Case Of Lymphoma Research Articles

S2876 An Extremely Rare Case of Extra-Nodal Marginal Zone B-Cell Lymphoma in Transverse Colon Found During Screening Colonoscopy in an Asymptomatic Patient

S2876 An Extremely Rare Case of Extra-Nodal Marginal Zone B-Cell Lymphoma in Transverse Colon Found During Screening Colonoscopy in an Asymptomatic Patient

Exploring the Potential of Enhanced Prognostic Performance of NCCN-IPI in Diffuse Large B-Cell Lymphoma by Integrating Tumor Microenvironment Markers: Stromal FOXC1 and Tumor pERK1/2 Expression.

FOXC1 and ERK1-2 are proteins implicated in aggressive biological behavior of various malignancies including lymphomas. We investigate the additive prognostic value of stromal FOXC1 expression and tumor phosphorylated ERK1-2 (pERK1-2) expression to the established National Comprehensive Cancer Network International Prognostic Index (NCCN-IPI), in 92 diffuse large B-cell lymphoma (DLBCL) cases. Multidimensional analysis using statistics and machine learning (ML) models assessed prognostic value of established clinicopathologic variables with stromal FOXC1 and tumor pERK1-2 expressions. Both high FOXC1 stroma group and high pERK1-2 tumor group were significantly associated with shorter progression-free survival (PFS) and overall survival (OS) compared with low group (p = 0.015, 0.034 and p = 0.025, 0.025 each respectively). In multivariable analysis, high FOXC1 stromal expression was an independent prognostic factor of OS (p = 0.037). The addition of stromal FOXC1 and tumor pERK1-2 to the NCCN-IPI score significantly improved prediction of time to death compared with NCCN-IPI score alone (Harrell's C-index = 0.801 vs. 0.764; p = 0.030). ML models reconfirmed the addition of stromal FOXC1 expression and tumor pERK1-2 to NCCN-IPI score had the highest C-index (0.952) among combinations. Stromal FOXC1 and tumor pERK1-2 were determinants of DLBCL prognosis, whose addition significantly improved prognostic performance of the NCCN-IPI.

Incidence of intestinal & extra-intestinal cancers among individuals with Crohn's disease in northern India.

Background & objectives Crohn's disease (CD) is associated with a higher risk of malignancy, which is attributed to disease behaviour and the usage of immunosuppressants. The burden of malignancy in CD is scarcely reported from Asia. We report real-world data on CD-related malignancy from a northern Indian cohort. Methods This retrospective analysis included individuals with CD who were followed up at the All India Institute of Medical Sciences, New Delhi, from 2005 to 2021. The standardized incidence ratio (SIR) was used to calculate the relative risk of malignancy in CD affected individuals compared to the general population. Results In this study, 952 study participants were included, with a mean age at diagnosis of 36.9±15.11 yr; 61.1 per cent were male. The median follow-up duration was 34 months [IQR (interquartile range): 19-73]. Most study participants received steroids (76.7%), immunomodulators (68.7%), or anti-TNF therapy (10.8%). The overall incidence of malignancy was 1.05 per cent, indicating a 10.45 times higher risk in CD [SIR: 10.45; 95% Confidence interval (CI):4.98-17.96]. Eight out of 826, 1 of 106 and 1 of 25 study participants developed malignancy in the first, second and third decades, respectively. The cumulative risk of malignancy was 2.7, 5.5, and 13.4 per cent in the first, second, and third decades, respectively. Regarding bowel malignancies, one study participant each developed ileocaecal adenocarcinoma, anorectal adenocarcinoma, malignant rectal fibrous histiocytoma, and gastric adenocarcinoma. Extraintestinal malignancies included single cases each of follicular neoplasia of the thyroid, neuroendocrine tumour of the pancreatic tail, breast cancer, hepatocellular cancer, oral cancer, and prostate cancer. No cases of lymphoma or skin malignancy were reported. Interpretation & conclusions At 30 yr, the cumulative risk of malignancy among Indian CD-affected individuals was 13.4 per cent, with a SIR of 10.45 (95% CI: 4.98- 17.96). The risk increased with increasing age at disease onset and duration.

