[Clinicopathological and genetic analysis of interstitial disease-like pulmonary intravascular large B cell lymphoma].

Objective: To investigate the clinicopathological features and genetic mutation status of pulmonary intravascular large B cell lymphoma. Methods: The clinicopathological data of eight patients diagnosed with pulmonary intravascular large B cell lymphoma, from April 2018 to May 2023, were retrospectively analyzed. The genetic profile of six patients was detected via next-generation sequencing (NGS) and followed up. Results: All patients included one male and seven females, with a median age of 64 years (ranging from 45 to 66 years). Respiratory symptoms were the most common (7 cases), B symptoms in two cases, hemophagocytic syndrome in two cases. Multiple diffuse ground-glass opacities in both lungs were observed based on the high-resolution chest CT scan. Six cases of mild to moderate ventilation or diffusion dysfunction were observed based on the pulmonary function tests. Moreover, two cases of hypoxemia and two cases with type Ⅰ respiratory failure were recorded. The serum lactate dehydrogenase level increased (7/8), ß2-MG level increased (2/8), neuron-specific enolase level increased (7/8), total number of peripheral blood lymphocytes decreased (7/8), and clinical stages were all stage Ⅳ. The neoplastic lymphoid cells were lodged in the lumina of venules and capillaries of the alveolar septum; the tumor cells were large, with prominent nucleoli and frequent mitotic figures. The malignant cells were detected in the extravascular surrounding lung tissue in all cases. The tumor cells expressed mature B cell-associated antigens CD20 and CD79a, and the vascular endothelial markers CD31 and CD34 showed that the tumor cells were filled in the blood vessels, infiltrated blood vessel walls, and perivascular areas. One case was germinal center-type, seven cases were non-germinal center-type, two cases were double-expressing lymphoma, and all cases were EBER-negative. Furthermore, the top five genes with mutation frequencies detected by NGS were MYD88 (5/6), PIM1 (5/6), CD79B (4/6), TCF3 (4/6), and TP53 (3/6). Of the eight cases, seven patients received R-CHOP-based chemotherapy, six cases had complete remission after chemotherapy, one case died, and one case was lost to follow-up. Conclusions: Pulmonary vascular large B cell lymphoma is rare, which shares similar patterns with interstitial lung disease on imaging. Transbronchial lung biopsy is an effective method to confirm the diagnosis. Immunochemotherapy with BTK inhibitors can provide a survival advantage for patients in the future based on molecular typing.

Long-term Outcomes and Prognostic Factors of Gastric MALT Lymphoma.

PURPOSE: This study aimed to evaluate the long-term prognosis of patients with gastric mucosa-associated lymphoid tissue (MALT) lymphoma, including overall survival (OS), remission, and factors associated with an aggressive disease course. MATERIALS AND METHODS: Medical records of 153 patients diagnosed with gastric MALT lymphoma between 2013 and 2020 were retrospectively reviewed. Patients experiencing relapse, progression, high-grade transformation, or residual diseasewere included in the aggressive group and were compared with those in the indolent group. Additionally, the endoscopic findings of Helicobacter pylori-negative patients were reviewed. RESULTS: Patient characteristics were as follows: mean age (56.9±11.2 years), sex (male, 51.0%), H. pylori infection (positive, 79.7%), endoscopic location (distal, 89.5%), endoscopic feature (superficial, 89.5%), clinical stage (stage I, 92.8%), invasion depth by endoscopic ultrasound (mucosa, n=115, 75.7%), and bone marrow result (no involvement, n=77, 100.0%). The median follow-up period was 59 months (mean, 61; range, 36-124) and the continuous remission period (n=149) was 51 months (mean, 50; range, 3-112). The 5-year survival rate was 97.7% while the 5-year continuous remission was 88.3%. Factors associated with the patients in the aggressive group were old age, sex(male), and clinical stage II or higher. H. pylori-negative patients' endoscopy revealed a high incidence of atrophic gastritis in the antrum. CONCLUSIONS: The long-term prognosis of gastric MALT lymphoma appears indolent and is indicated by the 5-year OS and continuous remission rates. Aggressive disease courses are associated with old age, sex (male), and clinical stage II or higher, but are not related to OS.

[A consistency comparison between next-generation sequencing and the FISH method for gene rearrangement detection in B-cell lymphomas].

