Rare disease Background: Primary mediastinal diffuse large B cell lymphoma (DLBCL) presenting as a large intracardiac tumor is extremely rare and has not been significantly reported in the literature. Cardiac lymphoma consists of 2 subtypes: mediastinal DLBCL invading the heart and primary cardiac lymphoma. Both subtypes have a poor prognosis and are treated similarly. Mediastinal DLBCL is a life-threatening condition that, if diagnosed early, has a better survival rate. This is a rare case of a mediastinal DLBCL invading the right atrium as a large intracardiac mass, causing partial obstruction of the tricuspid valve without hemodynamic compromise. Case Report: A 57-year-old female presented with unintentional weight loss, fatigue, exertional dyspnea, and cough for 8 weeks. Transesophageal echocardiogram showed a mass (3.5×3.5 cm) in the posterior wall of the right atrium partially obstructing the tricuspid valve. Biopsy revealed DLBCL. Given new-onset lymphoma, a human immunodeficiency virus (HIV) test was done and came back positive. CD4 count was 100 cells/mm 3. Chemotherapy was initiated with rituximab, cyclophosphamide, epirubicin, vincristine, and prednisone (R-CHOP). Highly active anti-retroviral (HAART) therapy was started for HIV. After treatment with R-CHOP and HAART, the patient had complete resolution of the mass and symptoms on follow-up imaging and evaluation at 6 months. Conclusions: Mediastinal DLBCL invading the heart is a life-threatening form of non-Hodgkin's lymphoma (NHL) and early diagnosis and treatment is critical as prognosis is poor especially if diagnosed in later stages of the disease. Testing for HIV is important as 5% of HIV patients are susceptible to developing NHL.
ObjectiveMorbidity and mortality in Rheumatoid Arthritis (RA) is partly mitigated by maintaining immune and hematologic homeostasis. Identification of those at risk is challenging. Red cell distribution width (RDW) and absolute lymphocyte count (ALC) associate with cardiovascular disease (CVD) and mortality in the general population, and with disease activity in RA. How these parameters relate to inflammation and mortality in RA were investigated.MethodsIn a retrospective single VA-Rheumatology Clinic cohort of 327 RA patients treated with methotrexate (MTX)+/-TNF-blocker we evaluated RDW and ALC before and during therapy, and in relation to subsequent mortality. Findings were validated in a national VA cohort (n=13,914). In a subset of patients and controls we evaluated inflammatory markers.ResultsIn the local cohort, High RDW and Low ALC prior to MTX treatment each associated with subsequent mortality over 10 years (p<0.001 and p=0.004). The highest mortality was observed in those with both high-RDW and low ALC. This remained after adjusting for age and co-morbidities, and was validated in the national RA cohort. In the immunology cohort, soluble and cellular inflammatory markers were higher in RA than controls, ALC correlated with plasma TNFR2, NK/Monocyte-HLADR-MFI, and CD4CM/CD8CMHLADR/CD38%, while RDW associated with CD4%/CD8% and CD4EM/CD4TE HLADR/CD38%. MTX initiation was followed by an increase in RDW and decrease in ALC. TNF-blocker therapy added to MTX resulted in an increase in ALC.ConclusionRDW and ALC before DMARD therapy associate with biomarkers of monocyte/macrophage inflammation and subsequent mortality. Mechanistic linkage between TNF signaling and lymphopenia here warrants further investigation.
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