Patient With a Diffuse Large B-Cell Non-Hodgkin Lymphoma in the Right Heart Chamber That Caused Cardiogenic Shock Was Well-Responded to Corticosteroids and Chemotherapy.

Heart tumors are sporadic. Secondary heart tumors are 30 times more common than primary ones. Depending on the location and origin of the tumor, clinical pictures vary from asymptomatic to severe manifestations such as arrhythmia, heart failure, pericardial effusion, and cardiogenic shock. We report hereby a rare case who presented with faint clinical symptoms, rapidly progressing to right heart failure within a month. Echocardiography and computed tomography of the chest revealed a tumor in the right heart chamber of 72.0 × 43.0 mm, in addition to large mediastinal lymph and left supraclavicular lymph nodes, cardiogenic shock appeared 4 days after admission. Through examination, it was suspected that this was a cardiac lymphoma. The patient was treated with 2 mg methylprednisolone per kg body weight. Symptoms of cardiogenic shock improved significantly and disappeared after 6 hours of treatment. After supraclavicular lymph node biopsy and immunohistochemistry, the final result was diagnosed as diffuse large B-cell non-Hodgkin lymphoma with large lymphoma in the right heart. The patient received chemotherapy with the R-CHOP regimen (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisolone). Re-examination before the 5th chemotherapy cycle showed no signs of right heart failure, normal self-activity, and no dyspnea on exertion, and the tumor size in the heart on the echocardiogram was 23.8 × 19.1 mm. The report shows that a large right heart tumor with a clinical picture of cardiogenic shock in a patient with diffuse large B-cell non-Hodgkin's lymphoma was well-responded to initial treatment with methylprednisolone at a dose of 2 mg/kg body weight and R-CHOP chemotherapy.

A Curious Case of Pericardial Effusion Diagnosed as Diffuse Large B-cell Lymphoma.

Lymphoma arises from mature B, T, and natural killer (NK) cells. Lymphomas are classified into Hodgkin's lymphoma (HL) and non-Hodgkin's lymphoma (NHL). Diffuse large B-cell lymphoma (DLBCL) is a type of NHL. It can present with symptoms such as fever, chills, or night sweats, as well as symptoms due to extranodal involvement. Extranodal sites can include the gastrointestinal tract or renal involvement. A higher risk of developing diffuse large B-cell lymphoma (DLBCL) is seen in patients with congenital or acquired immunodeficiency, those on immunosuppression, and those with autoimmune disorders. In this case report, we present a case of pericardial effusion that, upon further evaluation, was diagnosed as diffuse large B-cell lymphoma (DLBCL). A 64-year-old male presented with complaints of retrosternal chest pain that progressed from New York Heart Association (NYHA) Grade II to IV over a month. The chest pain was moderate intensity, dull aching, and non-radiating. It was associated with orthopnea, paroxysmal nocturnal dyspnea, and anasarca. A chest X-ray (posteroanterior {PA} view) showed cardiomegaly with an increased cardiothoracic ratio, mediastinal widening, and pulmonary congestion. Echocardiography revealed moderate non-tappable pericardial effusion. A high-resolution computed tomography (HRCT) chest scan showed moderate pericardial effusion and a homogeneous enhancing mass in the left anterior superior mediastinum. A computed tomography (CT)-guided biopsy was performed to check for lymphoma, thymoma, or tuberculosis. The patient was diagnosed with diffuse large B-cell lymphoma (DLBCL). Owing to the diverse manifestations of diffuse large B-cell lymphoma (DLBCL), prompt diagnosis is required for controlling disease progression.

Validation of single nucleotide polymorphisms potentially related to R-CHOP resistance in diffuse large B-cell lymphoma patients.