Objective: To compare the consistency of lymphoma multigene detection panels based on next-generation sequencing (NGS) with FISH detection of B-cell lymphoma gene rearrangement. Methods: From January 2019 to May 2023, fusion genes detected by lymphoma-related 413 genes that targeted capture sequencing of 489 B-cell lymphoma tissues embedded in paraffin were collected from Henan Cancer Hospital, and the results were compared with simultaneous FISH detection of four break/fusion genes: BCL2, BCL6, MYC, and CCND1. Consistency was defined as both methods yielding positive or negative results for the same sample. The relationship between fusion mutation abundance in NGS and the positivity rate of cells in FISH was also analyzed. Results: Kappa consistency analysis revealed high consistency between NGS and FISH in detecting the four B-cell lymphoma-related gene rearrangement (P<0.001 for all) ; however, the detection rates of positive individuals differed for the four genes. Compared with FISH, NGS demonstrated a higher detection rate for BCL2 rearrangement, a lower detection rate for BCL6 and MYC rearrangement, and a similar detection rate for CCND1 rearrangement. No correlation was found between fusion mutation abundance in NGS and the positivity rate of cells in FISH. Conclusions: NGS and FISH detection of B-cell lymphoma gene rearrangement demonstrate overall good consistency. NGS is superior to FISH in detecting BCL2 rearrangement, inferior in detecting MYC rearrangement, and comparable in detecting CCND1 rearrangement.

B-cell lymphoma-2 phosphorylation at Ser70 site-related autophagy mediates puerarin-inhibited the apoptosis of MC3T3-E1 cells during osteoblastogenesis.

OBJECTIVE: To explore the relationship between autophagy and apoptosis regulated by puerarin during osteoblastogenesis. METHODS: In this study, the effects of puerarin on the autophagic activity and apoptosis level of osteoblast precursors (MC3T3-E1 cells) was observed. Subsequently, the roles of puerarin on B-cell lymphoma-2 (Bcl-2) phosphorylation at different sites in osteoblast precursors were observed. The effect of puerarin on the interaction between Bcl-2 and autophagy regulatory molecule or pro-apoptotic molecule was also investigated using Co-immunoprecipitation assays. In addition, the effect of puerarin on mitochondrial membrane potential of osteoblast precursors was also identified by mitochondrial membrane potential fluorescence probe assays. RESULTS: Our results showed that puerarin can promote the autophagic activity and apoptosis level of MC3T3-E1 cells. In addition, puerarin promoted Bcl-2 phosphorylation at Ser70 site, and the dissociation of Bcl-2-Beclin1 complex. Moreover, puerarin could enhance the binding of Bcl-2-Bcl-2-Associated X (Bax) complex in MC3T3-E1 cells. Furthermore, puerarin increased the mitochondrial membrane potential of MC3T3-E1 cells. CONCLUSIONS: Therefore, puerarin promotes Beclin1 into autophagy flux through Bcl-2 phosphorylation at Ser70, thereby enhancing autophagy of osteoblast precursors, which mediates its anti-apoptotic role during osteoblastogenesis. Furthermore, the dissociation of Bcl-2-Beclin1 complex is conducive to the binding of Bcl-2-Bax complex, which resists the apoptosis of osteoblast precursors viathe increased mitochondrial membrane potential.

Causa inusual de obstrucción traqueal: linfoma de células B no Hodgkin / Unusual cause of tracheal obstruction: B cell non-Hodgkin lymphoma

Las neoplasias de la tráquea son poco comunes, siendo más infrecuente aún el linfoma de células B no Hodgkin a este nivel. La presentación clínica de esta entidad es muy variable y sus síntomas son muy inespecíficos, simulando varias enfermedades, generando así un diagnóstico tardío. Actualmente se dispone de varias ayudas diagnósticas que permiten descartar otras patologías y confirmar la neoplasia traqueal, además la biopsia es fundamental para llegar al diagnóstico final y así instaurar el manejo en estadios tempranos, para impactar en la morbimortalidad. A continuación presentamos el caso de una paciente con linfoma de células B no Hodgkin como causa de obstrucción traqueal.

Tracheal neoplasms are uncommon, with non-Hodgkin B-cell lymphoma being even rarer at this level. The clinical presentation of this entity is highly variable and its symptoms are very non-specific, simulating several diseases, thus generating a late diagnosis. Currently, there are several diagnostic aids that allow us to rule out other pathologies and confirm tracheal neoplasia, in addition, biopsy is essential to reach the final diagnosis and thus establish management in early stages to impact morbidity and mortality. We present the case of a patient with non-Hodgkin's B-cell lymphoma as a cause of tracheal obstruction.

