Manifestaciones relacionadas con alteraciones en la coagulación en lupus eritematoso sistémico de inicio juvenil. Reporte de casos y revisión de la literatura / Manifestations related to alterations in coagulation in systemic lupus erythematosus of juvenile onset. Case report and literature review

RESUMEN Las alteraciones hematológicas son comunes en los pacientes con lupus eritematoso sistémico (LES). Pueden expresarse relacionadas con el compromiso de las líneas celulares y con la presencia de alteraciones de la coagulación. El compromiso en la coagulación se asocia con manifestaciones trombóticas. Se han descrito factores de riesgo asociados a trombosis, como la presencia de niveles elevados de homocisteína, déficit adquirido de la proteína S, proteína C y antitrombina. Sin embargo, la diátesis hemorrágica también se ha descrito con menor frecuencia y relacionada con el déficit de factores de la coagulación, secundaria a la presencia de inhibidores. Presentamos 3 pacientes con LES juvenil con manifestaciones hematológicas poco usuales y revisión de la literatura relacionada. Se concluye que las manifestaciones hematológicas en LES juvenil no solo se relacionan con alteraciones en las líneas celulares. Trombosis vasculares y trastornos hemorrágicos deben sospecharse. El diagnóstico precoz y el tratamiento temprano disminuyen la morbimortalidad relacionada con este tipo de manifestaciones.

ABSTRACT Haematological alterations are common in patients with systemic lupus erythematosus (SLE). These haematological manifestations may be expressed related to the involvement of cells affected and coagulation changes. The compromise in coagulation is associated with thrombotic manifestations. Risk factors associated with thrombosis have been described, such as the presence of elevated levels of homocysteine, acquired deficit of protein S, protein C, and antithrombin. However, the haemorrhagic diathesis has also been described at a lower frequency and related to the acquired deficiency of coagulation factors caused by the development of autoantibodies directed against coagulation factors. The cases are presented of 3 patients with juvenile SLE with unusual haematological manifestations, as well as a review of the literature in relation to them. The haematological manifestations in juvenile SLE are not only related to alterations in cell lines, vascular thrombosis and bleeding disorders should also be suspected. Early diagnosis and treatment reduces morbidity and mortality related to this type of manifestations.

A strategy to improve treatment-related mortality and abandonment of therapy for childhood ALL in a developing country reveals the impact of treatment delays.

BACKGROUND: Treatment-related mortality and abandonment of therapy are major barriers to successful treatment of childhood acute lymphoblastic leukemia (ALL) in the developing world. PROCEDURE: A collaboration was undertaken between Instituto Nacional de Cancerologia (Bogota, Colombia), which serves a poor patient population in an upper-middle income country, and Dana-Farber/Boston Children's Cancer and Blood Disorders Center (Boston, USA). Several interventions aimed at reducing toxic deaths and abandonment were implemented, including a reduced-intensity treatment regimen and a psychosocial effort targeting abandonment. We performed a cohort study to assess impact. RESULTS: The Study Population comprised 99 children with ALL diagnosed between 2007 and 2010, and the Historic Cohort comprised 181 children treated prior to the study interventions (1995-2004). Significant improvements were achieved in the rate of deaths in complete remission (13% to 3%; P = 0.005), abandonment (32% to 9%; P < 0.001), and event-free survival with abandonment considered an event (47% to 65% at 2 years; P = 0.016). However, relapse rate did not improve. Medically unnecessary treatment delays were common, and landmark analysis revealed that initiating the PIII phase of therapy ≥4 weeks delayed predicted markedly inferior disease-free survival (P = 0.016). Conversely, patients who received therapy without excessive delays had outcomes approaching those achieved in high-income countries. CONCLUSIONS: Implementation of a twinning program was followed by reductions in abandonment and toxic deaths, but relapse rate did not improve. Inappropriate treatment delays were common and strongly predicted treatment failure. These findings highlight the importance of adherence to treatment schedule for effective therapy of ALL.

Linfoma de Burkitt en un niño con infección por VIH / Report of a non-hodkin lymphoma in a child with HIV infection

La asociación de cáncer y sida en el paciente pediátrico es de muy baja incidencia. Sin embargo, el linfoma No Hodgkin (LNH) es el tumor más común en el paciente VIH-positivo. El caso reportado es el de un niño de 5 años de edad con diagnóstico de LNH,estado IV(por compromiso de médula ósea y de sistema nervioso central, con el antecedente de padres con VIH, el cual fue confirmado en el paciente. El ,manjeo de la quimioterpia fue difícil debido a la toxicidad combinada (terapia antineoplásica y antirretrovirales), por lo que el paciente presentó recaída durante el tratamiento y falleció. Este tipo de patología ha venido en aumento en los últimos años y cada caso aporta experiencia para establecer pautas en cuanto al diagnóstico y al tipo de terapia óptima.(au)