Clinical networking results in continuous improvement of the outcome of patients with acute promyelocytic leukemia.

The introduction of all-trans retinoic acid (ATRA) combined with anthracyclines has significantly improved the outcomes for patients with acute promyelocytic leukemia (APL), and this strategy remains the standard of care in countries where arsenic trioxide is not affordable. However, data from national registries and real-world databases indicate that low- and middle-income countries (LMIC) still face disappointing results, mainly due to high induction mortality and suboptimal management of complications. The American Society of Hematology established the International Consortium on Acute Leukemias (ICAL) to address this challenge through international clinical networking. Here, we present the findings from the ICAPL study involving 806 patients with APL recruited in Brazil, Chile, Paraguay, Peru, and Uruguay. The induction mortality rate has decreased to 14.6% compared to the pre-ICAL rate of 32%. Multivariable logistic regression analysis revealed as factors associated with induction death: age ≥ 40 years, ECOG = 3, high-risk status based on the PETHEMA/GIMEMA classification, albumin level ≤ 3.5 g/dL, bcr3 PML/RARA isoform, the interval between presenting symptoms to diagnosis exceeding 48 hours, and the occurrence of central nervous system and pulmonary bleeding. With a median follow-up of 53 months, the estimated 4-year overall survival (OS) rate is 81%, the 4-year disease-free survival (DFS) rate is 80%, and the 4-year cumulative incidence of relapse (CIR) rate is 15%. These results parallel those observed in studies conducted in high-income countries, highlighting the long-term effectiveness of developing clinical networks to improve clinical care and infrastructure in LMIC.

Comparison of two different anti-infectious approaches after high-dose chemotherapy and autologous stem cell transplantation for hematologic malignancies in a 12-year period in British Hospital, Uruguay.

Autologous stem cell transplant (ASCT) is a widely used and safe procedure to treat mostly hematologic diseases. These patients are at risk of infectious complications, which represents a major cause of morbidity and it is the second cause of mortality. This retrospective 12-year analysis of the incidence, type, and severity of infections in 266 consecutive unselected ASCT patients at our institution provides novel information addressing this issue. We included 266 ASCT procedures. Patients included in the 2006-2013 period are referred to as group 1 (ciprofloxacin prophylaxis and ceftazidime-amikacin as empirical antibiotics), and those in the 2013-2017 period are group 2 (levofloxacin prophylaxis and meropenem as empirical antibiotics). The incidence of febrile neutropenia was 72% in group 1 and 86.2% in group 2 (p = 0.004). The majority of infectious episodes were associated with fever of unknown origin: 55% in group 1 and 59% in group 2. Febrile of unknown origin episodes were 82.6% in group 1 and 80% in group 2. Significant differences between both groups were found in age, hypogammaglobulinemia, and advanced disease at ASCT. No differences were found between groups regarding the most common agent documented in positive blood cultures (Gram+ were 66.6% in group 1 and 69% in group 2 (p = 0.68)). Mortality within 100 days of transplant was low, 1.87%. Regardless of the prophylactic regimen used, most patients experience febrile episodes in the ASCT setting, fever of unknown origin is the most common infection complication, and Gram+ agents are prevalent in both groups. Mortality rates were low. According to our results, ASCT is a safe procedure and there is no clear benefit in favor of levofloxacin versus ciprofloxacin prophylaxis. Both anti-infectious approaches are acceptable, yielding similar outcomes.

[Magnetic resonance as initial screening diagnosis of secondary involvement of central nervous system in NHL diffuse large B cell lymphoma] / Resonancia magnética como screening inicial de diagnóstico de compromiso secundario del sistema nervioso central en LNH difuso de grandes Células B.

