Síndrome hemofagocítico asociado a varicela / Hemofagocytic syndrome associated with varicella

Presentación del caso. Lactante femenina de 14 meses de edad con desarrollo psicomotor normal, sin comórbidos. Con historia de un día de fiebre de 40 °C, intermitente, acompañada de evacuaciones diarreicas y vómitos. Fue llevada por sus padres a una clínica privada sin notar mejoría con el tratamiento médico indicado. Posteriormente, presentó deterioro clínico y fue llevada a un hospital, donde se diagnosticó un síndrome febril agudo, diarrea con deshidratación leve y faringitis. Al cuarto día de evolución inició con máculas y pápulas que progresaron a vesículas y costras. Además, presentó intolerancia a la vía oral, disnea, distensión abdominal, coma y desequilibrio hidroelectrolítico. Intervención terapéutica. Inició el tratamiento con hidratación parenteral, antivirales, esteroides endovenosos y antihistamínicos; se diagnosticó shock séptico con compromiso respiratorio, se proporcionó ventilación mecánica asistida y fue referida al hospital de tercer nivel para atención por medicina crítica. Los estudios reportaron un derrame pleural derecho del 40 % y hepatomegalia. Continuó el tratamiento con antibiótico terapia, hidratación parenteral, antivirales, diuréticos, antipiréticos y hemoderivados, presentó mejoría, continuó el manejo terapéutico. Evolución clínica. El día 18 presentó fiebre, hepatoesplenomegalia, los exámenes reportaron elevación de ferritina, triglicéridos y citopenia se diagnosticó un síndrome hemofagocítico que evolucionó con una falla multisistémica y falleció al siguiente día

Case presentation. A 14-month-old female infant with normal psychomotor development, without comorbidities. With a one-day history of fever of 40 °C, intermittent, accompanied by diarrhea and vomiting. She was taken by her parents to a private clinic without improvement with the indicated medical treatment. Subsequently, she presented clinical deterioration and was taken to a hospital, where she was diagnosed with acute febrile syndrome, diarrhea with mild dehydration, and pharyngitis. On the fourth day of evolution, she started with macules and papules that progressed to vesicles and crusts. In addition, she presented oral intolerance, dyspnea, abdominal distension, coma, and hydro electrolytic imbalance. Therapeutic intervention. She started treatment with parenteral hydration, antivirals, intravenous steroids, and antihistamines; septic shock with respiratory distress was diagnosed, assisted mechanical ventilation was provided, and she was referred to a tertiary hospital for critical care medicine. Studies reported a 40 % right pleural effusion and hepatomegaly. She continued treatment with antibiotic therapy, parenteral hydration, antivirals, diuretics, antipyretics, and hemoderivatives, presented improvement, and continued therapeutic management. Clinical evolution. On day 18 he presented fever and hepatosplenomegaly. Tests reported elevated ferritin, triglycerides, and cytopenia, and was diagnosed with hemophagocytic syndrome that evolved with multisystemic failure and died the following day

Síndrome hemofagocítico en pacientes con leucemia linfocítica crónica tratados con ibrutinib. A propósito de dos casos / Haemophagocytic syndrome in patient with chronic lymphocytic leukaemia treated with ibrutinib. Two cases report

Se presentan dos casos de sospecha de síndrome hemofagocítico secundario en pacientes con leucemia linfocítica crónica, tratados con ibrutinib, síndrome que puede ser letal y que tiene lugar por una activación inmune excesiva. En el presente artículo se describe el desarrollo del síndrome, su diagnóstico, el tratamiento y su desenlace, acorde a las guías y protocolos de diagnóstico, actuación y tratamiento. Es destacable por el hecho de ser una reacción adversa rara de la que no se conoce muy bien la causa, multifactorial, de diferente inicio y evolución, como ocurre en los dos casos que se presentan, no recogida en ficha técnica. Se sitúan los casos junto a un pequeño número de casos también publicados. (AU)

