Treatment and Survival Outcomes of Waldenstrom Macroglobulinemia in Latin American Patients: A Multinational Retrospective Cohort Study.

PURPOSE: Waldenstrom Macroglobulinemia (WM) is a rare lymphoma with distinct clinical features, and data from Latin American patients are lacking. Therefore, we aim to investigate the clinical, therapy, and outcome patterns of WM in Latin America. METHODS: We retrospectively analyzed patients with WM diagnosed between 1991 and 2019 from 24 centers in seven Latin American countries. The study outcomes were overall survival (OS) and progression-free survival (PFS). RESULTS: We identified 159 cases (median age 67 years, male 62%). Most patients (95%) were symptomatic at diagnosis. The International Prognostic Scoring System for WM (IPSSWM) at diagnosis was available in 141 (89%) patients (high-risk 40%, intermediate-risk 37%, and low-risk 23%). Twenty-seven (17%) patients were tested for MYD88L265P, with 89% (n = 24 of 27) carrying the mutation. First-line and second-line therapies were administered to 142 (89%) and 53 (33%) patients, respectively. Chemoimmunotherapy was the most commonly used first-line (66%) and second-line (45%) approach; only 18 (11%) patients received ibrutinib. With a median follow-up of 69 months, the 5-year OS rate was 81%. In treated patients, the 5-year OS and PFS rates were 78% and 59%, respectively. High-risk IPSSWM at treatment initiation was an independent risk factor for OS (adjusted hazard ratio: 4.73, 95% CI, 1.67 to 13.41, P = .003) and PFS (adjusted hazard ratio: 2.43, 95% CI, 1.31 to 4.50, P = .005). CONCLUSION: In Latin America, the management of WM is heterogeneous, with limited access to molecular testing and novel agents. However, outcomes were similar to those reported internationally. We validated the IPSSWM score as a prognostic factor for OS and PFS. There is an unmet need to improve access to recommended diagnostic approaches and therapies in Latin America.

Epidemiology and Risk Factors for the Development of Infectious Complications in Newly Diagnosed Multiple Myeloma: A Multicenter Prospective Cohort Study in Latin America.

PURPOSE: Infections are a significant cause of morbidity and mortality in patients with multiple myeloma (MM). In Latin America, data on infectious complications in this patient population are lacking. METHODS: We conducted a prospective cohort study of patients with newly diagnosed MM (NDMM) in seven Latin American countries between June 2019 and May 2020. Patients with active disease, on active therapy, and with a follow-up of 6 months from the time of diagnosis were included. Our primary end point was the number of infectious events that required hospitalization for ≥ 24 hours. RESULTS: Of 248 patients with NDMM, 89 (35.9%) had infectious complications (113 infectious events), the majority (67.3%) within the first 3 months from diagnosis. The most common sites of infection were respiratory (38%) and urinary tract (31%). The microbial agent was identified in 57.5% of patients with gram-negative bacteria (73.5%) as the most common pathogen. Viral infections were infrequent, and no patients with fungal infection were reported. In the multivariable analysis, diabetes mellitus (odds ratio [OR], 2.71; 95% CI, 1.23 to 6.00; P = .014), creatinine ≥ 2 mg/dL (OR, 4.87; 95% CI, 2.29 to 10.35; P < .001), no use of trimethoprim-sulfamethoxazole prophylaxis (OR, 6.66; 95% CI, 3.43 to 12.92; P < .001), and treatment with immunomodulatory drugs (OR, 3.02; 95% CI, 1.24 to 6.29; P = .003) were independent factors associated with bacterial infections. At 6 months, 21 patients (8.5%) had died, 47.6% related to infectious complications. CONCLUSION: Bacterial infections are a substantial cause of hospital admissions and early death in patients with NDMM. Antibiotic prophylaxis should be considered to reduce infectious complications in patients with MM.

Manejo de neoplasias oncohematológicas en el contexto de la pandemia de COVID-19: recomendación de expertos / Management of oncohematological neoplasms in the COVID-19 pandemic: experts recommendation

RESUMEN La pandemia por COVID-19 originado por el Coronavirus 2 causante de síndrome respiratorio agudo severo (SARS-CoV-2) es causante de una crisis de salud pública a nivel global. Muchos reportes indican resultados desalentadores en pacientes con cáncer respecto a la población general. Por ello, los expertos en el manejo de neoplasias oncohematológicas del Instituto Nacional de Enfermedades Neoplásicas, hospitales nacionales y una clínica privada de Lima Metropolitana han desarrollado recomendaciones obtenidas por consenso para continuar con el manejo de pacientes con neoplasias oncohematológicas en forma segura ante la coyuntura de pandemia.