Cytotoxicity of bendamustine, alone and in combination with novel agents, toward adult T-cell leukemia cells.

Adult T-cell leukemia/lymphoma (ATL) develops from the infection of T cells with human T lymphotropic virus type 1 (HTLV-1). There are an estimated 5-20 million HTLV-1 carriers worldwide and the patients are frequently observed in subtropical Africa, the Caribbean, Middle East, South America, and South West Japan. The prognosis of ATL remains dismal due to rapid acquired resistance to treatment with cytotoxic chemotherapeutic agents. In particular, the development of novel therapies for relapsed or refractory (R/R) ATL is an unmet need. Previous clinical trials revealed that bendamustine (BDM) was effective as the first-line treatment for indolent lymphoma and R/R cases of diffuse large B-cell lymphoma. Its major advantage is that it has few side effects such as hair loss and peripheral neuropathy, and does not impair the quality of life. However, its efficacy has not been verified for ATL in pre-clinical or clinical studies. In this study, we have shown the cytotoxicity of BDM alone and in combination with novel agents including the histone deacetylase (HDAC) inhibitor tucidinostat, the enhancer of zeste homolog 1/2 (EZH1/2) dual inhibitor valemetostat, and the Bcl2 family inhibitor ABT-737. The combined in vitro effects of BDM and tucidinostat were reproduced in a murine model without any obvious hematological toxicity. Our present results suggest that the combination of tucidinostat and BDM could additively prolong the survival of patients with R/R progressive ATL. The efficacy and safety of this combination are thus worthy of investigation in clinical settings.

Incidental detection of non-melanoma neoplasms in sentinel lymph node biopsy for melanoma staging: A retrospective case series.

Patients with malignant melanoma have an increased risk of developing secondary hematologic malignancy, and patients with hematologic malignancies have an increased risk of developing melanoma. Rarely, sentinel lymph node biopsies (SLNBs) collected for melanoma staging might harbor lymphoma or even carcinoma, which may represent a second primary malignancy (SPM). Biopsied lymph node(s) might serve as the first site of recognition for a SPM. Yet, there has been little systematic investigation regarding the characteristics of incidental SPMs detected on SLNB for melanoma staging. A series of cases of lymphomas and carcinomas were detected incidentally during SLNB for melanoma staging from two tertiary academic centers between 2000 and 2021. Fifteen cases of incidentally detected SPMs were reviewed, comprising 12 lymphomas and three carcinomas. The most common incidentally detected second malignancy was chronic lymphocytic leukemia/small lymphocytic lymphoma (60%, 9/15). There were three cases of incidentally detected metastatic carcinoma. Of all incidentally detected malignancies, 2/3 carcinomas and 4/12 lymphomas represented first-time diagnoses of SPM in a melanoma patient. Forty percent of cases (6/15) also harbored metastatic melanoma in the sentinel lymph node. It is possible to incidentally detect SPMs in SLNBs for melanoma staging. Early detection of SPMs in melanoma patients has implications for the treatment of both incidental SPM and melanoma.

A Rare Case of Primary Bone Follicular Lymphoma with Multiple Osteolytic Lesions: A Case Report and Review of the Literature

Introduction: Primary bone lymphoma (PBL) is a rare clinical entity, accounting for less than 5% of all extranodal non-Hodgkin lymphomas and approximately 5% of primary bone tumors. Diffuse large B-cell lymphoma (DLBCL) is the most common histotype, accounting for about 80% of all PBL cases. Conversely, the incidence of indolent primary bone lymphomas (iPBL) represents less than 1% of all reported PBL cases, and data on these rarer lymphomas are scarce. Drawing on diagnostic criteria developed by the World Health Organization (WHO) and the International Extranodal Lymphoma Study Group (IELSG), we report a rare case of primary bone follicular lymphoma, focusing specifically on the clinical presentation and treatment. Discussion: Additionally, we provide a systematic review of the literature data on this very rare lymphoproliferative entity.