We presented two cases of suspected secondary haemophagocytic syndrome in patients with chronic lymphocytic leukaemia treated with ibrutinib, syndrome that could be letal and that occurs due to excessive immune activation. This article describes the evolution, diagnosis, treatment, and denouement, according to protocols and guidelines for diagnosis and therapeutic decision-making. It is noteworthy, because is a rare adverse event, not present in product monograph, whose cause is not weel known, multifactorial, with different onset and evolution, as in the two cases presented. The cases are placed next to a small number of publicated cases. (AU)

Síndrome hemofagocítico secundario a infección por virus del dengue / Hemophagocytic syndrome secondary to dengue virus infection

Introducción: El síndrome hemofagocítico se presenta como un cuadro clínico grave, provocado por una respuesta inadecuada del sistema inmunológico a un desencadenante infeccioso, neoplásico, reumatológico o metabólico, que origina una reacción inflamatoria no controlada; presenta una incidencia baja pero la letalidad sin el manejo adecuado es muy elevada. Objetivo: Destacar la importancia de diagnóstico oportuno del síndrome hemofagocítico en pacientes con dengue que presentan evolución tórpida. Presentación del caso: Paciente de 7 años de edad, con dengue grave dado por shock, hepatomegalia con elevación de transaminasas, con mala evolución clínica, quien cumple criterios de Síndrome hemofagocítico. Recibió manejo con inmunomoduladores con evolución satisfactoria. Conclusiones: Es importante considerar el Síndrome hemofagocítico como causa ante enfermedades con evolución tórpida a pesar de tener un manejo médico correcto(AU)

Introduction: Hemophagocytic syndrome is a severe clinical picture with an uncontrolled inflammatory reaction caused by an inadequate immune system response to an infectious, neoplastic, rheumatological, or metabolic trigger. The syndrome has low incidence but high fatality when the management is not adequate. Objective: To highlight the importance of a prompt diagnosis of hemophagocytic syndrome in patients with dengue who present a torpid evolution. Case presentation: Seven-year-old patient with severe dengue caused by shock, hepatomegaly with elevated transaminase levels and poor clinical evolution who meets hemophagocytic syndrome criteria. The patient had satisfactory progression after receiving immunomodulatory treatment. Conclusions: Hemophagocytic syndrome must be considered as a cause of pathologies in dengue patients with torpid evolution, even when correct medical management is made(AU)

Síndrome hemofagocítico en pediatría, reporte de un caso / Hemophagocytic syndrome in pediatrics: a case report

RESUMEN Introducción: el síndrome hemofagocítico es una grave enfermedad que se caracteriza por la activación exagerada del sistema inmune y aumento de la actividad linfocítica citotóxica y macrofágica, que puede ser fatal. Presentación del caso: lactante de tres meses de edad, femenina, blanca, nacida producto de un embarazo normal, parto eutócico a las 39 semanas de gestación, con un peso al nacer de 3,6Kg, Apgar 9:9, sin complicaciones pre, peri, ni post natal. Es remitida al hospital por presentar vómitos, somnolencia, febrícula y pobre progreso de peso, al examen físico se constata visceromegalia, en los complementarios realizados se reciben las aminotransferasas en cifras muy elevadas, TGP: 1540 y TGO: 1590, así como la FA 800 UI/L, GGT 100UI/L y triglicéridos 2mmol/L, hemograma con una leucocitosis 19 x 109 /L con un 82 % de linfocitos, en el medulograma se comprueba la actividad macrofágica intramedular. Se inicia el tratamiento según protocolo del mismo, no muestra mejoría, evoluciona tórpidamente hacia la disfunción múltiple de órganos y fallece. Conclusiones: el síndrome hemofagocítico es infrecuente y requiere un alto índice de sospecha. El conocimiento de los criterios presentes en esta enfermedad permitiría el pensamiento diagnóstico y la terapéutica precoz y efectiva para los pacientes, con una mayor supervivencia.