Background: Background: Central Nervous System (CNS) relapse in Diffuse Large B-cell Lymphoma occurs mostly 6-8 months after disease onset. This has led to propose that CNS infiltration is an early event in the evolution of the disease. We intend to evaluate the role of magnetic resonance imaging (MR) at diagnosis to detect early SNC compromise. Methods: Prospective longitudinal cohort's study in DGCB patients treated at Hospital de Clínicas between 2013 and 2015. Skull MRI was performed in all patients at diagnosis and lumbar puncture was done according to predefined risk factors. Results: 35 patients were analyzed. Median age: 68 years (24-85 years). Stage III-IV: 62%, 57% good prognosis according to RIPI score and 43% poor prognosis. MRI was performed in all patients, with no pathological findings in any of them. Twenty-one patients fullfilled criteria for cerebrospinal fluid study. Twenty-two patients were studied and received intrathecal methotrexate prophylaxis. Meningeal relapse was observed in a single patient who had negative studies at diagnosis and had received complete prophylaxis at the end of the 6 R-CHOP series. Conclusions: Only one of the 35 patients relapsed in the CNS. This patient had a noral MRI and CSF study at diagnosis and had received prophylaxis with intrathecal chemotherapy. This results lead us to believe that the value of MRI to detect early infiltration in asymptomatic patients at diagnosis is low.

Antecedentes: la recaída en el Sistema Nervioso Central (SNC) del Linfoma No Hodgkin Difuso de Grandes Células B ocurre más frecuentemente 6-8 meses del debut de la enfermedad. Esto ha levado a plantear que la infiltración en SNC es un evento temprano en esta enfermedad. Nos proponemos evaluar el valor de la realización de estudio imagenológico por Resonancia Magnética (RM) al debut para detectar compromiso precoz. Materiales y métodos: Estudio longitudinal prospectivo de cohortes en LNH DGCB tratados en el Hospital de Clínicas entre 2013 y 2015. A todos los pacientes se les realizó RM de cráneo al debut y estudio de líquido cefaloraquídeo (LCR) según factores de riesgo predefinidos. Resultados: Se analizaron 35 pacientes. Mediana de edad: 68 años (24-85 años). Estadío III-IV: 62%; 57% presentaron buen pronóstico según score RIPI al debut y 43% mal pronóstico. Se realizó RM a todos los pacientes, no habiéndose encontrado hallazgos patológicos en ninguno de ellos. Veintitres pacientes tuvieron criterio de estudio del LCR de los cuales veintidós se estudiaron y recibieron profilaxis con metotrexate intratecal. Se observó recaída meníngea en una única paciente con estudios negativos al debut y profilaxis completa al mes de finalizadas las 6 series de R-CHOP. Conclusiones: De 35 pacientes uno sólo recayó en el SNC; el mismo había recibido profilaxis con quimioterapia intratecal y presentó una RM normal al debut de su enfermedad. Los resultados nos conducen a pensar que el valor de la RM para detectar precozmente infiltración en pacientes asintomáticos al debut es bajo.

Jak-2 positive myeloproliferative neoplasms.

Originally described by Dameshek in 1951, myeloproliferative disorders are today classified as myeloproliferative Neoplasms (MPNs) in WHO's Classification of Tumors of Hematopoietic and Lymphoid Tissues. The term includes a range of conditions, [ie, BCR-ABL-positive chronic myelogenous leukemia (CML), chronic neutrophilic leukemia (CNL), polycythemia vera (PV), primary myelofibrosis (PMF), essential thromobocythemia (ET), chronic eosinophilic leukemia not otherwise specified (CEL-NOS), mastocytosis, and unclassifiable myeloproliferative neoplasm]. In the specific case of CML, a better understanding of the pathogenesis and pathophysiology of the disease has led to a targeted therapy. The presence of chromosome Philadelphia, t(9;22)(q34;11) results in the oncogene BCR-ABL, which characterizes the disease; this molecular rearrangement gives rise to a tyrosine-kinase, which in turn triggers the proliferation of the myeloid line through the activation of the signaling pathways downstream. Tyrosine-kinase inhibitors (TKIs) have altered the therapy and monitoring of CML patients and improved both their prognosis and quality of life. In 2005, various groups of investigators described a new point mutation of the gene JAK2 associated to MPNs. Although the presence of this mutation has led to a modification in the diagnostic criteria of these conditions, the impact of the use of JAK2 inhibitors on the prognosis and course of the disease continues to be controversial.