Loss of CCL28 and CXCL17 Expression and Increase in CCR1 Expression May Be Related to Malignant Transformation of LGBLEL into Lymphoma.

To investigate the differential expression of the chemokine signaling pathway in lacrimal gland benign lymphoepithelial lesion (LGBLEL) and lacrimal lymphoma, providing insights into the mechanisms underlying malignant transformation and aiding clinical differentiation. Transcriptome analysis was conducted on patients with LGBLEL, lymphoma, and orbital cavernous hemangioma (CH). Three cases of LGBLEL and three cases of lymphoma were randomly selected as control and experimental groups, respectively. A real-time quantitative polymerase chain reaction (RT-qPCR) was used to validate genes associated with the chemokine signaling pathway. Immunohistochemical (IHC) staining and quantitative Western blotting (WB) were performed for precise protein quantification. Transcriptome analysis revealed differential expression of the chemokine signaling pathway between the LGBLEL and lymphoma groups, identifying ten differentially expressed genes: CCL17, VAV2, CXCR5, NRAS, HCK, RASGRP2, PREX1, GNB5, ADRBK2, and CCL22. RT-qPCR showed that, compared to the lymphoma group, the LGBLEL group had significantly higher expression of CCL28, CXCL17, HCK, GNB5, NRAS, and VAV2 (p = 0.001, <0.001, <0.001, <0.001, =0.020, <0.001, respectively) and lower expression of CCR1 (p = 0.002). IHC staining and quantitative analysis confirmed significant differences in protein expression between the groups for CCL28, CCR1, CXCL17, HCK, GNB5, NRAS, and VAV2 (p = 0.003, 0.011, 0.001, 0.024, 0.005, 0.019, and 0.031, respectively). While IHC provided localization, WB offered greater precision. WB revealed that, compared to the lymphoma group, the LGBLEL group exhibited significantly higher expression of CCL28, CXCL17, HCK, GNB5, NRAS, and VAV2 (p = 0.012, 0.005, 0.009, 0.011, 0.008, and 0.003, respectively) and lower expression of CCR1 (p = 0.014). The chemokine signaling pathway plays a role in the malignant transformation of LGBLEL. The decreased expression of CCL28 and CXCL17, coupled with the increased expression of CCR1, may be linked to the progression of LGBLEL into lymphoma.

Dermoscopy as an additional method for diagnosis of mycosis fungoides

Mycosis fungoides is a primary epidermotropic T-cell lymphoma of the skin, characterized by the proliferation of small and medium-sized T-lymphocytes with cerebriform nuclei. Mycosis fungoides accounts for 60.6% of the total number of all cases of primary T-cell lymphomas of the skin. Diagnosis of mycosis fungoides remains a big challenge in dermatology: the average time for diagnosis from the onset of the disease is 5 years. The difficulty of differential diagnosis with inflammatory dermatoses, such as psoriasis, is due to the similarity of their clinical characteristics, especially in the early stages of the mycosis fungoides. The search for new methods aimed at early diagnosis of skin T-cell lymphoma is one of the most important tasks in dermatooncology. A number of morden studies aimed at assessing the capabilities of dermatoscopy as an additional method for diagnosing mycosis fungoides. Specific dermoscopic pattern of mycosis fungoides were identified: fine short linear vessels, orange-yellowish patchy areas and vascular structure resembling spermatozoa. Description of this dermoscopic pattern can influence the accuracy of diagnosis and timeliness of diagnosis, the prescription of effective methods of therapy in the early stages of the disease, improving the quality and increasing the life expectancy of patients. The article provides an overview of the most up-to-date information on the dermoscopic pattern of mycosis fungoides.