ABSTRACT Introduction: hemophagocytic syndrome is a serious disease characterized by exaggerated activation of the immune system and increased cytotoxic and macrophagic-lymphocytic activity, which can be potentially fatal. Case report: 3-month-old female infant, white skin, born of a normal pregnancy, eutocic delivery at 39 weeks of gestation, with a birth weight of 3,6 kg, Apgar 9:9, without pre-, peri- or post-natal complications. She was referred to the hospital because of vomiting, somnolence, fever and poor weight gain, the physical examination showed visceromegaly, in the complementary tests the aminotransferases were very high, TGP: 1540 and TGO: 1590, as well as FA 800 UI/L, GGT 100UI/L and triglycerides 2mmol/L, hemogram with leukocytosis 19 x 109 /L with 82 % of lymphocytes, in the medullogram an intramedullary macrophage activity is verified. Treatment was initiated according to protocol, but she did not show improvement, evolving torpidly to a multiple organ failure and died. Conclusions: hemophagocytic syndrome is infrequent and requires a high index of suspicion. Knowledge of the criteria present in this condition would allow the diagnostic thinking, as well as the early and effective therapeutic for patients, with better survival rate.

Fallo multiorgánico en pacientes portadores del virus de inmunodeficiencia humana, ¿cuándo pensar en Síndrome Hemofagocítico?. A propósito de dos casos clínicos

Resumen: El síndrome hemofagocítico es una enfermedad infrecuente y de alta mortalidad. El término hemofagocitosis describe la presencia de macrófagos activados que incorporan dentro de su citoplasma eritrocitos, leucocitos y plaquetas. Cuando esto ocurre en el contexto de una respuesta inmune exagerada e ineficaz, se denomina síndrome hemofagocítico. Se clasifica en primario cuando se asocia a alteraciones genéticas generalmente hereditarias, y secundario, más frecuente en adultos, cuando se desencadena por alguna enfermedad o condición subyacente. El diagnóstico requiere de un alto grado de sospecha y se realiza según criterios diagnósticos establecidos, siendo la sepsis el principal diagnóstico diferencial. Se comentan dos casos de hombres jóvenes con infección por virus de la inmunodeficiencia humana en estadío avanzado, que se presentan con fiebre y fallo multiorgánico, en los que se hace el diagnóstico de síndrome hemofagocítico secundario.

Abstract: The hemophagocytic syndrome is an infrequent and fatal disease. The concept of hemophagocytosis describes the presence of activated macrophages that incorporate within their cytoplasms erythrocytes, leukocytes and platelets. When it occurs in the context of an exaggerated and inefficient immune response, it is called hemophagocytic syndrome. It is classified in primary when it is associated with genetic alterations generally hereditary, and secondary, more frequent in adults, when it is triggered by a disease or an underlying condition. The diagnosis requires a high grade of suspicion and is based on established diagnostic criteria, being sepsis the main differential diagnosis. We present two cases of young men with advanced infection by the human immunodeficiency virus, who developed fever and multiorgan failure, arriving at the diagnosis of secondary hemophagocytic syndrome.

Resumo: A síndrome hemofagocítica é uma doença rara e com alta mortalidade. O termo hemofagocitose descreve a presença de macrófagos ativados que incorporam eritrócitos, leucócitos e plaquetas em seu citoplasma. Quando ocorre no contexto de uma resposta imune exagerada e ineficaz, é chamada de síndrome hemofagocítica. É classificada como primária quando é associada a alterações genéticas geralmente hereditárias, e secundária quando é desencadeada por uma doença ou condição de base, mais frequente em adultos. O diagnóstico exige alto grau de suspeita e é feito de acordo a critérios com diagnósticos estabelecidos, sendo a sepse o principal diagnóstico diferencial. São discutidos dois casos de homens jovens com infecção pelo vírus da imunodeficiência humana em estágio avançado, apresentando febre e falência de múltiplos órgãos, fazendo o diagnóstico de síndrome hemofagocítica secundária.

