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.

Bendamustine and rituximab as frontline therapy in extranodal marginal zone lymphoma:a single-institution experience

Extranodal marginal zone lymphoma(EMZL)encompasses 70%of cases of marginal zone lymphoma.Frontline bendamustine and rituximab(BR)were derived from trials involving other indolent non-Hodgkin’s lymphomas.Only one trial has evaluated frontline BR prospectively in EMZL.This retrospective study reports outcomes among EMZL patients receiving frontline BR.Twenty-five patients were included with a median age of 69 years(40–81).Five(20.0%)patients had stage Ⅰ/Ⅱ disease,and 20(80.0%)had stage Ⅲ/Ⅳ disease.The median number of cycles was 6.0(3.0–6.0).Maintenance rituximab was administered to 10(41.7%)individuals.Overall response rate(ORR)was 100.0%(60.0%complete response,40.0%partial response).Medians of overall survival and progression-free survival were not reached.The estimated 2-year progression-free survival was 85.2%and overall survival was 100.0%.Four(16.6%)patients had infections related to treatment;3(12.0%)transformed to diffuse large B-cell lymphoma;5(20.8%)had a relapse or progression of EMZL;and 3(12.0%)died unrelated to BR.BR is an efficacious and well-tolerated front-line regimen for EMZL with response data consistent with existing literature.

Transformation of marginal zone lymphoma into high-grade B-cell lymphoma expressing terminal deoxynucleotidyl transferase:A case report

BACKGROUND High-grade B-cell lymphoma(HGBL)is an unusual malignancy that includes myelocytomatosis viral oncogene(MYC),B-cell lymphoma-2(BCL-2),and/or BCL-6 rearrangements,termed double-hit or triple-hit lymphomas,and HGBL-not otherwise specific(HGBL-NOS),which are morphologically characteristic of HGBL but lack MYC,BCL-2,or BCL-6 rearrangements.HGBL is partially transformed by follicular lymphoma and other indolent lymphoma,with few cases of marginal zone lymphoma(MZL)transformation.HGBL often has a poor prognosis and intensive therapy is currently mainly advocated,but there is no good treatment for these patients who cannot tolerate chemotherapy.CASE SUMMARY We reported a case of MZL transformed into HGBL-NOS with TP53 mutation and terminal deoxynucleotidyl transferase expression.Gene analysis revealed the gene expression profile was identical in the pre-and post-transformed tissues,suggesting that the two diseases are homologous,not secondary tumors.The chemotherapy was ineffective and the side effect was severe,so we tried combination therapy including venetoclax and obinutuzumab.The patient tolerated treatment well,and reached partial response.The patient had recurrence of hepatocellular carcinoma and died of multifunctional organ failure.He survived for 12 months after diagnosis.CONCLUSION Venetoclax combined with obinutuzumab might improve the survival in some HGBL patients,who are unsuitable for chemotherapy.

Sclerotic marginal zone lymphoma:A case report

BACKGROUND Marginal zone lymphoma(MZL)is an indolent non-Hodgkin B cell lymphoma with various architectural pattern including perifollicular,follicular colonization,nodular,micronodular,and diffuse patterns.A sclerotic variant has not been previously reported and represents a diagnostic pitfall.CASE SUMMARY A 66-year-old male developed left upper extremity swelling.Chest computed tomography(CT)in September 2020 showed 14 cm mass in left axilla.Needle core biopsy of axillary lymph node showed sclerotic tissue with atypical B lymphoid infiltrate but was non-diagnostic.Excisional biopsy was performed for diagnosis and showed extensive fibrosis and minor component of infiltrating B cells.Flow cytometry showed a small population of CD5-,CD10-,kappa restricted B cells.Monoclonal immunoglobulin heavy chain and light chain gene rearrangement were identified.Upon being diagnosed with MZL,patient was treated with rituximab,cyclophosphamide,doxorubicin,vincristine,and prednisone and achieved complete remission by positron emission tomography/CT.CONCLUSION This is an important case report because by morphology this case could have easily been overlooked as non-specific fibrosis with chronic inflammation representing a significant diagnostic pitfall.Moreover,this constitutes a new architectural pattern.While sclerotic lymphomas have rarely been described(often misdiagnosed as retroperitoneal fibrosis),we do not know of any cases describing this architectural presentation of MZL.