Photogallery. HIV infection and neoplastic skin diseases

Malignant neoplasms constitute a group of the most important secondary diseases that develop in people infected with the human immunodeficiency virus (HIV). The first neoplastic disease described in HIV-infected patients is Kaposi's sarcoma. In contrast to other types of Kaposi's sarcoma, its epidemic (AIDS-associated) type is more common in young and middle-aged HIV-positive individuals, especially among men who have same-sex sex. Epidemic Kaposi's sarcoma does not have a favourite localisation. However, there is a tendency to lesions of the scalp (face, ear flaps), upper extremities, anogenital area, oral mucosa. On the trunk, the pathological process spreads along the Langer lines. Skin rashes are represented by spotty, papular, nodular elements of red-brown or purple colour. The aggressive course is characteristic: rapid dissemination and generalisation of rashes. Plasmoblastic lymphoma is associated with immunodeficiency states and is rare. The tumour lesion is usually located in the oral cavity. Few cases of plasmoblastic lymphoma with skin and soft tissue involvement have been described in the literature. Squamous cell skin cancer in the context of HIV infection is a dangerous disease. In people living with HIV, it manifests at a significantly earlier age and is associated with a high risk of local recurrence. The extent of invasion and the likelihood of metastasis are influenced by HIV-associated immunodeficiency. In the vast majority of cases, risk factors and clinical manifestations of basal cell skin cancer do not differ from those in immunocompetent individuals. Basal cell carcinomas rarely metastasise. Nevertheless, metastatic forms lead to unfavourable outcome. Here is a photo gallery of cases of neoplastic skin lesions developed in HIV-positive patients.

The Vitamin C Level in Cases with Hematological Malignancies

Background: Oxidative stress plays a significant role in the pathogenesis of hematological malignan-cies. Vitamin C, known for its antioxidant properties, has garnered attention in this context. There-fore, we investigated vitamin C levels in patients with hematological malignancies and evaluated the relationship between vitamin C levels and response to treatment. Materials and Methods: Our study examined 150 cases of hematological malignancies and 30 he-althy cases. The vitamin C levels of patients with hematological malignancies were compared with those of the healthy group. Results: Vitamin C levels in cases of acute myeloblastic leukemia (n=30) (p&amp;lt;0.001), acute lympho-blastic leukemia (n=30) (p&amp;lt;0.001), Hodgkin lymphoma (n=30) (p&amp;lt;0.001), diffuse large B-cell lympho-ma (n=30) (p&amp;lt;0.001), and multiple myeloma (n=30) (p&amp;lt;0.001) were significantly lower compared to healthy individuals. There was a significant relationship between vitamin C levels and the response to treatment in cases with acute myeloblastic leukemia, acute lymphoblastic leukemia, Hodgkin lymphoma, diffuse large B-cell lymphoma, and multiple myeloma (p=0.020, p=0.020, p=0.040, p&amp;lt;0.001, p&amp;lt;0.001, respectively). In Hodgkin and non-Hodgkin lymphoma cases, a negative correla-tion was found between vitamin C levels and LDH and beta-2 microglobulin levels (p=0.001; p=0.008; p=0.017; p=0.019, respectively). Conclusions: Our study underscores the lower levels of vitamin C in patients with hematological malignancies compared to healthy individuals. Furthermore, the findings suggest that vitamin C levels could serve as a potential biomarker for predicting the response to treatment in these cases.

Intraparenchymal low-grade B-cell lymphomas of the central nervous system: Clinicopathologic and molecular analysis of three cases and a review of the literature

Primary central nervous system (CNS) lymphomas represent 1 % of all non-Hodgkin lymphomas, with diffuse large B-cell lymphomas as the prevailing subtype. Low-grade B-cell lymphomas are exceptional with only 24 marginal zone B-cell lymphomas (EMZL) and 1 follicular lymphoma (FL) previously reported so far. While their molecular profiles are studied elsewhere, data on primary intraparenchymal CNS cases remain limited. The objective of the present study is to contribute new cases of primary intraprenchymal low-grade B-cell lymphomas in the CNS and characterize their mutational profile. We conducted a comprehensive review of cases and a literature review to identify similar instances. Clinical, imaging, histological, immunohistochemical, and molecular characteristics were analyzed. Diagnoses were established according to established criteria. We present three novel cases of intraparenchymal CNS low-grade B-cell lymphomas. One case of intraparenchymal EMZL exhibited plasmacytic differentiation, while another lacked a plasma cell component. The third case was diagnosed as FL. The L265P mutation of MYD88 was absent in all cases. Next generation sequencing revealed pathogenic mutations in SPEN (Glu1970ValfsTer64) and ARID1A (Pro1355LeufsTer118) genes in one EMZL case. In conclusion, intraparenchymal CNS low-grade B-cell lymphomas are rare, with few reported cases. Our findings expand knowledge on their clinical and molecular features. We present the first molecular profile of primary CNS intraparenchymal EMZL, underscoring the need for further research to understand their biology and optimize treatment strategies.

