Infección grave por Rickettsia conorii asociada a síndrome hemofagocítico / No disponible

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Linfohistiocitosis hemofagocítica desencadenada por el virus de Epstein-Barr en adulto con enfermedad de Still / Hemophagocytic lymphohistiocytosis associated with Epstein-Barr virus in an adult with Still disease

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Síndrome hemofagocítico secundario en pediatría: experiencia clínica en ocho casos / Pediatric secondary hemophagocytic síndrome: clinical report of eight cases

El Síndrome hemofagocítico secundario (SHS) es una entidad poco frecuente caracterizada por activación macrofágica asociada a infecciones, inmunodeficiencias o neoplasia, pudiendo presentarse como un cuadro grave y de alta letalidad. El objetivo de este estudio es describir las características clínicas de un grupo de pacientes con SHS y su evolución en relación a los tratamientos utilizados. Pacientes y Método: Análisis retrospectivo de 8 casos de SHS diagnosticados en 3 años. Resultados: Edad promedio de 6 años. Los diagnósticos de base fueron: Neoplasia (3), Artritis reumatoidea (2), Síndrome de Down (1) y 2 pacientes sin patología asociada. En todos se asoció a infecciones, documentándose agente etiológico en 4 de ellos (adenovirus, Mycoplasma pneumoniae, Streptococo viridans y Pseudomona aeruginosa). Seis pacientes recibieron gammaglobulina EV y Metilprednisolona además de los antibióticos. Fallece 1 paciente. Comentario: La sospecha precoz del SHS y el inicio de tratamiento inmunomodulador se asociaron en esta serie a respuesta favorable y menor mortalidad.

Disseminated Fusarium oxysporum infection in hemophagocytic lymphohistiocytosis.

The portal of entry of disseminated Fusarium spp. infections is still not clearly defined. We report on a disseminated Fusarium oxysporum infection occurring during a long period of severe neutropenia in a child with hemophagocytic lymphohistiocytosis. A nasogastric feeding tube was the possible source of entry of the fungus.

Infection-associated hemophagocytic syndrome in a diabetic patient undergoing chronic hemodialysis.

Hemophagocytic syndrome (HPS) is an uncommon but severe illness associated with a variety of infections, malignant tumors, and autoimmune diseases. We report a case of infection-associated HPS in a patient receiving chronic hemodialysis. Peptostreptococcus-induced sepsis and abscess formation in the left iliopsoas muscle led to the onset of infection-associated HPS in this patient. The patient had diabetes mellitus and end-stage renal disease, and it is likely that immunological dysfunctions from these disorders played a part in the onset of both severe bacterial infections and HPS.

Atypical hemophagocytic lymphohistiocytosis following bacterial tonsillitis in acute lymphoblastic leukemia.

We report a rare case of a cute lymphoblasticleukemia (ALL) who developed dyspnea, neurological disturbance with illusions, pancytopenia, phagocytosis and coagulation disturbances following bacterial tonsillitis. The values of soluble interleukin-2 receptor (sIL-2R), IL-6 and IL-8 were also elevated. Her clinicolaboratory findings were similar to hemophagocytic lymphohistiocytosis (HLH), which is a cytokine disease induced by activated T cells and macrophages. Atypical HLH following bacterial tonsillitis should be kept in mind in leukemia patients.

Mycobacterium avium complex-associated hemophagocytic syndrome in systemic lupus erythematosus patient: report of one case.

Hemophagocytic syndrome (HPS) in systemic lupus erythematosus(SLE) patients has not commonly been reported. In this case study, we report the first case of Mycobacterium avium complex (MAC)-associated hemophagocytic syndrome in a patient with systemic lupus erythematosus (SLE). This SLE patient, a 15-year-old girl, had been on a high dose of prednisolone (> 0.5mg/kg/day) for more than 3 years. She presented with a spiking fever, hepatosplenomegaly, pancytopenia, hyperferritinemia and adult respiratory distress syndrome. Bone marrow examination revealed hemophagocytosis as well as non-caseating granulomatosis. There was no indication of SLE fare-up. She responded poorly to initial treatment with methyl-prednisolone, intravenous immumoglobulin, etoposide, and drugs for Mycobacterium tuberculosis including rifampin, ethambutol, isoniazid and pyramide. However, gastric lavage culture revealed MAC. Following treatment with clarithromycin, ciprofloxacin and amikacin, her condition gradually improved and she was discharged 3 months after admission. In SLE patients with pancytopenia and hyperferritinemia, MAC-associated HPS should be considered in the differential diagnosis.

Síndrome hemofagocítico reactivo a infección por "Salmonella typhi" / Reactive hemophagocytic síndrome caused by "salmonella typhi" infection

El síndrome hemafagocítico (SHF) o histiocitosis reactiva es un trastorno de la inmunorregulación en el que, de forma primaria o secundaria, se desencadena una activación y proliferación incontrolada, no maligna de linfocitos T y macrófagos, con intensa actividad fagocítica de células hematopoyéticas. Se asocia a un estado de hipercitocinemia responsable de la mayoría de los síntomas y su diagnóstico incluye un conjunto de datos clínicos, de laboratorio y hallazgos histológicos, con hemofagocitosis prominente. Se presenta el caso de un niño afectado por síndrome hemafagocítico reactivo (SHFR) a infección por Salmonella typhi (AU)

Adverse outcomes in primary hemophagocytic lymphohistiocytosis.