Splenectomy with chemotherapy vs surgery alone as initial treatment for splenic marginal zone lymphoma

AIM： To evaluate the clinical characteristics of splenic marginal-zone lymphoma （SMZL） following antigen expression and the influence of therapeutic approaches on clinical outcome and overall survival （OS）.METHODS： A total of 30 patients with typical histological and immunohistochemical SMZL patterns were examined. Splenectomy plus chemotherapy was applied in 20 patients, while splenectomy as a single treatment-option was performed in 10 patients. Prognostic factor and overall survival rate were analyzed.RESULTS： Complete remission （CR） was achieved in 20 （66.7%）, partial remission （PR） in seven （23.3%）, and lethal outcome due to disease progression occurred in three （10.0%） patients. Median survival of patients with a splenectomy was 93.0 mo and for patients with splenectomy plus chemotherapy it was 207.5 mo （Log rank = 0.056, P 〉 0.05）. Time from onset of first symptoms to the beginning of the treatment （mean 9.4 too） was influenced by spleen dimensions, as measured by computerized tomography and ultra-sound （t = 2.558, P = 0.018）. Strong positivity （＋＋＋） of CD20 antigen expression in splenic tissue had a positive influence on OS （Log rank = 5.244, P 〈 0.05）. The analysis of factors interfering with survival （by the Kaplan-Meier method） revealed that gender, general symptoms, clinical stage, and spleen infiltration type （nodular vs diffuse） had no significant （P 〉 0.05） effects on the OS. The expression of other antigens （immunohistochemistry） also had no effect on survival-rate, as measured by a χ^2 test （P 〉 0.05）.CONCLUSION： Initial splenectomy combined with chemotherapy has been shown to be beneficial due to its advanced remission rate/duration; however, a larger controlled clinical study is required to confirm our findings.

Primary mucosal-associated lymphoid tissue extranodal marginal zone lymphoma of the bladder from an imaging perspective: A case report

BACKGROUND Mucosal-associated lymphoid tissue extranodal marginal zone(MALT)lymphoma is a low-grade tumor that rarely occurs in the urinary bladder.There is currently no consensus on the common imaging findings or most appropriate treatment in MALT lymphoma in the urinary bladder due to the limited number of reports.CASE SUMMARY A 48-year-old woman was admitted to the hospital with a 1-year history of macroscopic hematuria.Imaging showed a large homogeneous mass with an unclear boundary and an irregular morphology in the bladder.The mass had an abundant blood supply.For further diagnosis,transurethral cystoscopic biopsy and bone marrow biopsy was performed,and the patient was finally diagnosed with primary MALT lymphoma of the bladder.R-CHOP chemotherapy was carried out.After three cycles of chemotherapy,the mass disappeared and the bladder wall thickness was only 4 mm,which indicated excellent therapeutic response to the chemotherapy.To date,the patient remains asymptomatic and she visits our hospital regularly for the completion of the remaining chemotherapy cycles.CONCLUSION Primary MALT lymphoma of the bladder is rare,and there are certain characteristics in the ultrasonographic findings.Imaging findings play an important role in evaluating the therapeutic efficacy and are critical during long-term follow-up after therapy.

Secondary light chain amyloidosis with Waldenstr?m’s macroglobulinemia and intermodal marginal zone lymphoma:A case report

BACKGROUND The co-existence of Waldenstr?m’s macroglobulinemia(WM) with internodal marginal zone lymphoma(INMZL) is rare and often associated with poor prognosis.CASE SUMMARY We present a Chinese female patient who developed secondary light chain amyloidosis due to WM and INMZL and provides opinions on its systemic treatment.A 65-year-old woman was diagnosed with WM 6 years ago and received Bruton tyrosine kinase inhibitor monotherapy for two years.Her INMZL was confirmed due to left cervical lymphadenopathy.The patient presented with oedema in both lower limbs one year ago,and was diagnosed with secondary light chain amyloidosis.Treatment with the BC regimen(rituximab 375 mg/m~2 monthly for 6-8 courses,and bendamustine 90 mg/m~2 per day × 2,monthly for six courses) was initiated,but not tolerated due to toxic side effects.Bortezomibbased therapy was given for two months,including bortezomib,dexamethasone,and zanubrutinb.Oedema in both lower limbs was relieved and treatment efficacy was evaluated as partial remission.CONCLUSION A detailed clinical evaluation and active identification of the aetiology are recommended to avoid missed diagnosis and misdiagnosis.

Role of Helicobacter pylori in gastric mucosa-associated lymphoid tissue lymphomas

Mucosa-associated lymphoid tissue (MALT) lymphoma is an indolent extranodal marginal zone B-cell lymphoma, originating in acquired MALT that is induced in mucosal barriers as part of a normal adaptive immune response to a chronic immunoinflammatory stimulus, most notably chronic infection by Helicobacter pylori (H. pylori). This antigenic stimulation initially leads to lymphoid hyperplasia; the acquisition of additional genetic aberrations culminates in the activation of intracellular survival pathways, with disease progression due to proliferation and resistance to apoptosis, and the emergence of a malignant clone. There are descriptions of MALT lymphomas affecting practically every organ and system, with a marked geographic variability partially attributable to the epidemiology of the underlying risk factors; nevertheless, the digestive system (and predominantly the stomach) is the most frequently involved location, reflecting the gastrointestinal tract’s unique characteristics of contact with foreign antigens, high mucosal permeability, large extension and intrinsic lymphoid system. While early-stage gastric MALT lymphoma can frequently regress after the therapeutic reversal of the chronic immune stimulus through antibiotic eradication of H. pylori infection, the presence of immortalizing genetic abnormalities, of advanced disease or of eradication-refractoriness requires a more aggressive approach which is, presently, not consensual. The fact that MALT lymphomas are rare neoplasms, with a worldwide incidence of 1-1.5 cases per 105 population, per year, limits the ease of accrual of representative series of patients for robust clinical trials that could sustain informed evidence-based therapeutic decisions to optimize the quality of patient care.

