Rare case of mantle cell lymphoma of the lacrimal sac

ABSTRACT Mantle cell lymphoma of the ocular and periorbital regions is extremely rare but should be considered in the differential diagnosis of lesions affecting the periorbital tissues. In this study, we present a rare case of mantle cell lymphoma of the lacrimal sac in a 65-year-old male presenting with a mass in the lacrimal sac region and epiphora. After clinical examinations and imaging studies, the mucocele was misdiagnosed. Considering the unexpected findings during external dacryocystorhinostomy, a frozen biopsy was performed, which confirmed the diagnosis of lymphoma.

Benefits of BV-AVD (Brentuximab vedotin, doxorubicin, vinblastine, and dacarbazine) versus ABVD (doxorubicin hydrochloride, bleomycin sulfate, vinblastine sulfate, dacarbazine) in patients with advanced-stage Hodgkin's lymphoma: an analysis of the ECHELON 1 trial by the GATLA group using the Delphi Method.

INTRODUCTION: The BV-AVD (Brentuximab vedotin, doxorubicin, vinblastine, and dacarbazine) combination for first-line treatment of advanced stage Hodgkin's lymphoma has been approved by regulatory authorities and included in international guidelines. However, several factors influence its incorporation as standard of care. MATERIALS AND METHODS: A group of experts from different institutions was identified and, using the Delphi method, an analysis of the results of the ECHELON 1 trial for the indication of BV-AVD over ABVD (doxorubicin hydrochloride, bleomycin sulfate, vinblastine sulfate, dacarbazine) in patients with Hodgkin's lymphoma Stages III and IV in Argentina was done. The clinical and academic experience of the authors and the context of the Argentine healthcare system were considered. RESULTS AND DISCUSSION: Seven statements on general aspects of the management of Hodgkin's lymphoma and nine on specific aspects related to the use of BV-AVD over ABVD reached a consensus of agreement. There was a strong expert consensus in favor of indicating BV-AVD in the presence of extranodal disease or pulmonary disease. Moderate to severe neuropathy, pregnancy and drug allergy were considered absolute contraindications to prescribe BV. CONCLUSIONS: The authors agreed that BV-AVD could be considered a new treatment option in high-risk patients. However health system-dependent factors (such as high cost, lack of availability, reimbursement difficulties, irregular delivery, and issues with granulocyte-colony stimulating factor availability) could pose limitations for this prescription. While awaiting new data from clinical trials and real-world studies, these recommendations can represent a useful tool for hematologists in different parts of the world.

[Simultaneous presentation of bullous pemphigoid and mycosis fungoides]. / Presentación simultánea de penfigoide ampollar y micosis fungoide.

The relationship between bullous pemphigoid (BP) and neoplasms has been debated in the medical literature. Although numerous case reports have described the coexistence of BP with various neoplasms, case-control studies have yielded conflicting results. We present the case of a male patient who developed BP shortly after being diagnosed with mycosis fungoides (MF). He was a 77-year-old man with a history of type 2 diabetes mellitus and hypertension who was diagnosed with MF. Subsequently, he developed blisters after sun exposure, and was diagnosed with BP through histopathology and direct immunofluorescence. The patient was treated with prednisone and methotrexate, with favorable evolution without recurrence of MF or appearance of new blisters. The association between cutaneous T-cell lymphoma and autoimmune blistering disease is rare, although similar cases have been reported, some associated with phototherapy. In this case, the onset of BP after sun exposure suggests a potential connection. The coexistence of BP and MF remains controversial, and this case highlights the importance of considering autoimmune blistering diseases in patients with oncohematological neoplasms.

