Diagnostic and management challenges in primary cutaneous anaplastic large cell lymphoma with necrosis,inflammation,and surgical intervention:A case report

BACKGROUND Primary cutaneous anaplastic large cell lymphoma(PC-ALCL)poses significant diagnostic difficulties due to its similarity in the appearance of skin lesions with chronic inflammatory disorders and other dermatological conditions.This study aims to investigate these challenges by conducting a comprehensive analysis of a case presenting with PC-ALCL,emphasizing the necessity of accurate differentiation for appropriate management.CASE SUMMARY An 89-year-old female patient with diabetes and hypertension presented with arm and abdominal ulcerated mass lesions.Diagnostic procedures included skin biopsies,histopathological assessments,and immunohistochemistry,complemented by advanced imaging techniques to confirm the diagnosis.The patient’s lesions were determined as PC-ALCL,characterized by necrosis,chronic inflammation,and a distinct immunophenotypic profile,including CD30,CD3,CD4,and EBER,CD56,MUM-1,Ki 67-positive in>80%of tumor cells,CD10,but negative for anaplastic lymphoma kinase,CD5,CD20,PAX-5,Bcl-2,Bcl-6,CD8,and CD15.Recurrence was not reported at the 6-month follow-up.CONCLUSION Accurate PC-ALCL differentiation from similar conditions is crucial for effective management and requires a multidisciplinary approach.

Relative frequency and survival of primary cutaneous lymphomas： a retrospective analysis of 98 patients

Background The World Health Organization and European Organization for Research and Treatment of Cancer （WHOEORTC） classification in 2005 promoted the comparisons of primary cutaneous lymphoma （PCL） subtypes between different countries.The relative frequency of PCL varied according to geography.The study aimed to analyze the relative frequency and survival of PCLs in China and to compare the data with the published results from other countries.Methods We analyzed 98 patients with PCLs over a 6-year period and reclassified them according to the most recent WHO-EORTC classification （2005）.Disease-specific survival rate and curves according to specific subtypes such as mycosis fungoides,lymphomatoid papulosis,and primary cutaneous peripheral T-cell lymphoma,unspecified was also calculated.Results The relative rate of PCL in China was distinct from those in Western countries.Our study showed a higher frequency of cutaneous T-and NK-cell lymphomas （CTCLs） （94%）,and a lower frequency of cutaneous B-cell lymphomas （CBCLs） （6%）.The 5-year survival rate of the total PCLs was 82%.There was no significant difference in the 5-year survival rate （P 〉0.05 by Log-rank test） between CTCL （80%） and CBCL （100%）.Conclusions The higher percentage of CTCL in China may provide a clue to further study the etiological factors of PCLs.Racial variations in factors such as HLA determinants may play a role in the development of CTCL.