ABSTRACT The ongoing COVID-19 pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a global public health crisis. Many reports indicate disappointing results in cancer patients compared to the general population. Therefore, experts in the management of oncohematological malignancies from the National Institute of Neoplastic Diseases, national hospitals and a private clinic in Metropolitan Lima have developed recommendations obtained by consensus to continue with the management of patients with oncohematological neoplasms safely in the face of the pandemic.

Addition of amifostine to the CHOP regimen in elderly patients with aggressive non-Hodgkin lymphoma: a phase II trial showing reduction in toxicity without altering long-term survival.

BACKGROUND AND OBJECTIVES: We report the 8-year follow-up of 34 patients aged ≥69 years old with NHL included in a phase IIb open-label randomized parallel groups study to evaluate the effectiveness of amifostine in preventing the toxicity of cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP regime) . PATIENTS AND METHODS: Patients were randomized to receive classical CHOP (cyclophosphamide 750 mg/m(2), doxorubicin 50 mg/m(2), vincristine 1.4 mg/m(2) [maximum 2 mg] on day 1 and prednisone 100 mg/day for 5 days) or CHOP plus amifostine (6 cycles of amifostine 910 mg/m(2) on day 1). Efficacy (time to progression, TTP; disease-free survival, DFS; overall survival, OS) and toxicity endpoints were evaluated. RESULTS: Thirty-four patients were randomized to A-CHOP (n=18) or CHOP (n=16). Patients with A-CHOP vs CHOP had significantly lower toxicity; neutropenia grade 4 ocurred in 13/92 (13%) vs 23/85 (27%, P=0.007) cycles, febrile neutropenia in 3/92 A-CHOP (3%) vs 8/85 (10%, P=.056) CHOP cycles, hospitalization for toxicity in 4/92 (4%) A-CHOP vs 11/85 (13%, P=.05) CHOP cycles. Median hospitalization stay for toxicity was 5 days with A-CHOP vs 8 days with CHOP (P=.05). There were no significant differences at 8 years in TTP (A-CHOP, 48.9% vs CHOP, 36.3%; P=.65), DFS (A-CHOP, 72.9% vs CHOP 55.6%; P=.50) and OS (A-CHOP, 44.3% vs CHOP, 54.4%). There was no long-term toxicity of clinical interest. The only prognostic factor identified to 8 years was the International Prognostic Index (IPI low/low intermediate risk vs high intermediate/high risk; HR=2.98; CI 95%:1.01-8.77; P=.048). CONCLUSION: These results show that amifostine can be added to the standard CHOP treatment schedule with less acute toxicity and without influencing the outcome.

Linfoma difuso de células grandes b primario de vulva: presentación de un caso / Diffuse large b cell primary vulva: case report

Los linfomas no Hodking (LNH) constituyen un grupo heterogéneo de canceres que tienen diferentes modelos de comportamiento y diversas respuestas al tratamiento . Se originan en ganglios linfáticos o en sitios extranodales y suelen diseminarse a diversos órganos. Los LNH de linaje B cutáneos primarios (LNHB) representan únicamente el 20-25% de todos los linfomas primarios cutáneos. En este trabajo reportamos el caso de una mujer con diagnóstico final de LDCGB. Este tumor es extremadamente raro e implica un particular abordaje diagnóstico y terapéutico.La localización vulvar del Linfoma no Hodgkin de células ôBõ primario cutáneo (LNHB) es extremadamente rara, , con menos de 100 casos reportados en la literatura. Los LNHB cutáneos, como los de otra localización, son característicamente CD20 positivos , aunque se ha reportado un caso CD 30+ . Conclusiones: Los LNH extranodales genitales constituyen el 1.5 % de los linfomas ,teniendo una gran variedad de presentaciones clínicas. Los LNHB cutáneos primarios del tracto genital femenino son entidades clínicas y patológicas poco frecuentes, con un comportamiento indolente y un pronóstico favorable incluso con terapia local, siendo importante el diagnostico temprano de esta entidad clínica.