La relación entre el penfigoide ampollar (PA) y las neoplasias ha sido objeto de debate en la literatura médica. Aunque numerosos informes de casos han descrito la coexistencia del PA con diversas neoplasias, estudios de casos y controles han arrojado resultados contradictorios. Presentamos el caso de un paciente masculino que desarrolló un PA poco después de ser diagnosticado con una micosis fungoide (MF). Se trata de un hombre de 77 años con antecedentes de diabetes mellitus tipo 2 e hipertensión arterial que fue diagnosticado con MF. Posteriormente, desarrolló ampollas después de una exposición solar, siendo diagnosticado con PA mediante histopatología e inmunofluorescencia directa. El paciente fue tratado con meprednisona y metotrexato, evolucionando favorablemente sin recurrencia de MF ni aparición de nuevas ampollas. La asociación entre un linfoma cutáneo de células T y una enfermedad ampollar autoinmune es rara, aunque han sido reportados casos similares, algunos asociados con fototerapia. En este caso la aparición del PA después de la exposición solar sugiere una conexión potencial. La coexistencia entre PA y MF sigue siendo controvertida, y este caso destaca la importancia de considerar enfermedades ampollares autoinmunes en pacientes con neoplasias oncohematológicas.

Gut Microbiota Disruption in Hematologic Cancer Therapy: Molecular Insights and Implications for Treatment Efficacy.

Hematologic malignancies (HMs), including leukemia, lymphoma, and multiple myeloma, involve the uncontrolled proliferation of abnormal blood cells, posing significant clinical challenges due to their heterogeneity and varied treatment responses. Despite recent advancements in therapies that have improved survival rates, particularly in chronic lymphocytic leukemia and acute lymphoblastic leukemia, treatments like chemotherapy and stem cell transplantation often disrupt gut microbiota, which can negatively impact treatment outcomes and increase infection risks. This review explores the complex, bidirectional interactions between gut microbiota and cancer treatments in patients with HMs. Gut microbiota can influence drug metabolism through mechanisms such as the production of enzymes like bacterial ß-glucuronidases, which can alter drug efficacy and toxicity. Moreover, microbial metabolites like short-chain fatty acids can modulate the host immune response, enhancing treatment effectiveness. However, therapy often reduces the diversity of beneficial bacteria, such as Bifidobacterium and Faecalibacterium, while increasing pathogenic bacteria like Enterococcus and Escherichia coli. These findings highlight the critical need to preserve microbiota diversity during treatment. Future research should focus on personalized microbiome-based therapies, including probiotics, prebiotics, and fecal microbiota transplantation, to improve outcomes and quality of life for patients with hematologic malignancies.

Mantle Cell Lymphoma in the Oral Cavity: Exploring the Differential Microscopic Diagnosis of a Rare Case.

Mantle cell lymphoma (MCL) is a rare subtype of B-cell non-Hodgkin's lymphoma (NHL), which generally has an aggressive course. Its pathophysiology seems to be related with the malignant transformation of B-cell mantle zone lymphocytes due to the CCND1 rearrangement. The occurrence of MCL in the oral cavity is especially rare. In this report, we present an exceptional case of oral MCL diagnosed in the palate in a 56-year-old male patient, highlighting its distinct morphological and immunohistochemical features that may assist in the accurate diagnosis.

Ultrasound in Skin Cancer: Why, How, and When to Use It?

BACKGROUND: Skin cancer is the most common cancer in human beings. Ultrasound is a powerful and non-invasive imaging technique that has expanded its use in dermatology, including in the skin cancer field. The full range of critical anatomical information provided by ultrasound cannot be deduced from a naked eye examination, palpation, or other imaging techniques such as dermoscopy, confocal microscopy, magnetic resonance imaging, or PET-CT (Positron Emission Tomography-Computed Tomography). METHODS: This review practically analyzes the main ultrasonographic features of the most common types of skin cancers and the performance of the locoregional staging according to the literature, which is illustrated by state-of-the-art clinical and ultrasonographic correlations. RESULTS: The most common types of skin cancer show recognizable ultrasonographic patterns. CONCLUSIONS: Among the current radiological imaging techniques, ultrasound has the highest axial spatial resolution. Compared to other imaging techniques used in dermatology, it shows the great advantage of penetrating the soft tissues thoroughly, which allows us to detect and identify the most common skin types of skin cancer, including both the primary tumor and its locoregional metastases.

Proceedings from the First Onco Summit: LATAM Chapter, 19-20 May 2023, Rio de Janeiro, Brazil.