Improvement of Severe Colon Stricture after Rituximab Therapy for Concomitant Mucosa-associated Lymphoid Tissue Lymphoma in a Patient with Ulcerative Colitis.

Colorectal strictures are uncommon in patients with ulcerative colitis (UC). An extranodal marginal zone B-cell lymphoma of mucosa- associated lymphoid tissue (MALT) lymphoma is rarely involved in the colon but may be associated with inflammatory bowel diseases. A 41-year-old female with a six-year history of UC presented with a severe stricture of the sigmoid colon that prevented the passage of a colonoscope. A histological examination revealed non-specific inflammation and fibrosis without dysplasia or cancer. Despite conventional treatment, including mesalazine and azathioprine for one year after that visit, the stricture persisted. In addition, diffuse, edematous exudative inflammation and multiple shallow ulcers were observed in the distal rectum, revealing a MALT lymphoma testing positive for CD20, CD43, CD5, and Bcl-2, but negative for CD3, CD10, CD23, and cyclin-D1. Four weekly doses of rituximab were administered. Follow-up colonoscopy performed one month after treatment revealed slight improvement in the rectal lesion without remnant histological evidence of a MALT lymphoma. In addition, the stricture showed marked improvement, and the colonoscope could pass easily through the stricture site. This is the first case report on an improvement of a severe sigmoid colon stricture in a patient with UC after rituximab treatment for a concomitant rectal MALT lymphoma.

[Gray zone lymphoma: five cases report and literature review].

Objective: To investigate the clinical and pathological features, treatment, and prognosis of gray zone lymphoma (GZL) . Methods: From July 2, 2013, to February 10, 2021, the clinical and pathological features, treatment, and outcomes of five patients with GZL at the Blood Diseases Hospital, Chinese Academy of Medical Sciences were studied retrospectively. Results: There were one male and 4 females, with a median age of 28 (16-51) years at diagnosis. Four patients had mediastinal (thymic) involvement, two of which had superior vena cava obstruction syndrome, and 3 patients had extra-nodal involvement. There was one case with a limited Ann Arbor stage and 4 cases with a progressive stage. Three patients had cHL-like pathomorphology with scattered Hodgkin-like cells, strongly positive for CD20, positive for CD30, and CD15 was negative; the other two patients had both cHL and DLBCL morphology, with some areas resembling Hodgkin cells and some areas resembling immunoblasts, strongly positive for CD30, and CD15 but negative CD20. Two patients were treated with cHL-like regimens for induction and achieved only partial remission; after salvage therapy with enhanced DLBCL-like regimens, all achieved complete remission (CR) . Three patients were treated with enhanced DLBCL-like immunochemotherapy regimens for induction, and two patients were effective, one of whom achieved CR. Four patients who did not achieve CR were given second or third-line salvage therapy, and all of them recovered. One patient lost parity, one died of disease progression at 35.9 months after diagnosis, and the remaining three maintained sustained remission. Conclusions: GZL is uncommon, usually affects younger patients, is mediastinal and is diagnosed using path morphology and immunophenotype. Patients with newly diagnosed GZL appear to be more sensitive to DLBCL-like immunochemotherapy regimens; relapsed or refractory patients were tended with non-cross-resistant combination chemotherapy or with new drugs.

ASIA and BIA-ALCL as adverse reactions to silicone breast implants / ASIA e BIA-ALCL como reações adversas aos implantes mamários de silicone

Adjuvant-induced autoimmune/inflammatory syndrome leads to capsular contracture and fibrosis from the oxidation that takes place in silicone. Anaplastic large cell lymphoma occurs through the development of a seroma, with the formation of a periprosthetic effusion, or through the infiltration of the condition itself. To analyze these conditions, a review of the literature was carried out on the symptoms and pathophysiology of the autoimmune/inflammatory syndrome induced by adjuvants and anaplastic large cell lymphoma, searched using the terms "ASIA breast silicone," "Lymphoma," "Adjuvants" "Immunologic" " Breast Implants" on the PubMed platform. Analyzing the data obtained, it was noted that the symptoms of the autoimmune/inflammatory syndrome induced by adjuvants are nonspecific, such as fatigue, myalgia, arthralgia, morning stiffness, and night sweats, and therefore need attention. Anaplastic large cell lymphoma presents with breast pain, periprosthetic effusion, and palpable mass, among other characteristics. Because of these aspects, it is concluded that a good investigation should be carried out when nonspecific symptoms appear, regardless of the time the surgery was performed since these complications can occur years later.

