ABSTRACT
Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is a highly lethal hyper-inflammatory disorder that leads to a storm of cytokines, hemophagocytosis and multiple organ failure. It can be primary, which is inherited, or secondary. In the latter, virus infections are a frequent trigger, predominantly the family of herpes viruses, such as Epstein-Barr virus. An early treatment is recommended. Until recently there was no consensus about the management of secondary cases. The protocols for the treatment of primary HLH were used, which include cytotoxic agents and corticosteroids. We herein review the current diagnostic and therapeutic approach of HLH, based on a case associated with a reactivation of the Epstein-Barr virus in an immunocompetent adolescent. We highlight the importance of suspecting this disease in patients with a persistent inflammatory response state or with a fever of unknown origin, in order to carry out a timely treatment, with the least toxicity, and appropriate to the characteristics of each individual, which is the current therapeutic trend.
Subject(s)
Humans , Lymphohistiocytosis, Hemophagocytic , Cytokines , Adrenal Cortex Hormones , Epstein-Barr Virus InfectionsABSTRACT
The objective of high activity antiretroviral therapy (HAART) in patients with AIDS, is to obtain immune restoration. This means a reduction of the viral load and restitution of the CD4 cell count. A decreased rate of HIV replication improves both the number and function of CD4 cells. Nevertheless, this treatment sometimes results in the reappearance of previous symptoms from treated conditions due to opportunistic infections (ie: tuberculosis, criptococcosis, hepatitis, Pneumocystis jirovesi, toxoplasmosis, etc) or non infectious condition such as sarcoidosis, Graves disease or Kaposi sarcoma. This is known as Inflammatory Reconstitution Immune Syndrome (IRIS). We report a 37 year-old woman in stage C3-AIDS with a previous criptococcal meningitis. She was treated, achieving a marked improvement with treatment and subsequent suppressive therapy with fluconazole 200 mg/day. IRIS appeared after 8 months of ongoing antiretroviral therapy with immune restoration with the development of aseptic meningitis and intracranial hypertension. The opportunistic agent could not be identified by cultures. Additional laboratory tests excluded toxoplasmosis, tuberculosis, bacterial cerebral abscesses, syphilitic cerebral gummas, and lymphoma. Brain CT and magnetic resonance studies were compatible with brain vasculitis and leptomeningitis. The patient condition improved with general measures, such as a repeated lumbar punctures and non steroidal anti-inflammatory drugs. We conclude that this patient had an IRIS due to a Cryptococcus neoformans antigen.
Subject(s)
Adult , Female , Humans , AIDS-Related Opportunistic Infections/chemically induced , Acquired Immunodeficiency Syndrome/drug therapy , Antiretroviral Therapy, Highly Active/adverse effects , Immune Reconstitution Inflammatory Syndrome/complications , Meningitis, Cryptococcal/chemically induced , AIDS-Related Opportunistic Infections/cerebrospinal fluid , Cryptococcus neoformans , Immune Reconstitution Inflammatory Syndrome/cerebrospinal fluid , Immune Reconstitution Inflammatory Syndrome/immunology , Meningitis, Cryptococcal/cerebrospinal fluid , Viral LoadABSTRACT
La terapia antiretroviral (TARV) de alta actividad cambió la epidemiología de la neumonía por Pneumocystis jiroveci (NPj) en pacientes con SIDA. La incidencia global ha descendido y ahora prevalece en pacientes sin TARV o con fracaso de ésta. Además, la restauración inmune por TARV genera una forma de NPj incluida en los síndromes inflamatorios por restauración inmune (SIRI). A fines del 2004, 75,5% de pacientes con infección por VIH en control en el Hospital de Enfermedades Infecciosas Dr. Lucio Córdova tenían TARV. Esta serie describe las características clínicas de la NPj, comparando el grupo con TARV (n: 6) y sin TARV (n: 12). De aquellos con TARV, 83,3% (5/6) estaban con fracaso inmunológico y 16,7% (1/6) en éxito virológico. El recuento de CD4 fue bajo en ambos grupos (mediana 20 céls/mm3 sin TARV y 51 céls/mm3 con TARV). No hubo diferencia en la mayoría de las características de la NPj, tampoco casos de SIRI. El grupo con TARV tuvo menos severidad y complicaciones, y menos indicación de corticoterapia (p 0,023).