Aim: Diffuse large B-cell lymphoma (DLBCL) is the most common B-cell non-Hodgkin lymphoma (NHL). Despite the availability of clinical and molecular algorithms applied for the prediction of prognosis, in up to 30%-40% of patients, intrinsic or acquired drug resistance occurs. Constitutional genetics may help to predict R-CHOP resistance. This study aimed to validate previously identified single nucleotide polymorphisms (SNPs) in the literature as potential predictors of R-CHOP resistance in DLBCL patients, SNPs. Methods: Twenty SNPs, involved in R-CHOP pharmacokinetics/pharmacodynamics or other pathobiological processes, were investigated in 185 stage I-IV DLBCL patients included in a multi-institution pharmacogenetic study to validate their previously identified correlations with resistance to R-CHOP. Results: Correlations between rs2010963 (VEGFA gene) and sex (P = 0.046), and rs1625895 (TP53 gene) and stage (P = 0.003) were shown. After multivariate analyses, a concordant effect (i.e., increased risk of disease progression and death) was observed for rs1883112 (NCF4 gene) and rs1800871 (IL10 gene). When patients were grouped according to the revised International Prognostic Index (R-IPI), both these SNPs further discriminated progression-free survival (PFS) and overall survival (OS) of the R-IPI-1-2 subgroup. Overall, patients harboring the rare allele showed shorter PFS and OS compared with wild-type patients. Conclusions: Two out of the 20 study SNPs were validated. Thus, these results support the role of previously identified rs1883112 and rs1800871 in predicting DLBCL resistance to R-CHOP and highlight their ability to further discriminate the prognosis of R-IPI-1-2 patients. These data point to the need to also focus on host genetics for a more comprehensive assessment of DLBCL patient outcomes in future prospective trials.

Effective management of a rare case of primary renal intravascular large B-cell lymphoma with modified R-CHOP regimen: A case report.

Intravascular large B-cell lymphoma, known for its diverse organ involvement, presents significant diagnostic challenges, particularly when it affects the kidneys. This report highlights a rare case of primary renal intravascular large B-cell lymphoma in a 60-year-old male patient, who presented with persistent fever and renal dysfunction. The case underscores the intricacy of diagnosis and the efficacy of personalized treatment. Following the identification of primary renal intravascular large B-cell lymphoma, a modified R-CHOP regimen was administered, resulting in notable amelioration of symptoms and renal function following the initial treatment cycle. The patient achieved sustained complete remission without any complications after completing five subsequent R-CHOP cycles and two additional cycles of rituximab monotherapy, as confirmed by recent assessments. He is currently under regular follow-up for ongoing monitoring and improvement. This case adds to the limited yet expanding pool of knowledge concerning intravascular large B-cell lymphoma, emphasizing the necessity for personalized therapeutic strategies in atypical presentations. It also highlights the importance of early detection and customized intervention in managing rare lymphoma subtypes with unique organ involvement.

Central nervous system metastasis in a young female patient with primary mediastinal large B-cell lymphoma: A case report and literature review.

Background: Primary mediastinal B-cell lymphoma (PMBCL) is a subtype of diffuse large B-cell lymphoma that originates from a B cell in the thymus. It usually affects young female. Case description: A 30-year-old woman presented with mediastinal mass with history of shortness of breath and chest pain. blood analysis showed low levels of hemoglobin, hematocrit, and mean corpuscular volume and high red cell distribution width. A computed tomography (CT)-guided mediastinal core biopsy disclosed primary mediastinal large B-cell lymphoma (PMLBL) with a nongerminal center phenotype and lung tissue infiltrate. Moreover, after undergoing six cycles of rituximab, cyclophosphamide, hydroxydaunomycin, Oncovin, and prednisone (R-CHOP) chemotherapy and mediastinal radiotherapy, the patient presented with headache and visual disturbance due to multiple supratentorial lesions. Conclusion: Till date, only a few cases of central nervous system (CNS) metastasis have been reported in the literature. Moreover, CNS metastasis of refractory PMBCL is an uncommon event with a poor prognosis. Brain metastases are often the ultimate fatal consequence of many aggressive cancers, so early detection and treatment are important.

An Unusual Presentation of Supraglottic Low-Grade B-Cell Non-Hodgkin's Lymphoma with Tracheostomal Myiasis.

60-year-old male patient presented with dysphagia and a change in voice for eight months. It was established after Direct laryngoscopy surgery and biopsy, that it was a low-grade B cell non-Hodgkin lymphoma. The primary lesion is resolved with Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisolone regimen. Four months later, patient presented with a discharge and maggots at the tracheostomy site. Ifosfamide, Etoposide, Carboplatin was started after a secondary recurrence of disease progression. Hereby we infer this is an unusual case presentation, myiasis with lymphoma recurrence and tough exacting to the otolaryngologist as there are more chances of misdiagnosing as squamous cell carcinoma..

Pancreatic Non-Hodgkin Lymphoma, a rare cause for obstructive jaundice. A Case reports.

