lymphoma

Objectives:1.To investigate the epidemiological characteristics of primary cutaneous lymphoma in Yunnan region.2. To study and analyze the clinical and pathological features of primary cutaneous lymphoma, and to deepen the cognition of the disease.3. Summarized the similarities,differences and main differential points of clinical manifestations and histopathology of various types of PCL in order to raise the diagnostic accuracy rate and reduce misdiagnosis, and therefore to improve early treatment and prognosis.Methods: 72 patients with primary cutaneous lymphoma from the First Affiliated Hospital of Kunming Medical University which had been diagnosed by clinical character and skin biopsy between Jan 2006 and Dec 2016 were reviewed.Analyse the clinical and pathological character by combined with clinical and pathological data of selected subjects and grouped according to different conditions.Results:1. A total of 72 cases of primary cutaneous lymphoma(PCL) were studied. Among of PCL, primary cutaneous T-cell lymphoma (PCTCL) was more common(86.11%), primary cutaneous B-cell lymphoma (PCBCL)was fewer(13.89%).2. Among of the patients with PCL, the male-to-female ratio was 1.4:1. The mean age of onset was (46.49±19.95) years,with a range from 3 to 83 years old,and the age fastigium of the patients was from 32 to 61 years.3. Among 72 cases of PCL, the shortest duration was 0.5 month, the longest duration was up to 30 years, and the average interval from onset to being diagnosed was 41.27 months. MF had the longest duration of PCL (55.98 months), and the difference between the duration before diagnosis of PCTCL and PCBCL was statistically significant(P=0.011<0.05).4. 86.11% of skin lesions were multiple and 13.89% were solitary. The solitary rate of PCBCL (50%) was significantly higher than that of PCTCL (8.06%). Skin lesions were occurring in all parts of the body, with the case of generalized lesions as the most common scenario, accounting for 45.83%; followed by trunk cases, accounting for 22.22%; limbs is 16.67%; while head and neck with least proportion, accounting for 15.28%.5. 48.61% cases had different extent itching, 19.44% cases had pain and 31.94% cases without evident self-conscious symptom.The cases without evident self-conscious symptom of PCBCL(80%) were significantly more than PCTCL(24.19%).6. Clinical and pathological features of PCTCL: mycosis fungoides (MF) is the most common type( rate 55.56%),the early MF is polymorphic skin lesions and atypical features of pathology. Its histopathology is characterized by epidermotropism,Pautrier's microabscesses and heterotypic lymphocytes infiltration in the dermis.Primary cutaneous CD30+ T-cell lymphoproliferative disorders have various clinical manifestations, spontaneous regression and ulcers were usually found. The pathological manifestation were dense infiltration of allotypic lymphocytes in dermis, these cells had large nucleus, rich in cytoplasmand most of the tumor cells were positive to CD30.Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) occur mostly on the trunk and limbs,presented with single or multiple subcutaneous nodules or plaques,some patients have fever and pancytopenia. Histologically, the lesions were primarily confined to the subcutaneous adipose tissue and atypical lymphocytes infiltrated and rounded the lipocytes like rosettes in subcutaneus adipose tissue. Tumor cells showed immunophenotype of cytotoxic T lymphocytes.Hydroa vacciniforme-like lymphoma have similar clinical features with HV,and tends to be present in younger children.The pathological manifestation were various atypical lymphocytic infiltrate surrounding the small vessels.Tumor cells showed immunophenotype of cytotoxic T lymphocytes and cytotoxic-granule-associated proteins. In some cases, NK cell-associated antigen CD56 was positive, and EBER was generally positive.Extranodal NK/T cell lymphoma, nasal type (ENKTCL-N) is a type of malignant lymphoma characterized by progressive destructive lesions of nasal and facial midline structures.Diffused infiltration around the dermis, coagulation necrosis and angiocentric infiltration were often observed in ENKTCL-N. Immunohistochemical staining express related antigen phenotype of NK or T cell, cytotoxic-granule -associated protein and EBER.7. The clinical manifestations of PCBCL were one or more asymptomatic nodules and(or) tumour. Histologically, the lymphoid infiltration was deep, dense infiltration of allotypic lymphocytes in dermis and subcutaneous adipose tissue .Epidermotropism was found in PCTCL but not in PCBCL. Tumor cells expressed B cell makers (CD20,CD79a were positive).Conclusions:1. Among of PCL,primary cutaneous T-cell lymphoma (PCTCL) was more common(86.11%), and MF accounted for 55.56% of PCTCL.Primary cutaneous B-cell lymphoma(PCBCL) was fewer (13.89%).2. PCL can be seen at any age, but usually seen in the elderly population. Skin lesions are mostly generalized and trunk-based. Except for severe itching in part of MF, no obvious symptoms or only mild itching can be found.3. The classification of PCL is complex, and various types of clinical manifestations and histopathological characteristics are distinct. The skin lesions of PCBCL is more limited than PCTCL, with rarer ulcers and deeper pathology infiltration. In PCBCL, involvement of the epidermis is rarely observed, whereas PCTCL often displays epidermotropism and is associated with shallow infiltration.4. MF is the most common type of PCL,and it can be easily misdiagnosed due to the fact that its early clinical manifestations and histopathology are lack of specificity. Accurate diagnosis of it requires strengthened clinical observation and histopathological follow-up.5. The combination of clinical manifestations, histopathological features,immunohistochemistry and gene rearrangement techniques is critical for the diagnosis and specific typing of PCL.