Primary cardiac lymphoblastic B-cell lymphoma: Should we treat more intensively?

Primary cardiac lymphoma (PCL) is a rare neoplasm, the majority of cases of which are non-Hodgkin's, diffuse large B-cell (DLBCL). We report the first case of an adult with PCL B-cell lymphoblastic lymphoma whose disease evolution was grim. A 52-year-old male reported dyspnea and facial swelling lasting for 4 months and upon a physical examination he presented bradycardia, jugular venous engorgement, and hypophonesis of cardiac sounds. An electrocardiography (Echo) revealed a right atrial mass and nodules at the pericardium. The patient was treated with R-Hyper-CVAD (rituximab plus cyclophosphamide, vincristine, doxorubicin, and dexamethasone) and presented very short remission. At this time, we used R-ICE (rituximab plus ifosfamide, carboplatin, and etoposide) chemotherapy and the patient underwent complete remission after two courses and received autologous bone marrow transplantation (auto-BMT). After 75 days of follow-up, the patient reported dyspnea and a new Echo showed a recurrence of the disease. The patient died due to cardiac failure. PCL is a rare disease with an unfavorable prognosis and a prompt diagnosis and treatment are fundamental to survival. We believe that more intensive therapies, such as auto-BMT, should be considered as a first treatment option.

Prognostic factors in adolescent and adult patients with acute lymphoblastic leukemia with two protocols of chemotherapy: a cross-sectional study.

BACKGROUND: We evaluated the clinical, laboratory, and prognostic factors in adolescent and adult patients with acute lymphoblastic leukemia (ALL). MATERIALS AND METHODS: In this observational, retrospective, cross-sectional study, we examined the medical records of all consecutive patients with ALL admitted to a public hospital in Brazil from 1990 to 2005. RESULTS: Of the 102 patients included, 88 were treated with 2 protocols of chemotherapy (Berlin-Frankfurt-Münster [BFM] 86 modified [BFM-86M] and UCLA [University of California, Los Angeles] protocol). The complete remission (CR), disease-free survival, and overall survival (OS) rate was 70.6%, 27%, and 30.5%, respectively (median follow-up, 49 months). Age < 18 years and no leukemic infiltration in the central nervous system (CNS) at diagnosis were positively associated with CR (P = .03); no bleeding and hepatomegaly at diagnosis and age < 35 years were associated with better OS on multivariate analyses of the whole population (P = .01). OS at 4 years was superior with BFM-86M than with UCLA (49.5% vs. 16%; P = .004), especially in young adults without risk factors. CONCLUSION: We identified age as the most important prognostic factor in patients with ALL. CNS infiltration, hepatomegaly, and bleeding were associated with lower OS but must be validated in future research with South American populations and worldwide. The BFM-86M protocol can be considered a therapeutic option for young adults (age < 35 years) without adverse prognostic factors. For other patients with ALL, we emphasize the need for different therapeutic approaches.