INTRODUCTION: Primary pancreatic lymphoma (PPL) is an uncommon condition. Clinical features of PPL are nonspecific & likely to be misrecognized as pancreatic malignancy. CASE PRESENTATION: 71 years old male patient presented with upper abdominal pain with obstructive jaundice. CLINICAL FINDINGS AND INVESTIGATIONS: Examination reveals RHC tenderness and deep icteric. CT shows a large pancreatic head and uncinate process mass. Final diagnosis made with USS guided core biopsy which confirmed B cell, Non-Hodgkin Lymphoma (NHL). INTERVENTION AND OUTCOME: Complete remission of PPL occurred following six cycles of chemotherapy with R-CHOP regimen. RELEVANCE AND IMPACT: PPL is rare condition, accounts 1% of extra nodal lymphomas and 0.5% of malignant pancreatic neoplasm. Ultrasonography, Endoscopic ultrasonography, CT and MRI are the imaging modalities use to diagnose the pancreatic neoplasm. Biopsy of all pancreatic lesion is crucial which can diagnose curable condition such as PPL. Combined therapy with chemotherapy and radiotherapy without surgery is advisable for PPL.

Primary B-Cell lymphoma of the uterine cervix presenting with right ureter hydronephrosis: A case report.

Primary lymphoma of the uterine cervix is a very infrequent disease, usually affecting perimenopausal women. Symptoms are very similar to other gynecological malignancies, but treatment and prognosis completely differ, as most of these patients have a better survival. This condition has to be suspected in women with recent normal Pap smear test, rapidly growing tumor and initially non-contributory biopsies. We report a case of primary diffuse large-B-cell lymphoma of the uterine cervix mimicking a locally advanced cervical cancer with right ureter hydronephrosis at diagnosis. She was medically managed with a combination of rituximab and chemotherapy with cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisone, associated to methotrexate for central nervous system prophylaxis. We will discuss about the role of combined treatments with surgery and radiotherapy, and the fertility sparing management in young women.

Transient adrenal insufficiency in diffuse large B cell lymphoma patients after chemotherapy with short-course, high-dose corticosteroids.

Data on the rate of adrenal insufficiency (AI) in patients receiving short-course and high-dose corticosteroids are limited. In this study, we aimed to determine the incidence of AI in newly diagnosed, diffuse large B cell lymphoma (DLBCL) patients after receiving rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone [or prednisolone] (R-CHOP/CHOP) regimen. We enrolled newly diagnosed DLBCL patients who were scheduled to receive 6-8 cycles of R-CHOP/CHOP regimen. One-microgram adrenocorticotropic hormone (ACTH) stimulation tests were performed at the study entry and 3 weeks after each cycle of chemotherapy (CMT). AI was defined by a peak-stimulated serum cortisol of less than 18 µg/dL. For patients who had AI after completing a course of CMT, 1-µg ACTH stimulation tests were carried out at 60 and 90 days after the last CMT cycle to assess the duration of hypothalamic-pituitary-adrenal (HPA) axis recovery. Ten DLBCL patients were included in this study, with a total of 84 1-µg ACTH stimulation tests. Their mean age was 52 years. AI occurred in 3 out of the 10 patients (30%). The first occurrence of AI was after the third CMT cycle, and the incidence was highest after the fifth cycle. Adrenal function recovered completely 3 to 5 weeks after completing the course of CMT, except for 1 patient, whose HPA axis suppression persisted 90 days after the last CMT cycle. Receiver operating characteristic (ROC) analysis revealed that a basal cortisol level of < 8.7 µg/dL was predictive of AI, with a sensitivity and specificity of 80% and 72.2%, respectively. Transient HPA axis suppression can occur in DLBCL patients receiving R-CHOP/CHOP regimen. We strongly encourage careful observation and examination for potential adrenal insufficiency in such patients, particularly after the fifth cycle of chemotherapy.

How gene polymorphisms can influence clinical response and toxicity following R-CHOP therapy in patients with diffuse large B cell lymphoma.

The treatment of diffuse large B cell lymphoma (DLBCL) is generally based on multidrug chemotherapy, for instance the therapy with rituximab together with cyclophosphamide, vincristine, doxorubicin, and prednisone (R-CHOP). A significant proportion of DLBCL patients benefit from rituximab-based chemoimmunotherapy. However, among patients with DLBCL toxic effects due to therapy treatment are still very frequent, as well as inter-individual differences in the outcomes of patients even having similar stage, histological grade and histopathological type of the tumor. The present paper reviews the actual status of pharmacogenomics studies concerning gene polymorphisms that may affect response and tolerability to R-CHOP therapeutic regimen used to treat DLBCL. There are clear evidences that polymorphisms of genes codifying for protein are involved in cytotoxicity induced by R-CHOP regimen. Moreover, polymorphisms in genes encoding TNF-superfamily cytokines and proteins involved in controlling cellular cycle and tumor growth may be related to variability in efficacy of R-CHOP therapy in DLBCL patients. This knowledge emphasizes the clinical meaning and importance of pharmacogenetics in oncology. The main merit of our study seems to have tried for the first time a comprehensive review of gene polymorphisms that are involved in the response to an entire therapeutic protocol, R-CHOP, in a specific disease, DLBCL, rather than examining polymorphisms referred to individual drugs among themselves not connected or used to treat different pathological conditions. Indeed, it seems clear that only the analysis of a constellation of polymorphisms can really be useful in clinical practice, while knowledge of a single polymorphism seems to give a limited contribution to our ability to use genetic analysis to the management of patients with malignant blood disorders.

