| Objective:Cutaneous T-cell lymphomas(CTCL)is a group of extra-nodal non-Hodgkin with initial presentation in the skin,characterized by malignant proliferation of mainly T-helper lymphocytes.International society for skin lymphoma(ISCL)in 2002 to discuss the clinical definition of CTCL erythroderma sex,it can be divided into three subtypes:Sezary syndrome(erythroderma CTCL phase of leukemia),erythrodermic mycosis granuloma and other undefined erythroderma CTCL.This article mainly focuses on the effectiveness of interferon and retinoids in treating E-CTCL.Methods:We retrospectively studied two cases of E-CTCL admitted in First Affiliated Hospital of China Medical University.With the key word of“红皮病型蕈样肉芽肿”、“Sezary 综合征”、“erythrodermic cutaneous T-cell lymphoma”、“erythrodermic mycosis fungoides”、“Sezary syndrome”,we also collected 34 cases that domestic and foreign literature reported from January 2001 to May 2017.Then choose ten cases whose data more complete and reliable from them.Compare their serologic abnormality,treatment,onset time,prognosis,and so on.Results:Case 1,female,30 years old,peripheral blood,Sezary cell 0.11.Skin tissue pathology:Angle of layer exists,a few parakeratosis,granular layer exists,in the stratum spinosum edema is not obvious,focal single nuclear cell spillover,have seen Pautrier cyst,leather can be obviously a single nuclear cell infiltration,shallow slightly assumes the moss samples,part of the enlarged hyperchromatic nuclei.Sezary syndrome was diagnosed.Case 2,male,56 years old,no obvious abnormality in laboratory examination.Skin tissue pathology:Angle of layer exists,epidermis rules decurrent,as a single nuclear cell within the epidermis,cells are alien,base layer focal liquefaction,dermal see density within a single nuclear cell infiltration,had mild atypia.The diagnosis was erythroderma mushroom granuloma.Here we report 2 cases of E-CTCL presenting with erythema on the trunk and extremities,intensegeneralized refractory pruritus.According to 2006,the European organization for cancer treatment studies recommended treatment,erythrodermic mycosis granuloma first-line treatment are:PUVA(psoralen and ultraviolet)combined interferon,interferon,MTX(methotrexate),TSEB(total skin electron beam)/shallow X-ray irradiation,nitrogen mustard or comer SiTing,photochemical treatment,in vitro PUVA joint class of drugs.Second-line treatments:bezalotine,chemotherapy.First-line treatment Sezary syndrome are:chemical treatment,TSEB and(or)shallow X-ray irradiation,Mr Perotin,ground,interleukin,interferon,CD52 sheet resistance,resistance small dose MTX.Case 1 and case 2 both treated with combination of interferon a2b(both dose 3 MU three times weekly)and etretinate(initial dose 30mg daily and 40mg daily),case 1 and case 2 both improve markedly.In case 1,the body and limbs were reduced after 24 days,and a small number of flake scales were seen in the skin folds,and the red tide gradually decreased,and the lymph nodes decreased slightly.In case 2,the skin lesions were relieved after 9 days of drug use,and the body was diffuse red,a small amount of scales,and the swelling subsided,and the cervical,axillary and inguinal lymph nodes were significantly reduced..Case 1 and case 2 are both alive,with a definite diagnosis of 94 months and 90 months.The average survival time of erythroderma CTCL in the United States was 3 years.In Japan,the average survival time of patients with erythroderma CTCL was 5 years.Recently,the average survival time in the UK was 3.4 to 4.7 years.The two cases in this paper were extended in comparison with the average survival time of both domestic and foreign reports.Conclusion:1.Interferonα combined with retinoic acid for treatment of erythrodermic cutaneous t-cell lymphoma is effective.2.Interrupt interferon in the treatment process can cause the disease to relapse and progress rapidly.3.The prognosis of erythroderma mushroom granuloma is better than that of sezary syndrome. |
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