Haemophagocytic syndrome associated with infections: Not so uncommon. / Síndrome hemofagocítico asociado a infecciones: no tan infrecuente.

BACKGROUND: Haemophagocytic syndrome (HPS) is a severe immunological disorder characterised by uncontrolled inflammation and multiple organ failure. HPS can be triggered by viral, bacterial, fungal and parasitical infections. We report our experience with infection-related HPS and estimate its local incidence. MATERIAL AND METHOD: We conducted an observational retrospective study of infection-associated HPS in patients treated in the Department of Infectious Diseases of a university hospital within a 5-year period, as well as a review of the published series in Europe. RESULTS: HPS was associated with infection by cytomegalovirus in 2 women with Crohn's disease and was associated with visceral leishmaniosis in 4 patients (3 men, 1 woman; 1 case of multiple myeloma; 2 cases of solid tumours; 1 case of no apparent disease). Two patients died, and the estimated incidence rate was 0.58/100,000 inhabitants/year. The published series are mixed. CONCLUSIONS: Infection-related HPS must be more common than reported. The geographical environment can influence the triggering infections (in our environment, Leishmania should be considered).

[Disseminated histoplasmosis and haemophagocytic syndrome in HIV patients: A case series in a Peruvian hospital]. / Histoplasmosis diseminada y síndrome hemofagocítico en pacientes VIH: serie de casos en un hospital peruano.

BACKGROUND: Disseminated histoplasmosis (DH) is an opportunistic fungal infection in severely immunocompromised patients with HIV infection. Haemophagocytic syndrome (HFS), which can occur in these co-infected patients when the immune response is significantly altered, is often associated with high mortality. AIMS: To describe the epidemiological, clinical, analytical and microbiological characteristics, along with studying the presence of HFS, in patients with DH-HIV. METHODS: A retrospective study was conducted on a case series using data from the clinical records of patients diagnosed with DH and HIV infection during the years 2014 and 2015. RESULTS: DH was diagnosed in 8 (1.3%) of 597 HIV patients. All patients were in stage C3, and 75% (6/8) were not receiving combined antiretroviral therapy (CART). The remaining two patients had recently begun CART (possible immune reconstitution syndrome). Five (62.5%) of the 8 patients met criteria for HFS. The most frequent clinical symptoms were lymphoproliferative and consumptive syndrome, respiratory compromise, and cytopenia. Histoplasma was isolated in lymph nodes of 75% (6/8) of the patients, in blood samples in 25% (2/8), and also in intestinal tissue in one patient. The antifungal therapy was amphotericin B deoxycholate, without adjuvants. The overall mortality was 50%. CONCLUSIONS: In this case series, DH-HIV co-infection frequently progressed to HFS with high mortality. The clinical picture may resemble that of other systemic opportunistic infections, such as tuberculosis, or can take place simultaneously with other infections. Clinical suspicion is important in patients with severe cytopenia and lymphoproliferative and consumptive syndrome in order to establish an early diagnosis and prescribing a timely specific therapy.

Síndrome hemofagocítico. Reporte de un caso y revisión de la literatura / Hemophagocytic syndrome. Case report and review of the literature

Resumen El síndrome hemofagocítico es una enfermedad caracterizada por fiebre, citopenias, esplenomegalia y otras alteraciones en laboratorios debido a una activación excesiva de los macrófagos, debido a mutaciones genéticas o secundario a infecciones, malignidad o enfermedades reumatológicas. Debido a la poca especificidad de los síntomas, el diagnóstico usualmente se realiza de manera tardía. Su manejo requiere el tratamiento de la causa subyacente, y de ser necesario, medicamentos que disminuyan la respuesta inflamatoria.

Abstract Hemophagocytic syndrome is a disease characterized by fever, cytopenia, splenomegaly and other alterations in laboratories due to excessive activation of macrophages, by genetic mutations or secondary to infections, malignancy or rheumatological diseases. Because of the low specificity of the symptoms, the diagnosis is usually late. Its management requires the treatment of the underlying cause and, if necessary, medications that decrease the inflammatory response.