A case of grade1 follicular lymphoma diagnosed by laparoscopic lymph node resection: differentiating from late lymph node recurrence of endometrial cancer.

Follicular lymphoma is a common hematologic malignancy; however, it is less common among all malignant diseases and is difficult to suspect in advance due to the lack of specific clinical findings. Here, we report a case in which a late recurrence of corpus cancer was first suspected and finally diagnosed as follicular lymphoma. A 67-year-old female presented to our department with enlarged pelvic lymph nodes. She was diagnosed with breast cancer (HER2-posiotive with lymph node metastasis) and corpus cancer (endometrioid carcinoma grade 2, stage IA) 16years prior, received definitive therapy and was followed up. A positron emission tomography scan was performed, and an accumulation of 18F-fluorodeoxyglucose (FDG) was detected in multiple lymph nodes, including the lymph nodes with no change in size or enlargement. We performed laparoscopic resection of the enlarged and FDG-accumulated lymph nodes and a pathological examination. The patient was diagnosed with follicular lymphoma (FL) grade 1 and is currently under observation at the Department of Hematology. FL can be considered when there is a discrepancy between the change in lymph node size and the degree of FDG accumulation. A pathological examination is useful for accurate diagnosis. Therefore, it is important to consider tissue collection; however, care must be taken to minimize the invasiveness of the procedure for the patient.

Clinical features of 20 cases with Pneumocystis jirovecii pneumonia after allogeneic hematopoietic stem cell transplantation

This study included 20 patients with hematological diseases who developed Pneumocystis jirovecii pneumonia (PJP) after receiving allogeneic hematopoietic stem cell transplantation (allo-HSCT) from April 2014 to October 2022 at Peking University People's Hospital. The 20 patients comprised 13 males (65.0% ) and seven females (35.0% ), with a median age of 34 (19-60) years. Eleven cases (55.0% ) of acute myeloid leukemia, four cases (20.0% ) of acute lymphocytic leukemia, two cases (10.0% ) of myelodysplastic syndrome, one case (5.0% ) of chronic myelomonocytic leukemia, one case (5.0% ) of non-Hodgkin lymphoma, and one case (5.0% ) of aplastic anemia were analyzed. Three cases (15.0% ) of HLA-identical sibling hematopoietic stem cell transplantation, three cases (15.0% ) of matched unrelated donor hematopoietic stem cell transplantation, and 14 cases (70.0% ) of haploid hematopoietic stem cell transplantation were identified. The median onset time of PJP was 353 (74-1121) days after transplantation. The clinical symptoms mainly included fever, cough, expectoration, and dyspnea. All patients presented signs of infection based on the CT scan, including bilateral diffuse ground-glass opacities, patchy shadows, and solid nodules. Nine patients (45.0% ) required respiratory support via nasal catheter oxygen inhalation, while seven patients (35.0% ) required ventilator-assisted breathing. Seven (35.0% ) severe infections and 13 (65.0% ) mild to moderate infections were recorded. Moreover, eight patients (40.0% ) were complicated with human cytomegalovirus infection, whereas two patients were complicated with EB virus infection. Furthermore, all 20 patients received treatment with compound sulfamethoxazole (standard dose, 11 cases; low dose, 9 cases). Furthermore, 19 patients survived and one patient died.