T-CELL RECEPTOR GENE REARRANGEMENT ANALYSIS IN THE PRIMARY CUTANEOUS T-CELL LYMPHOMA

Object: The present paper is to evaluate the significance of T cell receptor (TCR) gene rearrange ments in primary cutaneous T cell lymphomas (PCTCL) as detected by analysis of Southern Blot (SBA) and polymerase chain reaction (PCR). Patients and Methods: Skin specimens and peripheral blood samples were taken from 44 patients with PCTCL, including 30 patients with mycosis fungoides (MF), 2 patients with Sezary's syndrome (SS), and 12 patients with PCTCL other than MF and SS (PNCTCL). 11 patients with a presumptive diagnosis of MF, 23 patients with lymphoproliferative dermatoses including lymphomatoid papulosis (LyP) and 8 patients with benign cutaneous lymphoid infiltrates were simultaneously studied by the amplification of junctional V (variable) J (joining) sequences of the rearranged TCRγ genes by PCR(TCRγPCR) and the analysis of TCRb chain genes by SBA(TCRβSBA) for detection of clonal gene rearrangements (GR). One lymph node specimen of a case with MF IIA was also detected by TCRγ PCR and TCRβSBA. Results: In MF, GR were detected by TCRγPCR and TCRβSBAb in 83.3 85.7% and 66.7% 71.4% of skin specimens of cases IIA IIB and in 57.1% 70.0% and 14.3% 10.0% of those of cases IA IB, respectively. GR were seen in 66.7% 71.4% and 33.3% 43.0.% of blood samples of cases IIA IIB, and 42.9% 40.0% and 0 10.0% of those of cases IA IB, respectively. GR was confirmed by TCRγ PCR and TCRβSBA in one lymph node showing dermato pathic lymphadenopathy of a case with MF IIA. In 11 patients of clinically suspected MF, GR were present in skin specimens of 5 cases (45.4%) and in blood samples of 3 cases ( 27.3% ) by TCRγ PCR. In PNCTCL, GR were found in 9 skin specimens (90.0%) from 10 patients detected by TCRγ PCR and in 6 skin specimens (75.0%) from 8 patients detected by TCRβSBA. GR were also seen in 6 blood samples (72.8%) from 11 patients detected by TCRγ PCR, and in 7 blood samples (70.0%) from 10 patients by TCRβSBA. In SS and LyP, GR were detected by TCRγ PCR and TCRβSBA in each of the two skin specimens of two cases with LyP and in each of the two blood samples of two cases with SS. GR were seen in one skin specimen of one case with SS and one blood sample of one case with LyP detected by TCRγPCR. Conclusions: This study demonstrated that TCRγ PCR is a rapid, more sensitive tool than TCRβSBA, can be used in the analysis of T cell clonality in skin, lymph node and blood samples of patients with PCTCL and indicated that this method forms a useful supplement to other methods for diagnosis of early and suspected MF, confirmation of PNCTCL and determination of extracutaneous involvement of lymph node and blood.

Chidamide combined with traditional chemotherapy for primary cutaneous aggressive epidermotropic CD8+cytotoxic T-cell lymphoma:A case report

BACKGROUND Traditional chemotherapy has benefited many patients with non-Hodgkin's lymphoma,but results in a very poor response in patients with rare lymphomas or refractory lymphomas.Previous studies have shown that chidamide has potential anti-lymphoma activity and reverses lymphoma cell chemoresistance to increase the chemosensitivity of lymphoma cells to traditional chemotherapy.CASE SUMMARY A 14-year-old boy was admitted to our hospital with a 5-d history of generalized erythema,papules,and blisters.Initially,the disease was refractory to potent antiallergic and anti-infective treatment,and his condition progressively worsened.Skin biopsy revealed primary cutaneous aggressive epidermotropic CD8+cytotoxic T-cell lymphoma.Considering that the disease is extremely rare in clinical practice,existing case reports have shown poor efficacy with traditional chemotherapy alone.We recommend chidamide combined with traditional chemotherapy for treatment.The regimen was as follows:Chidamide 30 mg/biw,cyclophosphamide 1100 mg/d1,pirarubicin 70 mg/d1,vincristine 2 mg/d1,dexamethasone 20 mg/d1-5,etoposide 100 mg/d1-5,in a 21 d cycle.The treatment effect was considerable,and complete remission was achieved after 4 cycles of treatment,after which the patient completed a total of 6 cycles of treatment.Subsequently,the patient regularly took chidamide 20 mg/biw as maintenance therapy for 1 year.To date,the patient has been disease-free for 3 years.CONCLUSION This case suggests that the combination of chidamide and traditional chemotherapy is effective in primary cutaneous aggressive epidermotropic CD8+cytotoxic T-cell lymphoma.

Marginal zone lymphoma with severe rashes: A case report

BACKGROUND Marginal zone lymphoma(MZL)is an indolent subtype of non-Hodgkin lymphoma(NHL),which is rare clinically with severe rashes as the initial symptom.CASE SUMMARY This study reports a case of MZL with generalized skin rashes accompanied by pruritus and purulent discharge.First-line treatment with rituximab combined with zanubrutinib had poor effects.However,after switching to obinutuzumab combined with zanubrutinib,the case was alleviated,and the rashes disappeared.CONCLUSION For patients with advanced stage MZL not benefiting from type I anti-CD20 monoclonal antibody(mAb)combination therapy,switching to a type II anti-CD20 mAb combination regimen may be considered.This approach may provide a new perspective in the treatment of MZL.