A síndrome autoimune/inflamatória induzida por adjuvantes leva à contratura capsular e fibrose pela oxidação que acontece no silicone. O linfoma anaplásico de grandes células ocorre através do desenvolvimento de um seroma, com a formação de derrame periprotético ou por uma infiltração da própria afecção. Para análise destes acometimentos, foi realizada uma revisão da literatura acerca da sintomatologia e fisiopatologia da síndrome autoimune/inflamatória induzida por adjuvantes e linfoma anaplásico de grandes células, pesquisada através dos termos "ASIA breast silicone" "Lymphoma" "Adjuvants" "Immunologic" "Breast Implants" na plataforma PubMed. Analisando os dados obtidos, notou-se que os sintomas da síndrome autoimune/inflamatória induzida por adjuvantes são inespecíficos, como fadiga, mialgia, artralgia, rigidez matinal e suores noturnos, e, portanto, necessitam de atenção. Já o linfoma anaplásico de grandes células se apresenta com dor mamária, derrame periprotético, massa palpável, dentre outras características. Em vista destes aspectos, conclui-se que uma boa investigação deve ser realizada ao surgirem sintomas inespecíficos, independentemente do tempo que a cirurgia foi realizada, uma vez que estas complicações podem ocorrer anos após a cirurgia.

Diagnosis and management of rare paediatric Non-Hodgkin lymphoma.

Mature B-cell lymphomas, (B- or T-cell) lymphoblastic lymphomas (LBL), and anaplastic large cell lymphoma (ALCL) correspond to about 90% of all non-Hodgkin lymphoma (NHL) cases occurring in children and adolescents. The remaining 10% encompass a complex group of entities characterized by low/very low incidences, paucity of knowledge in terms of underlying biology in comparison to their adult counterparts, and consequent lack of standardization of care, information on clinical therapeutic efficacy and long-term survival. At the Seventh International Symposium on Childhood, Adolescent and Young Adult NHL, organized on October 20-23, 2022, in New York City, New York, US, we had the opportunity to discuss clinical, pathogenetic, diagnostic, and treatment aspects of certain subtypes of rare B- or T-cell NHL and they will be the topic of this review.

A Bizarre Progression Of Chronic Lymphocytic Leukaemia Into Diffuse Large B-Cell Lymphoma.

A 65-year-old male presented with complaints of weakness, lethargy, abdominal pain, and low-grade fever for the last few months. His examination revealed generalized lymphadenopathy and splenomegaly. A subsequent laboratory workup revealed atypical lymphoid cells with prominent double-bright positivity of CD19 and CD5 markers. Further investigations revealed deletion of the ATM (11q22.3) gene, and by other diagnostic factors, the patient was diagnosed with B-cell chronic lymphocytic leukaemia. Thus, treatment was initiated with oral chemotherapy followed by rituximab-bendamustine. After three weeks, he presented to the emergency room with a fever and worsening abdominal pain. On examination, massive splenomegaly was found. After stabilization, a bone marrow biopsy revealed findings which, in light of the clinical symptoms, were consistent with Richter's transformation of B-Cell chronic lymphocytic leukaemia into Diffuse large B-Cell Lymphoma.

Non-Hodgkin lymphoma mimicking acute leukemia: a report of six cases and review of the literature.