Development and validation of machine learning models for predicting cancer-related fatigue in lymphoma survivors

BackgroundNew cases of lymphoma are rising, and the symptom burden, like cancer-related fatigue (CRF), severely impacts the quality of life of lymphoma survivors. However, clinical diagnosis and treatment of CRF are inadequate and require enhancement. ObjectiveThe main objective of this study is to construct machine learning-based CRF prediction models for lymphoma survivors to help healthcare professionals accurately identify the CRF population and better personalize treatment and care for patients. MethodsA cross-sectional study in China recruited lymphoma patients from June 2023 to March 2024, dividing them into two datasets for model construction and external validation. Six machine learning algorithms were used in this study: Logistic Regression (LR), Random Forest, Single Hidden Layer Neural Network, Support Vector Machine, eXtreme Gradient Boosting, and Light Gradient Boosting Machine (LightGBM). Performance metrics like the area under the receiver operating characteristic (AUROC) and calibration curves were compared. The clinical applicability was assessed by decision curve, and Shapley additive explanations was employed to explain variable significance. ResultsCRF incidence was 40.7 % (dataset I) and 44.8 % (dataset II). LightGBM showed strong performance in training and internal validation. LR excelled in external validation with the highest AUROC and best calibration. Pain, total protein, physical function, and sleep disturbance were important predictors of CRF. ConclusionThe study presents a machine learning-based CRF prediction model for lymphoma patients, offering dynamic, data-driven assessments that could enhance the development of automated CRF screening tools for personalized management in clinical practice.

Efficacy of nebulized GM-CSF inhalation in preventing oral mucositis in patients undergoing hematopoietic stem cell transplantation: A retrospective study

ObjectiveTo study the efficacy of oxygen atomization inhalation of granulocyte-macrophage colony-stimulating factor (GM-CSF) for preventing oral mucositis in patients following hematopoietic stem cell transplantation. MethodsData from patients who received hematopoietic stem cell transplantation and were treated with GM-CSF for the prevention/treatment of oral mucositis in our hospital from June 2021 to June 2023 were collected. The enrolled patients were divided into an observation group and a control group according to the use of GM-CSF. The WHO Mucositis Scale Assessment Criteria were utilized to evaluate the characteristics of patients with oral mucositis (OM) from the beginning of the pretreatment period until they were discharged from the hospital. The general data, preconditioning protocol, transplantation method, overall grade and duration of oral mucositis, pain score, nutritional score and number of days of parenteral nutrition use, oral mucosal infection status and antibiotic use intensity, the granulocyte and megakaryocyte reconstruction time, and adverse reaction reports of the patients were collected and summarized through the medical records system. ResultsA total of 143 patients were included in this study, including 75 patients in the observation group. In the observation group, there were 36 males and 39 females aged 22–67 years. There were 45 patients who received autologous transplantation and 30 patients who received allogeneic transplantation. In terms of the disease distribution, there were 33 cases of leukemia, 24 cases of lymphoma, 11 cases of multiple myeloma, and 8 other cases (3 cases of aplastic anemia, 2 cases of myelodysplastic syndrome, 2 cases of myelofibrosis, 1 case of POEMS syndrome). There were 68 patients in the control group, including 33 males and 35 females; the control group patients were aged 25–74years. Forty-one patients received autologous transplantation, and 27 patients received allogeneic transplantation. The disease distribution included 29 cases of leukemia, 17 cases of lymphoma, 12 cases of multiple myeloma, and 7 other cases (3 cases of aplastic anemia, 2 cases of myelodysplastic syndrome, 1 case of myelofibrosis, 1 case of POEMS syndrome). There were no significant differences between the two groups concerning age, sex, disease distribution or the transplantation method (P > 0.05). In the observation group, 13 cases did not develop oral mucositis, and 32 cases developed oral mucositis (16 cases of Grade I, 14 cases of Grade II, 2 cases of Grade III, and 0 cases of Grade IV). In the control group, there were 5 cases without mucositis and 36 cases with oral mucositis (6 cases of Grade Ⅰ, 16 cases of Grade Ⅱ, 8 cases of Grade Ⅲ, and 6 cases of Grade Ⅳ), the difference was statistically significant (P < 0.05). The pain score and duration of mucositis in the observation group were significantly lower than those in the control group (P < 0.05). In addition, the oral infection rate, antibiotic use intensity, nutritional score, per capita number of days of parenteral nutrition use and hematopoietic reconstruction time in the observation group were significantly lower than those in the control group (P < 0.05). In the observation group, 8 patients did not develop oral mucositis, and 22 patients developed oral mucositis (13 cases of Grade I, 7 cases of Grade II, 1 case of Grade III, and 1 case of Grade IV). In the control group, 1 case did not develop mucositis, and 26 cases developed oral mucositis (3 cases of Grade Ⅰ, 10 cases of Grade Ⅱ, 9 cases of Grade Ⅲ, and 4 cases of Grade Ⅳ). The difference was statistically significant (P < 0.05). The pain score and duration of mucositis in the observation group were significantly lower than those in the control group (P < 0.05). In addition, the oral mucosal infection rate, antibiotic use intensity, nutritional score, per capita number of days of parenteral nutrition use and hematopoietic reconstruction time in the observation group were significantly lower than those in the control group (P < 0.05). No adverse reactions were reported in either group. ConclusionIn both autologous transplantation and allogeneic transplantation patients, GM-CSF atomized inhalation can improve the prevention and treatment of oral mucositis in stem cell transplantation patients, reduce the incidence of oral infection, reduce the intensity of antibiotic use and the number of days of parenteral nutrition use, and thus promote the process of hematopoietic reconstruction.