The Onco Summit 2023: The Latin American (LATAM) Chapter took place over two days, from 19-20 May 2023, in Brazil. The event aimed to share the latest updates across various oncology disciplines, address critical clinical challenges, and exchange best practices to ensure optimal patient treatment. More than 30 international and regional speakers and more than 300 oncology specialists participated in the Summit. The Summit discussions centered on common challenges and therapeutic advances in cancer care, with a specific focus on the unique obstacles faced in LATAM and examples of adaptable strategies to address these challenges. The Summit also facilitated the establishment of a network of oncologists, hematologists, and scientists in LATAM, enabling collaboration to improve cancer care, both in this region and globally, through drug development and clinical research. This report summarizes the key discussions from the Summit for the global and LATAM oncology community.

Characterizing the Mutational Landscape of Diffuse Large B-Cell Lymphoma in a Prospective Cohort of Mexican Patients.

Diffuse large B-cell lymphoma (DLBCL) is the most common B-cell malignancy worldwide. Molecular classifications have tried to improve cure rates. We prospectively examined and correlated the mutational landscape with the clinical features and outcomes of 185 Mexican patients (median age 59.3 years, 50% women) with newly diagnosed DLBCL. A customized panel of 79 genes was designed, based on previous international series. Most patients had ECOG performance status (PS) < 2 (69.2%), advanced-stage disease (72.4%), germinal-center phenotype (68.1%), and double-hit lymphomas (14.1%). One hundred and ten (59.5%) patients had at least one gene with driver mutations. The most common mutated genes were as follows: TP53, EZH2, CREBBP, NOTCH1, and KMT2D. The median follow-up was 42 months, and the 5-year relapse-free survival (RFS) and overall survival (OS) rates were 70% and 72%, respectively. In the multivariate analysis, both age > 50 years and ECOG PS > 2 were significantly associated with a worse OS. Our investigation did not reveal any discernible correlation between the presence of a specific mutation and survival. In conclusion, using a customized panel, we characterized the mutational landscape of a large cohort of Mexican DLBCL patients. These results need to be confirmed in further studies.

Lower doses of dacarbazine (modified BEACODD) as a safer strategy with equal effectiveness in an intensive treatment protocol of Hodgkin's lymphoma: a preliminary retrospective analysis of a single public center in Brazil.

The German Hodgkin Study Group developed the escalated BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone) protocol as a treatment strategy for advanced-stage Hodgkin's lymphoma. In Brazil, as well as in other countries, procarbazine has been replaced with dacarbazine due to the limited availability of procarbazine. The Hematology Center at Irmandade da Santa Casa de Misericórdia in São Paulo adopted and modified the escalated BEACOPP protocol, substituting prednisone with dexamethasone and incorporating two different doses of dacarbazine: 375 mg/m2/day on Day 8 or the original dose of 250 mg/m2/day on Days 2 and 3. This adjustment was made in response to the anticipated toxicity profile. This study aimed to compare the two different doses in the protocols (375 mg/m2/cycle versus 500 mg/m2/cycle) administered to patients with advanced Hodgkin's lymphoma in similar periods. This retrospective study analyzed the data of 31 patients at a single center in Brazil from 2019 to 2021. Seventeen of the 31 patients received 500 mg/m2/cycle (500 Group), while 14 received 375 mg/m2/cycle (375 Group). At the end of the protocol, 71% of the patients in the 375 Group and 76% in the 500 Group achieved complete remission. On analyzing the number of cycles that patients presented with febrile neutropenia, the 500 Group had three times more events (17.9%) than the 375 Group (6.09% - p-value = 0.04). In the 500 Group, 47.1% needed to change the protocol to ABVD (doxorubicin hydrochloride, bleomycin sulfate, vinblastine sulfate, and dacarbazine) due to toxicity. In this limited cohort from a single public center in Brazil, the use of 375 mg/m2 of dacarbazine per cycle of the modified escalated BEACOPP protocol emerged as a safer strategy, maintaining treatment efficacy without compromising response in patients with advanced Hodgkin's lymphoma.

Clinical characteristics and outcomes of Brazilian patients with duodenal-type follicular lymphoma: a multicenter retrospective study.