Intensive chemotherapy and consolidation with high dose therapy and autologous stem cell transplant in patients with mantle cell lymphoma.

Mantle cell lymphoma (MCL) remains incurable with conventional chemotherapy without consensus on the optimal initial treatment. We examined our single center experience with frontline therapy for patients with MCL in consecutive cases diagnosed 1995-2011. Among 81 patients, median age was 59 (28% were ≥65 years of age), 95% had stage III/IV disease and 54% had a low risk MCL International Prognostic Index score. Thirty-five percent (n=28) received R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) and 65% received R-HCVAD (rituximab, hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone alternating with high-dose methotrexate/cytarabine; n=53). Forty-one patients were consolidated with autologous stem cell transplant (ASCT). There were no significant differences in 2-year survival for R-CHOP versus R-HCVAD (p=0.10) or for ASCT versus observation (p=0.06). After controlling for clinical factors, R-HCVAD followed by ASCT was associated with superior progression-free survival (hazard ratio 0.26, 95% confidence interval 0.09-0.75).

Prophylactic Effect of Pegfilgrastim on Febrile Neutropenia in Patients with Non-Hodgikin's Lymphoma

OBJECTIVE: Pegfilgrastim is recently introduced that is long acting G-CSF for prophylaxis of febrile neutropenia. Treatment of non-Hodgikin's lymphoma (NHL) with R-CHOP is classified with relative high risk of febrile neutropenia. The study evaluated the prophylactic effect of pegfilgrastim to reduce the incidence of febrile neutropenia associated with R-CHOP of patient in NHL. And the risk factors associated with the incidence of FN and related events were evaluated. METHODS: This retrospective study reviews the Electronic Medical Record of 68 NHL patients who received R-CHOP chemotherapy in single center between September 2013 and August 2014. These patients were classified who receive prophylaxis pegfilgrastim or no prophylaxis. RESULTS: Sixty eight patients received R-CHOP with NHL. In 144 cycles of patients receiving pegfilgrastim, compared with no prophylaxis 178 cycles, had a lower incidence of febrile neutropenia (5.5% vs. 23.6%, p = 0.001), grade 3 or grade 4 neutropenia (14.4% vs. 89.8%, p or = 65 (OR: 5.87, 95% CI 1.07-32.27, p = 0.042), IPI > or = 3 (OR: 7.2, 95% CI 1.31-39.6, p = 0.023), S.alb < 3.5 g/dL (OR: 31.01, 95% CI 6.32-152.17, p < 0.0001). In multiple logistic regression analysis, lower baseline serum albumin (OR: 21.1, 95% CI 3.8-116.98, p = 0.001) was significantly associated with occurrence of febrile neutropenia. CONCLUSION: The study recommends prophylactic pegfilgrastim through risk assessment of febrile neutropenia in patients with non-Hodgikin's lymphoma receiving R-CHOP.

Primary cutaneous diffuse large B-cell lymphoma, leg type, with multiple local relapses: case presentation and brief review of literature.

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBL-LT) is a primary cutaneous B-cell lymphoma of intermediate behavior. The disease predominantly affects elderly patients. A 76-year old man presented with red to violaceous nodules in the anterior aspect of both tibias. Histology confirmed the diagnosis of PCDLBL-LT. A thorough clinical and laboratory investigation was negative for any systemic involvement. However, computed tomography of the thorax showed mediastinal lymphadenopathy. Both bone marrow aspiration and trephine did not show any evidence of bone marrow infiltration. Initially R-CHOP regimen (rituximab-cyclophosphamide, doxorubicin, vincristine, prednisone) achieved a total clearance of the lesions. Nevertheless, five months later patient presented with a relapse and was managed with palliative radiotherapy. The same treatment modality was applied for the second recurrence, as well. PCDLBL-LT affects mostly elderly patients. The consequent age related comorbidities and the frequent relapses require a strict follow up of the patients.