A Case of Primary Adrenal Lymphoma: How Do We Detect Early Progression to Adrenal Insufficiency?

A Case of Primary Adrenal Lymphoma: How Do We Detect Early Progression to Adrenal Insufficiency?

Potential diagnostic and prognostic biomarkers of pediatric Burkitt lymphoma identified through miRNA expression profiling.

Pediatric Burkitt lymphoma (pBL) is the most common non-Hodgkin lymphoma in children. These patients require prompt diagnosis and initiation of therapy due to rapid tumor growth. The roles of tumor tissue and circulating microRNAs (miRNAs) in the diagnosis or prognostication have not been fully elucidated in pBLs. Differentially expressed (DE) miRNAs were identified with microRNA sequencing (miRNA-Seq) in tumor tissues and plasma of diagnostic pBLs. The diagnostic potential of total miRNA concentrations and overexpressed miRNAs were evaluated through receiver operating characteristic (ROC) analyses. Log-rank test was employed to evaluate survival differences associated with DE miRNAs. Selected miRNA expressions were cross-validated with quantitative reverse transcription PCR (qRT-PCR). Total circulating cell-free miRNAs were higher in pBL cases compared to controls. Cancer-associated pathways were enriched among miRNAs differentially expressed in pBL tumor tissues. Several upregulated miRNAs in pBL tumors demonstrated high diagnostic potential. Similarly, ROC analysis of overexpressed plasma miRNAs revealed circulating cell-free or exosomal miRNAs that can distinguish pBLs from control cases. Indeed, integrative analysis of overexpressed circulating exosomal miRNAs showed an enhanced diagnostic potential for certain triple combinations. Kaplan-Meier analyses of DE miRNAs in tumor tissues identified miRNAs predicting overall survival. Differentially expressed miRNAs in tumor tissue and plasma of pBL have the potential to improve diagnosis and prognosis. Differentially expressed miRNAs in treatment-naive pediatric Burkitt lymphoma cases have diagnostic or prognostic biomarker potential. This is the first study that applied miRNA-Seq on treatment-naive pediatric Burkitt lymphoma cases for identification of differentially expressed miRNAs both in tumor tissue and plasma samples with diagnostic potential. Through systematic analysis of differentially expressed miRNAs, tumor tissue miRNAs associated with the overall survival of pBLs have been discovered. The clinically significant, differentially expressed miRNAs identified in pediatric Burkitt lymphoma cases can potentially improve the current tissue-based or non-invasive clinical practice in terms of diagnosis or prognostication.

Diffuse large B-cell lymphoma of the gallbladder with hepatoduodenal invasion exhibiting a necrotic tendency.