NonûHodgkinÆs lymphoma form a heterogenous group of cancers that have different models from behavior and diverse answers from the treatment. They are originated in lymphatic ganglia or extranodal sites and usually they are scattered to diverse organs. The primary LNH of lineage B cutaneous (LNHB) represent the 20-25% of all the solely linfomas primary cutaneous. We reported the case of a woman with final diagnosis of LDCGB. This tumor is extremely rare and implies a particular boarding therapeutic and diagnosis. The location to vulvar of the Linfoma Hodgkin of cutaneous cells ôprimary Bõ (LNHB) is not extremely rare, with less than 100 cases reported in Literature. The cutaneous LNHB, like those of another location, are characteristically positive CD20, although it has been reported a case CD 30+. Conclusions: The genital extranodal LNH constitute 1,5% of linfomas, having a great variety of clinical presentations. Primary the cutaneous LNHB of the feminine genital tract are little frequent clinical and pathological organizations, with an indolent behavior and a favorable prognosis even with local therapy, being important the early diagnosis of this disease.

Development of rapidly progressive liver light chain deposition under VAD chemotherapy in multiple myeloma.

Light chain deposition disease (LCDD) is a multisystemic disorder seen in the setting of plasma cell dyscrasias. The histological characteristic of this disorder is the deposition of a homogeneous, granular, slightly eosinophilic and non-Congophilic material that shows immunostaining for monoclonal light chains (kappa or gamma), while in primary amyloidosis (AL) the proteinaceous substance is fibrillar and Congo red positive. In contrast with AL, the light chain in LCDD is usually of the kappa-type. Renal involvement, resulting in nephrotic syndrome, is usually the prominent feature of LCDD. Patients with this disease may also have heart, liver or other organ involvement, mimicking the picture of primary systemic amyloidosis. However, liver failure has rarely been described in patients with LCDD. A patient with myeloma-associated LCDD who developed rapidly progressive liver kappa light chain deposition with fatal outcome after undergoing the first cycle of vincristine/doxorubicin/dexamethasone chemotherapy is reported.

Acute promyelocytic leukaemia in patients originating in Latin America is associated with an increased frequency of the bcr1 subtype of the PML/RARalpha fusion gene.

The PML/RARalpha fusion gene in acute promyelocytic leukaemia (APL) has three subtypes based on the breakpoint site of the PML gene: long (bcr1), short (bcr3) and variable (bcr2) subtypes. The PML/RARalpha fusion protein is involved in the pathogenesis of APL and the breakpoint site of the PML gene might be associated with aetiological factor(s). Because APL is over-represented in patients that originate in Latin America (Latinos), we evaluated whether the distribution of the PML/RARalpha fusion mRNA in this population is different to that reported in non-Latinos. Among 52 APL patients (28 from Mexico and Central America diagnosed in Los Angeles and 24 from Peru, South America), bcr1, bcr2 and bcr3 expression was 75%, 10% and 15% respectively. However, bcr1 breakpoints were significantly higher compared with non-Latino patients (340/654, 52%) reported in four studies. Often bcr1 and bcr2 are reported together; 862 (60%) of 1429 non-Latino APL patients reported in nine studies were either bcr1 or bcr2, compared with 44 (85%) in our 52 Latino patients. This difference was also statistically significant when our patients were compared to each of the individual studies from USA and Europe, but not for a small series from China and Japan. These results suggest that the overrepresentation of APL among Latin American patients can be accounted for by an increase of a single subtype--bcr1, and the breakage sites in the PML gene may not be random but possibly influenced by genetic and/or environmental factor(s).

Estado intelectual en preescolares del Callao / Intellectual status of pre-scholar children in Callao

Objetivos: Estudiar el estado intelectual de preescolares de nivel socioeconómico bajo. Método: Se estudió a 134 preescolares entre 3 años y 5 años 10 meses de edad procedentes del Asentamiento Humano Bocanegra (Callao-Perú), mediante las Pruebas Goodenough-Harris (131 niños) y Wechsler Preschool and Primary Scale of Intelligence (81 niños). Resultados: De acuerdo a la prueba Goodenough-Harris, 6.11 por ciento de niños tuvo retardo mental leve y 8.4 por ciento inteligencia marginal. Según la prueba Weschler Preschool and Primary Scale of Intelligence, 1.23 por ciento de niños tenía deficiencia mental y 4.94 por ciento eran fronterizos. Encontramos una importante correlación entre la prueba Goodenough-Harris y la prueba Wechsler Preschool and Primary Scale of intelligence. Conclusiones: La prevalencia de retardo mental fue similar a la descrita en la literatura para la población general. La prueba de Goodenough-Harris es un instrumento rápido que puede emplearse en estudios que comparan niveles de intelectuales entre grupos pero no para determinar la prevalencia de retardo mental en una población.