Primary plasma cell leukemia in Latin America: demographic, clinical, and prognostic characteristics. A study of GELAMM group.

Primary plasma cell leukemia (pPCL) is an infrequent and aggressive plasma cell disorder. The prognosis is still very poor, and the optimal treatment remains to be established. A retrospective, multicentric, international observational study was performed. Patients from 9 countries of Latin America (LATAM) with a diagnosis of pPCL between 2012 and 2020 were included. 72 patients were included. Treatment was based on thalidomide in 15%, proteasome inhibitors (PI)-based triplets in 38% and chemotherapy plus IMIDs and/or PI in 29%. The mortality rate at 3 months was 30%. The median overall survival (OS) was 18 months. In the multivariate analysis, frontline PI-based triplets, chemotherapy plus IMIDs and/or PI therapy, and maintenance were independent factors of better OS. In conclusion, the OS of pPCL is still poor in LATAM, with high early mortality. PI triplets, chemotherapy plus IMIDs, and/or PI and maintenance therapy were associated with improved survival.

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.

Histoplasmosis diseminada con síndrome hemofagocítico asociado con infección por HTLV-1. reporte de caso / Disseminated histoplasmosis with hemophagocytic syndrome associated with HTLV-1 infection. case report

Síndrome hemofagocítico (SH) es un cuadro sistémico severo usualmente fatal, reportado como causa secundariaen adultos. Histoplasmosis ha sido descrita como una de las causas, siempre en pacientes con inmunosupresión. Sepresenta el caso de un paciente varón natural de Huánuco con historia de tos y expectoración de dos meses e infiltradopulmonar intersticial y parenquimal basal derecho con adenopatía mediastinal. Ingresó con fiebre, pancitopenia,alteración de pruebas hepáticas y pruebas de coagulación que cumplía criterios de SH. En el aspirado de médulaósea se observaron inclusiones intracitoplasmáticas en los macrófagos compatibles con Histoplasma capsulatum.La serología para HTLV-1 fue positiva por lo que se analiza el rol de este virus como agente inmunosupresor quecondiciona SH secundario a histoplasmosis diseminada.

The hemophagocytic syndrome (HS) is a severe systemic illness ultimately fatal associated with underlying conditions. Histoplasmosis has been associated with HS in immunosuppressed patients. We present the case of a male patient from Huanuco with a 2-month history of productive cough and presence of interstitial pulmonary infiltrates, and consolidation in the right lower lobe associated with mediastinal lymphadenopathies on the chest x-ray. The patient presented with fever, pancytopenia and abnormalities in the liver and coagulation tests fulfilling criteria for HS. A bone marrow aspiration showed intracytoplasmic structures within the macrophages compatible with Histoplasma capsulatum. Serology for HTLV-1 was positive. We analyzed the role of HTLV-1 inducing immunosuppression leading to the development of disseminated histoplasmosis and HS.

Efecto de un anticuerpo monoclonal anti CD20 (Rituximab) en trombocitopenia inmune / Effect of an anti CD20 monoclonal antibody (Rituximab) in immune thrombocytopenia

Objetivo: Describir la respuesta terapéutica con un anticuerpo monoclonal anti CD20 (Rituximab), en pacientes con Trombocitopenia Inmune (PTI). Material y métodos: Estudio retrospectivo, descriptivo y observacional tipo serie de casos. Se revisaron las historias clínicas de pacientes adultos con PTI que recibieron el anticuerpo monoclonal anti CD20 (Rituximab), desde diciembre 2005 hasta diciembre 2010. Se definió respuesta: conteo plaquetario >30 mil, por lo menos duplicar el conteo plaquetario inicial y no signos de sangrado, y respuesta completa: conteo plaquetario >100 mil y no signos de sangrado. Resultados: Se evaluaron 24 cursos de tratamiento. Hubo respuesta en 18 (75%), en una media de 11,9 semanas (rango 0,7 û 37,4), la duración media de respuesta fue 16 meses (rango 3,3 û 55,3). Se mantuvo la respuesta obtenida en 12 pacientes, seguimiento promedio de 22 meses (rango 4 - 62). Se logró respuesta completa en 13/23 (60%) casos, en una media de 17 semanas (rango 0,7 û 62,3), con una duración media de respuesta completa de 10,1 meses (rango 2,3 û 25,2), 5 casos mantuvieron respuesta completa con una media de seguimiento de 20 meses (rango 8 û 29). Conclusiones: Se obtuvo una alta tasa de respuesta al tratamiento con Rituximab (hasta 75%) en casos de PTI que fallaron al menos a una línea de tratamiento.

Objective: To describe the therapeutic response of an anti CD20 monoclonal antibody (Rituximab) in patients with immune thrombocytopenia (ITT). Methods: Retrospective, descriptive and observational case series. The clinical charts of ambulatory patients with ITT who received Rituximab from December 2005 to December 2010 were reviewed. Response was defined as platelet count > 30 thousand, at least duplicate the initial platelet count and no evidence of bleeding, and complete response was defined as platelet count > 100 thousand and no evidence of bleeding. Results: 24 courses of treatment were evaluated. Response was obtained in 18 patients (75%); mean time to response was 11.9 weeks (range: 0,7 to 37.4); mean duration of response was 16 months (range: 3.3 to 55.3); 12 patients remained with the achieved response during a mean follow up of 22 months (range: 2.3 to 25.2). Complete response was obtained in 13/23 cases (60%); mean time to response was 17 weeks (range: 0.7 to 62,3); mean duration of response was 10,1 months (range: 2.3 to 25.2); 5 patients remained with complete response after a follow up of 20 months (range: 8-29). Conclusions: A high response rate was achieved with Rituximab (75%) in patients with ITT who had failed at least to one treatment.

Differential diagnosis of IgM MGUS and WM according to B-lymphoid infiltration by morphology and flow cytometry.

The distinction between IgM monoclonal gammopathy of undetermined significance (MGUS), asymptomatic Waldenstrom's macroglobulinemia (WM; aWM), and symptomatic WM (sWM) relies on two features: the presence of infiltration by lymphoplasmacytic lymphoma in the bone marrow (BM) biopsy and the existence of signs or symptoms attributable to the disease. Nevertheless, some patients lack a BM biopsy or it is not conclusive for diagnosis. In this study we have investigated 94 patients with IgM monoclonal gammopathies, in which a BM trephine biopsy and morphological and flow cytometry (FCM) evaluation of BM aspirate were available at diagnosis. We found a clear correlation between BM infiltration of B-lymphocytes assessed by morphology and by FCM with a Pearson correlation of 0.62 (P<.001). Moreover, in the absence of a BM trephine biopsy, the cut-off points that would help in the differential diagnosis between MGUS, aWM, and sWM would be 20% for morphology and 5% for FCM, both assessed in the BM aspirate.

Síndrome de hiperinfección por strogyloides stercoralis en una paciente con infeccvión por el virus de la inmunodeficiencia humana (VIH): reporte de un caso y revisión de la literatura / Strongyloides stercoralis hyperinfection syndrome in a patient with human inmunodeficience virus infection (HIV): report of one case and review of the medical literature

Reportamos el caso de una paciente de 21 años, con la asociación síndrome de interinfección por Strongyloides stercoralis e infección por el virus de la inmunodeficiencia humana (VIH) que constituye el décimo quinto publicado en la literatura. Se revisan las hipótesis por las cuales esta asociación es infrecuente, así como las alternativas terapéuticas