Duodenal-type follicular lymphoma (DFL) is a rare subtype classified by the 5th edition of the WHO and international consensus classifications of lymphoid neoplasms, typically presenting as localized disease with favorable outcomes. This multicenter retrospective study examines 53 Brazilian DFL patients with a median age of 58.2 years (33-85), with males comprising 50% (n = 27). According to Lugano GI tract classification, 40 patients (75%) were stage I. Median follow-up was 2.9 years (range 0.1-11). Incidental diagnosis occurred in 28 patients (52.8%) during routine endoscopy; 24 patients (45%) presented mild gastrointestinal symptoms. Treatments included watchful waiting (32 patients, 60.4%), rituximab monotherapy (15 patients, 28.3%), radiotherapy (three patients, 5.7%), and chemoimmunotherapy (three patients, 5.7%). Three patients experienced disease progression; watchful waiting showed three spontaneous remissions. No deaths occurred during follow-up. This study, the first from Latin America, demonstrates a good prognosis across treatments, highlighting Watchful waiting's effectiveness.

The gut microbiota is altered significantly in primary diffuse large b-cell lymphoma patients and relapse refractory diffuse large b-cell lymphoma patients.

PURPOSE: Studies have shown that the gut microbiota may affect anti-tumor immunity by regulating the host immune system and tumor microenvironment. To date, little is known about whether the gut microbiota underlies the occurrence of diffuse large B-cell lymphoma (DLBCL) and drug resistance. METHODS: In the present study, we compared the gut microbiota structure of fecal samples from 26 patients with primary DLBCL, 28 patients with relapsed and refractory (RR) DLBCL, and 30 healthy people. RESULTS: Notably, Fusobacteria (from phylum to species) was enriched in the primary group. A decrease of Fusobacterium and an increase of Enterococcus were found in the RR group. PICRUSt analysis found that genes related to cytochrome P450 were upregulated in the RR group compared to the primary group, which likely contributes to the occurrence of DLBCL and the formation of drug resistance. CONCLUSIONS: Our study provides further evidence for the relationship between gut microbiota and DLBCL and the formation of drug resistance, highlighting the potential significance of the bacterial variations may be used as new biomarkers of DLBCL.

Follicular Lymphoma in Chile in the Adult Public Cancer Program: The Impact of Chemoimmunotherapy.

BACKGROUND: Follicular lymphoma (FL) is the most common indolent non-Hodgkin lymphoma (NHL) in the United States and Europe. However, data on FL from Latin America are scant. AIMS: This study aims at better understand the clinical features, treatment patterns and outcomes of patients with FL in Chile. Of special interest was to evaluate POD24 as an adverse marker. METHODS AND RESULTS: We collected retrospective data from 722 patients 15 years or older diagnosed with FL and treated in 17 cancer centers in Chile between 2000 and 2019. Time to first treatment (TTFT), progression-free survival (PFS) and overall survival (OS) were estimated using the Kaplan-Meier method. Cox proportional-hazard regression models were fitted to investigate prognostic factor. The median age at diagnosis was 62 with a female predominance (63%); 73% of patients had advance stage disease and 68% had bone marrow involvement; 63% had intermediate or high FLIPI scores. The 1-year TTFT rate was 96%, and 30% of patients received chemoimmunotherapy. Adding rituximab to chemotherapy was associated with a higher complete response (69% vs. 60%; p < 0.001) and superior median OS (16 vs. 8 years; p < 0.001). Patients who experience POD24 had an inferior median OS (2.4 vs. 15 years). CONCLUSION: Our study shows a female predominance in patients with FL in Chile and confirms superior response and survival outcomes with adding rituximab to chemotherapy. Our study also confirms a poor OS in patients who experience POD24.

Circulating tumor DNA in diffuse large B-cell lymphoma: analysis of response assessment, correlation with PET/CT and clone evolution.

INTRODUCTION: Circulating tumor DNA (ctDNA) can be obtained from cell-free DNA (cfDNA) andis a new technique for genotyping, response assessment and prognosis in lymphoma. METHODS: Eighteen patients with samples at diagnosis (ctDNA1), after treatment (ctDNA2) and extracted from diagnostic tissue (FFPE) were evaluated. RESULTS: In all patients, at least one mutation in cfDNA was detected at diagnosis. CREBBP was the most frequent mutated gene (67 %). In 12 of the 15 patients with complete remission, the mutation attributed to the disease found at diagnosis cleared with treatment. A reduction in the ctDNA was observed after treatment in 14 patients, 12 of whom achieved complete remission. Correlations were found between the ctDNA at diagnosis and total metabolic tumor volume (r = 0.51; p-value = 0.014) and total lesion glycolysis 2.5 (r = 0.47; p-value = 0.024) by PET at diagnosis and between ctDNA at diagnosis and radiomic features of the lesions with the largest standardized uptake value. There was a strong inverse correlation between ΔctDNA1 and ΔSUVmax by PET/CT (r = -0.8788; p-value = 0.002). CONCLUSION: Analysis of ctDNA and PET/CT in large B-cell lymphoma are complementary data for evaluating tumor burden and tumor clearance after treatment. Analysis of radiomic data might help to identify tumor characteristics and their changes after treatment.