Management of gastric mucosa-associated lymphoid tissue lymphoma in patients with extra copies of the MALT1 gene

AIM To identify the clinical features of gastric mucosaassociated lymphoid tissue(MALT) lymphoma with extra copies of MALT1.METHODS This is a multi-centered,retrospective study. We reviewed 146 patients with MALT lymphoma in the stomach who underwent fluorescence in situ hybridization analysis for t(11;18) translocation. Patients were subdivided into patients without t(11;18) translocation or extra copies of MALT1(Group A,n = 88),patients with t(11;18) translocation(Group B,n = 27),and patients with extra copies of MALT1(Group C,n = 31). The clinical background,treatment,and outcomes of each group were investigated.RESULTS Groups A and C showed slight female predominance,whereas Group B showed slight male predominance. Mean ages and clinical stages at lymphoma diagnosis were not different between groups. Complete response was obtained in 61 patients in Group A(69.3%),22 in Group B(81.5%),and 21 in Group C(67.7%). Helicobacter pylori(H. pylori) eradication alone resulted in complete remission in 44 patients in Group A and 13 in Group C. In Group B,14 patients underwent radiotherapy alone,which resulted in lymphoma disappearance. Although the difference was not statistically significant,event-free survival in Group C tended to be inferior to that in Group A(P = 0.10).CONCLUSION Patients with t(11;18) translocation should be treated differently from others. Patients with extra copies of MALT1 could be initially treated with H. pylori eradication,similar to patients without t(11;18) translocation or extra copies of MALT1.

Classification, diagnosis, and management of conjunctival lymphoma

Lymphoma is a malignant lymphoproliferative tumor that can involve the conjunctiva.Approximately 5–15%of all extranodal lymphomas are found in the ocular adnexal region,with approximately 25%of those involving the conjunctiva.Ninety-eight percent of conjunctival lymphomas arise from B-lymphocytes.The most common subtype of conjunctival lymphoma is extranodal marginal zone lymphoma(80%),followed by follicular lymphoma(8%),diffuse large B-cell lymphoma(3%)and mantle cell lymphoma(3%).Natural killer and T cells(NK/T)are rare causes of lymphoma.While most conjunctival lymphomas are localized to the ocular adnexa at the time of presentation,systemic examination and management are of key importance in the long-term care of the patient.This review outlines the classification,etiology,presentation,diagnosis,and management of conjunctival lymphoma.The novel use of high resolution optical coherence tomography,both as a diagnostic tool and as a means for ongoing evaluation during treatment,is illustrated.Treatment options discussed include external beam radiation,chemotherapy,immunotherapy,antibiotic therapy,and combination regimens.Future investigation of the etiology and pathogenesis of conjunctival lymphoma is expected to reveal opportunities for innovative and individualized therapeutic agents.Collaboration between multiple disciplines is key in the advancement of the field.

A rare case of B-lymphoproliferative disorder with villous lymphocytes harboring t（8;14）（q24;q32） translocation

Splenic lymphoma with villous lymphocytes （SLVL） or splenic marginal zone lymphoma with circulating villous lymphocytes is rare, and prolymphocytic transformation of SLVL is rarer. At present, only one case of SLVL with t（8;14）（q24;q32） translocation has been reported. In this study, we report a case of B- lymphoproliferative disorder with villous lymphocytes harboring t（8;14）（q24;q32） chromosome translocation that we inclined to SLVL with a prolymphocytic transformation. A 73-year-old female showed marked hepatosplenomegaly and high lymphocytosis （lymphocytes 〉 200 × 10^9/L）. The abnormal lymphocytes had short coarse villi and round nuclei with prominent nucleoli. The immunophenotypes showed CD19^＋, CD20^＋, HLA- DR^＋, CD22^＋, CD5^＋, Kappa^＋, CD25^dim, CD71^dim, Lambda, CD7, CD10-, CD23 , CD34, CD33, CD13 , CD14, CDll7, CD64, CD103, and CD11c-. The karyotype showed complex abnormality： 46XX,＋ 3,-10, t（8;14）（q24; q32）lll]/46XX[9]. The cytoplasmic projection, immunological characteristics, and trisomy 3 chromosome abnormality supported the diagnosis of SLVL. However, the presence of prominent nucleoli and high lymphocytosis suggested prolymphocytic transformation, probably as a result of t（8,14） chromosome translocation. In this report, we described an unusual case of B-lymphoproliferative disorder with villous lymphocytes harboring t（8;14）（q24;q32） translocation, which could provide help in the diagnosis and differential diagnosis of B-lymphocytic proliferative diseases.