Aggressive subtypes of non-Hodgkin lymphoma may uncommonly be referred to clinical oncologists for treatment of acute leukemia, due to an elevated or rapidly rising white blood cell count (WBC), with circulating neoplastic cells that morphologically resemble leukemic blasts seen in acute myeloid or lymphoblastic leukemia. We describe six cases of non-Hodgkin lymphoma that mimicked acute leukemia and were identified in the pathology records of the Brigham and Women's Hospital. The patients were older adults (mean age 70 years), who presented with leukocytosis (mean 79.7 × 109/L) with circulating neoplastic cells (mean 57%), which mimicked leukemic blasts, thrombocytopenia, and anemia (4/6 patients). In each case, immunophenotypic analysis identified a population of mature B cells or mature T cells. We identified 15 additional cases of non-Hodgkin lymphoma in the literature that mimicked acute leukemia; considering all 21 cases, 11 had an appearance of acute lymphoblastic leukemia, 4 had an appearance of acute monocytic leukemia, and 6 had an appearance of acute leukemia unable to be further categorized. In general, patients exhibited poor overall survival. These cases illustrate the importance of comprehensive immunophenotypic analysis in the initial evaluation of hematolymphoid neoplasms, and that occasional cases of non-Hodgkin lymphomas can resemble acute leukemia at initial presentation.

[Treatment and prognosis of primary ocular adnexal extranodal marginal zone mucosa-associated lymphoid tissue lymphoma: a report from a single center].

Objective: This study aimed to see how different initial treatment regimens affected the long-term prognosis of patients with extranodal marginal zone mucosa-associated lymphoid tissue lymphoma confining to the ocular adnexal (OAML) . Methods: Between April 2008 and April 2019, 109 patients with initial mucosa-associated lymphoid tissue confining to ocular adnexal were evaluated and followed-up, and the prognosis of various initial treatment regimens were examined. Results: A total of 36 patients underwent complete surgical resection of the lesions, and 73 patients had residual lesions after surgery, of which 37 patients chose watchful waiting, and 36 patients chose treatment. The treatment regimen included local radiotherapy and systemic treatment (chemotherapy, immunochemotherapy, the combination of radiotherapy and chemotherapy, etc.) , and no serious toxic and side effects were observed in patients receiving systemic treatment. The median follow-up time was 61 (10-142) months. The 5-year and 10-year progression-free survival (PFS) of monocular involvement patients were 78.2% and 76.0% . The 5-year and 10-year PFS rates of patients with binocular involvement were 64.4% and 23.5%. There was significant diference in PFS between patients with monocular and binocular involvement (P=0.010) . Patients who received additional treatment had higher PFS than those patients in the watchful waiting group (P=0.046) . The 5-year PFS was 71.4% and 90.1% among patients in the watchful waiting group and those who received additional treatment, whereas the 10-year PFS was 63.5% and 75.1% , respectively. Patients with OAML were still a risk of disease progression after 5 years. Conclusions: Patients with binocular involvement OAML at the start of the disease had a poor prognosis, but treatment could reduce the risk of recurrence/progression. Systemic therapy is one of the first-line treatment options for patients with OAML, who require long-term monitoring.

[Dose-enhanced immunochemotherapy followed by first-line autologous peripheral blood stem cell transplantation for young patients with high-risk aggressive B-cell lymphoma: an efficacy and prognostic factor analysis].

Objective: This study aimed to determine the efficacy of dose-enhanced immunochemotherapy followed by autologous peripheral blood stem cell transplantation (ASCT) in young patients with newly diagnosed high-risk aggressive B-cell lymphoma. Methods: A retrospective study was conducted to examine the clinical and survival data of young patients with high-risk aggressive B-cell lymphoma who received dose-enhanced immunochemotherapy and ASCT as first-line treatment between January 2011 and December 2018 in Blood Diseases Hospital. Results: A total of 63 patients were included in the study. The median age range was 40 (14-63) years old. In terms of the induction therapy regimen, 52 cases received R-DA-EP (D) OCH, and the remaining 11 received R-HyperCVAD/R-MA. Sixteen (25.4% ) patients achieved partial response in the mid-term efficacy assessment, and ten of them were evaluated as complete response after transplantation. The median follow-up was 50 (8-112) months, and the 3-year progression-free survival (PFS) rate and overall survival (OS) rate were (83.9±4.7) % and (90.4±3.7) % , respectively. Univariate analysis demonstrated that age-adjusted international prognostic index ≥2 scores was a negative prognostic factor for OS (P=0.039) , and bone marrow involvement (BMI) was an adverse prognostic factor for OS (P<0.001) and PFS (P=0.001) . However, multivariate analysis confirmed that BMI was the only independent negative predictor of OS (P=0.016) and PFS (P=0.001) . Conclusions: The use of dose-enhanced immunochemotherapy in combination with ASCT as first-line therapy in the treatment of young, high-risk aggressive B-cell lymphoma results in good long-term outcomes, and BMI remains an adverse prognostic factor.