Predictive value of prognostic nutritional index as prognostic biomarkers in patients with lymphoma: a systematic review and meta-analysis.

BACKGROUND: Several research have indicated the significant potential of the Prognostic Nutritional Index (PNI) as a prognostic biomarker in lymphoma patients. However, there is some inconsistency in the findings of a few studies. Hence, to offer a thorough evaluation of the predictive significance of PNI in lymphoma patients, we performed a meta-analysis to examine the prognostic value of PNI for survival outcomes in lymphoma patients. METHODS: We conducted a comprehensive search for pertinent works published up until December 2023 in databases such as PubMed, EMBASE, Cochrane Library, and Web of Science. We obtained hazard ratio (HR) data related to survival outcomes and computed aggregated HRs with their corresponding 95% confidence intervals (CIs) to evaluate the correlation between PNI and both overall survival (OS) and progression-free survival (PFS) in lymphoma patients. RESULTS: By analyzing data from 1260 patients in 28 studies, we found that PNI levels were associated with prognosis in lymphoma patients. High PNI levels predicted that patients had longer OS (HR: 0.46, 95% CI 0.37-0.58, P < 0.05) and better PFS (HR: 0.56, 95% CI 0.45-0.70, P < 0.05). Subgroup analyses showed that the predictive ability of PNI for patient prognosis may differ depending on the type of lymphoma. In addition, we found that the critical PNI value had greater predictive potential at 40-45 and above 45. CONCLUSION: Our study suggests a strong association between PNI and prognostic outcomes in lymphoma patients, indicating that PNI holds substantial prognostic value in this population.

Intravascular lymphoma: A diagnostic challenge for a treatable cause of rapidly progressive dementia.

Intravascular large B-cell lymphoma (IVLBCL) is a rare hematological malignancy where its development in the intravascular environment is the main characteristic. Despite its ability to affect multiple organic systems, there is a tropism for the central nervous system, which may be related to several clinical syndromes, making this condition a great mimic and consequently a diagnostic challenge. Rapidly progressive dementia may be one of the presenting phenotypes of IVLBCL. This case report aims to highlight the main red flags, such as sustained elevation of lactate dehydrogenase, organomegaly and specific lesions with vasculitis-like bleeding, all that can be used as clinical clues to direct the differential diagnosis. In addition, it reinforces the role of early brain biopsy in this context, since IVLBCL is a treatable disease.

Remisión de la psoriasis vulgar posterior a quimioterapia por neoplasia hematológica / Remission of psoriasis after chemotherapy for hematological malignancy

RESUMEN Divulgamos el caso de una paciente con psoriasis vulgar que presento empeoramiento de la misma luego de infección por Chikungunya, sin mejoría del brote con el tratamiento habitual de la psoriasis, en quien posteriormente se realiza el diagnóstico de Linfoma Hodgkin tipo clásico. Se inició tratamiento quimioterápico y se observó una mejoría completa de las lesiones cutáneas de la psoriasis.

ABSTRACT We report the case of a patient with psoriasis vulgaris who presented worsening of the disease after infection with Chikungunya, with no improvement of the outbreak with the usual treatment for psoriasis, in whom a diagnosis of classic Hodgkin lymphoma was subsequently made. Chemotherapy treatment was started and a complete improvement of the skin lesions of psoriasis was observed.

Obstacles to global implementation of CAR T cell therapy in myeloma and lymphoma.