We report a case of diffuse large B-cell lymphoma (DLBCL) of the gallbladder with extensive hepatoduodenal invasion, which was challenging to diagnose histologically due to a strong tendency to be necrotic. An 71year-old man presented with upper abdominal pain and was referred to our hospital. Computed tomography revealed a distended gallbladder with air within the irregular gallbladder wall and an indistinct border with the hepatoduodenum, suggesting invasion. Esophagogastroduodenoscopy detected an ulceration in the duodenal bulb. However, histologic analysis failed to provide a definitive diagnosis due to the presence of necrotic tissue. Furthermore, direct biopsy from the gallbladder mucosa by endoscopic retrograde cholangiography revealed only necrotic tissue and no diagnosis. Contrast ultrasonography for the hepatic invasion revealed enhancement with blood flow, suggesting non-necrotic tissue. Subsequently, an ultrasound-guided core-needle biopsy was conducted to obtain tissue samples from the described lesion. The pathology showed atypical lymphocytes with irregular nuclei. Immunostaining indicated positive expression of CD10, CD20, Bcl-6, and C-Myc, consistent with a diagnosis of DLBCL. In our case, the lymphoma exhibited a strong tendency to be necrotic, making histologic diagnosis difficult. However, selective biopsy from the site of blood flow made the diagnosis possible and proved to be useful.

Understanding splenic B-cell lymphoma/leukaemia with prominent nucleoli: Diagnosis, underpinnings for disease classification and future directions.

The 5th edition of the WHO classification of haematolymphoid tumours (WHO-HAEM5) introduced a new category, splenic B-cell lymphoma/leukaemia with prominent nucleoli (SBLPN). The diagnostic entity B-cell prolymphocytic leukaemia (B-PLL) has been discontinued and the category of hairy cell leukaemia variant (HCLv) has been conceptually reframed. B-PLL and HCLv diagnoses were uncommon. Overlap existed between B-PLL and other indolent lymphomas like chronic lymphocytic leukaemia/small lymphocytic lymphoma (CLL/SLL). HCLv lacked consistent cytomorphological, immunophenotypic and genetic features. To address these issues, the WHO-HAEM5 classification has introduced SBLPN to serve as a temporary holding ground for entities that do not neatly fit into the existing classification. Cases previously classified as CD5-negative B-PLL and HCLv fall under the SBLPN category. Some splenic marginal zone lymphoma and splenic diffuse red pulp small B-cell lymphoma cases with higher number of medium or large nucleolated B cells would also be classified as SBLPN under the WHO-HAEM5. This review explores the rationale for discontinuing B-PLL and HCLv diagnoses. It then examines the concept of SBLPN, offers practical guidance for diagnosis and discusses future directions in classifying splenic B-cell lymphomas.

Splenic cyst deroofing complicated with B lymphoma

BackgroundSplenic cysts are uncommon and very rarely malignant therefore their treatment isn’t standardized. In case of symptomatic cysts different surgical approaches have been suggested. Primary malignant lymphoma of the spleen comprises less than 1% of non-Hodgkin’s lymphomas. To our knowledge, only 203 cases of splenic large B-cell lymphoma (LBCL) have been reported to date and only 2 of them were fibrin-associated splenic cysts.Case presentation27-year-old model with a 19 × 13 cm splenic cyst without data of malignancy in the preliminary study and therefore treated with laparoscopic deroofing. After histological diagnosis of LBCL with a fibrin/EBV-associated splenic pseudocyst, the patient received 4 cycles of Rituximab and a laparoscopic splenectomy was performed due to resurgence of the pseudocyst. No evidence of malignancy has been found during follow up (EBV viral load every 3 months during the first year, PET-CT every 6 months during the first year and annual afterwards) performed after the splenectomy.Discussion and conclusionsThe value of tumor markers and radiology for diagnosis of splenic cysts is put into question.Only 60 cases of Fibrin-associated LBCL (FA-LBCL) have been described in the literature therefore there are no treatment guidelines for them even though surgery together with systemic treatment has been the prevalent route with good results in most cases.