Tratamento cirúrgico exclusivo de linfoma B primário cutâneo da zona marginal: relato de caso / Exclusive surgical treatment of a primary cutaneous marginal zone lymphoma: a case report

Os linfomas B primários cutâneos (LBPCs) são linfomas não Hodgkin, de acometimento exclusivamente cutâneo, e representam 25% dos linfomas primários cutâneos. São divididos, conforme comportamento clínico, em indolentes e intermediários. O tratamento das formas indolentes inclui a cirurgia, a radioterapia e, em casos extensos, o rituximabe. Relata-se o caso de mulher de 57 anos, com placa única no braço esquerdo, com diagnóstico de LBPC da zona marginal, tratado com excisão com margens de segurança de 5mm, sem recidiva após 36 meses de seguimento. A cirurgia é uma alternativa terapêutica com bom resultado clínico, sem impacto na sobrevida livre da doença.

Primary cutaneous B-cell lymphomas are non-Hodgkin lymphomas presenting only in the skin and represent 25% of all primary cutaneous lymphomas. Based on their clinical behavior, they are classified into indolent and intermediate forms. Treatment of indolent forms includes surgery, radiotherapy, and, in extensive disease, rituximab. We report a case of a 57-year-old woman with a single nodule in the left arm treated with surgical excision with 5-mm security margins, without relapse after 36 months. Surgery is a therapeutic option in these lymphomas without compromising disease-free survival.

[Clinical features and prognosis of eight patients with splenic diffuse red pulp small B-cell lymphoma].

Objective: To investigate the clinical characteristics, response, and prognosis of splenic diffuse red pulp small B-cell lymphoma (SDRPL) . Methods: Eight cases of SDRPL were diagnosed and treated at Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, between May 2017 and April 2022. Data on the clinical features, laboratory results, bone marrow and spleen biopsy results, response, and prognosis were collected and analyzed. Results: The median age at diagnosis was 54 (42-69) years. Splenomegaly and lymphocytosis were present in all cases, and PET/CT revealed normal to slightly elevated splenic FDG uptake. All cases were in stage Ⅳ, with spleen, peripheral blood, and bone marrow but no proximal lymph nodes involved. The cytoplasm of neoplastic villous cells was abundant, and splenic pathology showed that small homogenous lymphocytes permeated the splenic sinus and splenic cord, and the white pulp atrophied. Immunohistochemistry was not typical, and B-cell markers including CD19, CD20 and CD79α were positive. After a median follow up of 35.5 (4-60) months, 7 cases were alive after splenectomy with or without chemoimmunotherapy. The patient with CCND3 P284A and MYC S146L mutation developed to B-cell prolymphocytic leukemia (B-PLL) 1 month after splenectomy and died at 16 months of follow-up. Conclusion: A rare indolent B-cell lymphoma that primarily affects the elderly, SDRPL. Most patients achieved long-term survival, but the prognosis of patients who progress to B-PLL was poor.

[Bendamustine monotherapy for Chinese patient treatment with relapsed or refractory B cell non-Hodgkin lymphoma: a phase â ¡, prospective, multicenter, single-arm study].

Objective: To evaluate the efficacy and safety of bendamustine monotherapy in Chinese patients with relapsed/refractory (R/R) B cell non-Hodgkin lymphoma (B-NHL) . Methods: This prospective, multicenter, open label, single-arm, phase Ⅱ study investigated bendamustine's efficacy and safety in Chinese patients with R/R B-NHL. A total of 78 patients with B-NHL in 11 hospitals in China from March 2012 to December 2016 were included, and their clinical characteristics, efficacy, and survival were analyzed. Results: The median age of all patients was 58 (range, 24-76) years old, and 69 (88.4% ) patients had stage Ⅲ/Ⅳ disease. 61 (78.2% ) patients were refractory to previous treatments. Patients received a median of 4 (range, 1-10) cycles of bendamustine treatment. The overall response rate was 61.5 (95% CI 49.8-72.3) % , the median response duration was 8.3 (95% CI 5.5-14.0) months, and the complete remission (CR) rate was 5.1 (95% CI 1.4-12.6) % . In the full analysis set, median progression-free survival (PFS) and median OS were 8.7 (95% CI 6.7-13.2) months and 25.5 months (95% CI 14.2 months to not reached) , respectively, after a median follow-up of 33.6 (95% CI 17.4-38.8) months. Lymphopenia (74.4% ) , neutropenia (52.6% ) , and leukopenia (39.7% ) , thrombocytopenia (29.5% ) and anemia (15.4% ) were the most common grade 3-4 hematologic adverse events (AE) . The most frequent non-hematologic AEs included nausea (43.6% ) , vomiting (33.3% ) , and anorexia (29.5% ) . Univariate and multivariate analysis showed that <4 cycles of bendamustine treatment was a poor prognostic factor for PFS (P=0.003) , and failure to accept fludarabine containing regimen was a poor prognostic factor for OS (P=0.009) . Conclusion: Bendamustine monotherapy has good efficacy and safety in the treatment of patient with R/R B-NHL.