PURPOSE: Hemophagocytic lymphohistiocytosis (HLH) is a rare condition characterized by abnormal proliferation of macrophages. Although the mortality rate in children diagnosed with primary HLH is high, little has been described about the nature of adverse events. This review evaluates unfavorable events in children with primary HLH to suggest methods of improving outcomes. METHODS: Charts of patients who met diagnostic criteria for primary HLH at the Hospital for Sick Children between January 1985 and June 2000 were retrospectively reviewed. The primary outcome measure was an adverse event, defined as death, the subsequent diagnosis of malignancy, or developmental delay. RESULTS: Twenty children were diagnosed with primary HLH. The median age at diagnosis was 6.5 months (range 1-78 months). Nineteen children received chemotherapy and two underwent matched sibling donor bone marrow transplantation. Of the 20 children, 12 (60%) died. These deaths were attributed to progressive HLH in 4 cases and invasive infection in 8 cases. These infections consisted of disseminated cytomegalovirus infection (n = 1), sepsis (n = 1), and invasive fungal infections (n = 6). Eight children survived. Two were subsequently diagnosed with malignancy. Two others were found to have significant developmental delay. CONCLUSIONS: The overall mortality rate was 60% in our series of 20 children with primary HLH; 50% of deaths were directly attributable to invasive fungal infection. Developmental delay and the diagnosis of malignancy are important events in this cohort.

Effective control of infection-associated hemophagocytic syndrome with chloroquine.

A retrospective study was carried out to evaluate the efficacy and the side effects of chloroquine in the treatment of 27 patients with infection-associated hemophagocytic syndrome and compared these with patients described in the literature. All patients received 20 to 25 mg/kg chloroquine per day orally for 7 days (a single course). Twenty-one patients who received one or two courses of treatment showed excellent responses; two patients showed no response to treatment. Two other patients refused to receive treatment with chloroquine. The response rate was 85.2%. Side effects were minimal and well tolerated. No relapses occurred in 19 patients. These results suggest that effective control of disease in some patients with infection-associated hemophagocytic syndrome can be achieved with chloroquine.

Acute epiglottitis: a presentation of haemophagocytic lymphohistiocytosis.

The incidence of acute epiglottitis in children has declined with the introduction of the Haemophilus influenzae b vaccine in 1992. We report a case of acute epiglottitis in a child secondary to an immunocompromised state. We suggest that when acute epiglottitis is diagnosed in a child we should ensure there is no underlying predisposing condition.

Mycoplasma pneumoniae infection, meningoencephalitis, and hemophagocytosis.

Central nervous system manifestations are a common extrapulmonary complication of Mycoplasma pneumoniae infection, of which encephalitis is a well-recognized abnormality in children. In this report the first description of M. pneumoniae infection simultaneously complicated by meningoencephalitis and hemophagocytosis is presented.

Penicilliosis-associated hemophagocytic syndrome in a human immunodeficiency virus-infected child: the first case report in children.

Infection-associated hemophagocytic syndrome (IAHS) has been found in many systemic infectious conditions with a high mortality rate. Disseminated Penicillium marneffei infection is a common opportunistic condition among HIV-infected patients in many regions in Southeast Asia. We report the first case of IAHS caused by penicilliosis in an HIV-infected child who presented with cytopenias and recovered promptly after antifungal and intravenous immunoglobulin therapy.

Disseminated histoplasmosis with reactive hemophagocytosis: aspiration cytology findings in two cases.

Two cases of disseminated histoplasmosis associated with reactive hemophagocytic syndrome are described. The clinical presentation was with prolonged unexplained fever and hepatosplenomegaly. On a strong clinical possibility of tuberculosis, antitubercular treatment was initiated in both patients. Lymph node (case 1), splenic (case 2), and bone marrow aspiration, however, showed sheets of proliferating histiocytes, and intracellular and extracellular histoplasma organisms. Aspiration cytology was thus valuable in establishing the final diagnosis. The patients had a fulminant clinical course and died of hemorrhagic shock within 48 hr of hospital admission before specific therapy could be initiated. Histoplasmosis can mimic tuberculosis clinically. There is a need for an increased awareness of the clinicopathological spectrum of histoplasmosis, especially its rarer manifestations as hemophagocytic syndrome. In suspected cases, aspirations from the lymph node, liver, and spleen can be performed safely and should be utilized for early diagnosis.

Haemophagocytic syndrome associated with infections.

Haemophagocytic syndromes (HS) are the clinical manifestation of an increased macrophagic activity with haemophagocytosis. Pathophysiology is related to a deregulation of T-lymphocytes and excessive production of cytokines. The main clinicobiological features are fever, hepatosplenomegaly, adenopathies, skin rash, neurological features, cytopenias, hypertriglyceridaemia, hyperferritinaemia and coagulopathy. Diagnosis is based on examination of the bone marrow which shows benign histiocytes actively phagocytosing haemopoietic cells. Acquired HS are mostly associated with an underlying disease such as immunodeficiency, haematological neoplasias and autoimmune diseases. Infection-associated HS was originally described by Risdall in 1979, in viral disease. Since the initial description HS has also been documented in patients with bacterial, parasitic or fungal infections. Epstein-Barr virus (EBV) is the causative agent in most cases. In EBV-associated HS, which sometimes has a fatal course, unregulated T-cell reaction or uncontrolled B-cell proliferation may release cytokines. Management of HS consists of early diagnosis, careful screening for, and prompt treatment of, infections and detection and therapy of any underlying disease. Prognosis of infection-associated haemophagocytic syndrome (IAHS) is better than that in other types of secondary HS. Management of cytokine imbalance should be useful to improve the outcome and reduce the mortality rate in these cases.

Pseudomonas sepsis with neutrophagocytosis in a premature newborn.

Secondary hemophagocytic syndrome may develop during certain severe infections commonly due to viral infections, but is rarely associated with bacterial infections, and its appearance in a premature newborn is uncommon. We present a case of hemophagocytosis during Pseudomonas aeruginosa septicemia in a premature infant. After sepsis treatment with imipenem-cilastatin and aminoglycoside, remission of hemophagocytosis was achieved.