Chimeric Antigen Receptor T-cell (CAR-T) therapies are transforming the treatment of B-cell lymphoproliferative disorders and multiple myeloma, yet global access challenges and barriers for their implementation persist. Global access disparities persist, particularly for persons living in low and middle-income countries and for underserved populations in high income countries. In this review we address patient-related factors including age, comorbidities, fitness, race and ethnicity, and geographic location for CAR-T access. Also, we review disease-related and health system barriers like disease biology, potential for short and long-term toxicity, insurance access, referrals, supply and manufacturing, regulation, costs and treatment center capacity. Lastly, alternatives for overcoming these barriers exemplified by research efforts worldwide are discussed, emphasizing the need for a multifaceted approach from all stakeholders to improve global accessibility and ensure equitable access and improved outcomes for patients worldwide.

Cytokine release syndrome after chimeric antigen receptor T cell therapy in patients with diffuse large B-cell lymphoma: a systematic review.

INTRODUCTION: Chimeric antigen receptor T (CAR-T) cell therapy is an innovative technology that has shown promising results in clinical trials. Treatment is based on modifying the patient's own T cells to express artificial surface receptors to specifically recognize and attack the tumor cells. OBJECTIVE: To synthesize available evidence on the incidence and management strategies of cytokine release syndrome in patients with diffuse large B-cell lymphoma who received CAR-T cell therapy. METHODS: This is a systematic literature review. The search was conducted in the PubMed, Scopus, and Web of science databases. This review followed the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. The systematic review protocol is registered in the International Prospective Register of Systematic Reviews (PROSPERO) database under number CRD42022359258. RESULTS: Nineteen studies were included with a total of 1193 patients who received CAR-T cell therapy. Of these patients, 804 (67%) developed some degree of cytokine release syndrome. The frequencies of Grade 3 and 4 cytokine release syndrome were 10% and 3%, respectively. The regimen most used in the management of the syndrome included tocilizumab and/or glucocorticoids. CONCLUSION: The results obtained in this review demonstrate high rates of cytokine release syndrome in patients with diffuse large B-cell lymphoma treated with CAR-T cell therapy, however these events are manageable, supporting the conclusion that this therapy is safe in these patients.

The role of autologous bone marrow transplantation in primary effusion lymphoma: a case report and literature review.

Primary effusion lymphoma (PEL) is an aggressive and rare type of diffuse large B-cell lymphoma (DLBL) that commonly presents itself as pleural, pericardial or peritoneal effusion without lymph node or extranodal involvement in immunosuppressed patients, such as HIV-positive or transplanted receptors. On rare occasions, it may be found in solid sites without effusion, in an immunophenotypically and morphologically similar neoplasm well-known as extracavitary PEL (EC-PEL). Both PEL and EC-PEL are associated with extremely poor prognosis. Due to the rarity of these entities, ther e are no gold standard treatments . Here we discuss the role of autologous bone marrow transplant (auto-BMT) in the treatment of these patients as well as report the case of a young HIV-positive male diagnosed with both PEL and EC-PEL, who underwent a salvage therapy with auto-BMT and achieved complete and sustained remission eight years after the diagnosis.

Medical Imagery: Cytomegalovirus sialadenitis in a patient with B-cell acute lymphoblastic leukemia.

Cytomegalovirus (CMV) can cause a broad range of diseases, with severity depending on immune status, comorbidities, and age. Initial CMV infection usually occurs in childhood and is typically asymptomatic, leading to lifelong latency. In immunocompromised patients, CMV can affect multiple organs, but salivary gland infections are rare. This study presents a case of a 66-year-old woman with B-cell acute lymphoblastic leukemia who developed swelling and pain in the right preauricular region during pre-transplant consolidation therapy. Despite a recent bone marrow biopsy indicating morphological remission and a flow cytometry analysis detecting only 0.04 % B lymphoblasts, she exhibited these symptoms. A CT scan revealed enlargement, hyperdensity, and enhancement of the right parotid glands, with accompanying subcutaneous edema. A biopsy of the right parotid gland showed a dense interstitial lymphoplasmacytic infiltrate with numerous Cowdry bodies and smaller granular cytoplasmic inclusions, all testing positive for CMV immunohistochemistry. The findings confirm the diagnosis of CMV sialadenitis in an immunocompromised patient. This case underscores the importance of considering CMV infections in similar clinical scenarios, particularly in patients with compromised immune systems.