Leukemia Cutis As the Initial Manifestation of Chronic Lymphocytic Leukemia Progression.

Chronic lymphocytic leukemia (CLL) is a malignant proliferation of monoclonal mature B-cells in peripheral blood. Leukemia cells can commonly spread from the blood to other sites such as the lymph nodes, liver, and spleen. However, contrary to T-cell lymphomas that can involve the skin, CLL metastasis to the skin is unusual and is rarely the first manifestation of systemic disease. When leukemia cells invade the skin, it is termed leukemia cutis. Furthermore, multiple skin morphologies can be present in leukemia cutis making diagnosis challenging. Likewise, it can be mistaken for other common etiologies such as drug or substance allergy, infection, and scabies, among others. We herein present a case of CLL with leukemia cutis as the initial manifestation of systemic disease. The initial punch biopsy results were non-specific for inflammatory changes, but a subsequent biopsy revealed findings confirming leukemia cutis. This case not only demonstrates that identifying malignant skin manifestations in a timely manner and treating them is essential, as it improves the quality of life and survival, but also demonstrates that leukemia cutis can be a dynamic disease where multiple biopsies may be needed to confirm the diagnosis, as histopathology can change over time.

Clinical Features and Prognosis of Patients with Primary Intestinal B-cell Lymphoma Treated with Chemotherapy with or without Surgery.

Background/Aims: This multicenter study reviewed the clinical features and prognosis according to the primary site of involvement and the treatment modality in patients with B-cell primary intestinal lymphoma (PIL). Methods: Among 125 consecutive patients diagnosed with PIL, 100 patients were analyzed. Results: The median age was 59 years, and the male to female ratio was 1.86:1. Diffuse large B-cell lymphoma (66/100, 66.0%) was the most common histological subtype. The estimated 5-year survival rate (5-YSR) was 48.5%. The 5-YSR was similar regardless of the type of primary treatment (chemotherapy alone vs. surgery/chemotherapy, 50.7 vs. 45.3%, p=0.582). A comparison of the survival according to the primary site of involvement revealed a 5-YSR of 32.5% (p=0.027), 64.3% (reference), 46.5% (p=0.113), and 49.8% (p=0.024) for the small intestine, ileocecal region, large intestine, and multiple sites, respectively. Multivariate analysis, however, revealed a low hemoglobin level, advanced Ann Arbor stage, and aggressive histological type to be independent prognostic factors for shorter survival but not ileocecal region involvement. Conclusions: The Ann Arbor stage, hemoglobin level, and histological type were independent prognostic factors for survival, while the primary site of involvement and treatment modality did not affect the prognosis in patients with B-cell PIL.

CT and MRI Features of Localized Peritumoral Amyloidosis in a Patient with Head and Neck Mucosa-Associated Lymphoid Tissue Lymphoma: A Case Report.

Amyloidosis has been reported to be associated with non-Hodgkin lymphoma. Amyloidosis and lymphoma can be related in two ways: lymphoma-associated systemic amyloidosis and peritumoral amyloidosis with lymphoma. We report a rare case of peritumoral amyloidosis in a patient with head and neck mucosa-associated lymphoid tissue lymphoma. On CT, the oropharyngeal mass showed an irregularly shaped soft-tissue density with multifocal amorphous calcifications and heterogeneous enhancement. On MRI, the mass showed heterogeneous low signal intensity on both T1- and T2-weighted images. On contrast-enhanced MR images, the mass showed good enhancement with several inner non-enhancing foci. Concurrent pathologies, such as peritumoral amyloidosis, should be considered when calcifications are noted in patients with pre-treatment lymphoma.