Diagnóstico postmortem de un caso con síndrome hemofagocítico secundario / Postmortem diagnosis of a case with secondary hemophagocytic syndrome

Introducción: El síndrome hemofagocítico, llamado también linfohistiocitosis hemofagocítica o síndrome de activación macrofágica, es una grave enfermedad que se caracteriza por la activación exagerada del sistema inmune y aumento de la actividad linfocítica citotóxica y macrofágica, que puede ser potencialmente fatal. Objetivo: Describir un caso con este síndrome poco frecuente pero de alta mortalidad. Presentación del caso: Paciente de 10 meses, nacido de parto eutócico, a término, normopeso, con antecedentes de ingreso a los dos meses por sepsis, con aumento de las transaminasas y adenopatías cervicales. Se realizó biopsia del ganglio cervical y se diagnosticó adenitis granulomatosa. En esta ocasión, cuatro días antes del ingreso comenzó con fiebre y decaimiento; al examen físico presentó tiraje intercostal bajo, polipnea superficial, hepatomegalia y esplenomegalia. Exámenes complementarios, presentó anemia, transaminasas, albúmina y proteínas totales elevadas; orina con pigmentos biliares y cuerpos cetónicos positivos; plaquetas 100 x 109. Los especialistas de gastroenterología plantearon una colestasis del lactante. El paciente falleció y en la necropsia se constató una hepatoesplenomegalia, hígado amarillento, adenopatías mesentéricas y peripancreáticas, pulmones hemorrágicos con aumento de consistencia; en el estudio microscópico se encontró en hígado, bazo, médula ósea, y ganglios linfáticos, histiocitos con hemofagocítosis. Conclusiones: El síndrome hemofagocítico es una enfermedad poco frecuente que muchas veces no se sospecha y pasa inadvertido, por lo tanto hay que pensar en dicha entidad porque tiene implicaciones pronósticas graves para el paciente, como puede ser un desenlace fatal(AU)

Introduction: The hemophagocytic syndrome, also called hemophagocyticlymphohistiocytosis or macrophage activation´s syndrome is a serious disease characterized by exaggerated activation of the immune system and increased cytotoxic lymphocytic and macrophage activity, which can be potentially fatal. Objective: To describe a case with this rare syndrome with a high mortality rate, and the diagnosis was made postmortem in our hospital. Case presentation: A 10-month-old patient, born by natural delivery, with normal weight, with a history of admission at 2 months due to sepsis, with increased transaminases levels and cervical adenopathies. A cervical lymph node biopsy was performed, and granulomatous adenitis was diagnosed. On this occasion, four days before admission, he presented fever and weakness; physical examination revealed low intercostals retraction, superficial polypnea, hepatomegaly and splenomegaly. The complementary tests showed anemia, transaminases, albumin and total proteins with high levels; urine had bile pigments and positive ketone bodies; platelets 100 x 109. Gastroenterology specialists set out cholestasis of the infant. The patient died and at necropsy there were evidences of hepato splenomegaly, yellowish liver, mesenteric and peripancreatic adenopathies, hemorrhagic lungs with increased consistency. In the microscopic study, histiocytes with hemophagocytosis were found in liver, spleen, bone marrow, and lymph nodes. Conclusions: The hemophagocytic syndrome is a rare disease that often goes unnoticed. It has serious prognostic implications for the patient with a fatal outcome(AU)

Diagnóstico postmortem de un caso con síndrome hemofagocítico secundario / Postmortem diagnosis of a case with secondary hemophagocytic syndrome

Introducción: El síndrome hemofagocítico, llamado también linfohistiocitosis hemofagocítica o síndrome de activación macrofágica, es una grave enfermedad que se caracteriza por la activación exagerada del sistema inmune y aumento de la actividad linfocítica citotóxica y macrofágica, que puede ser potencialmente fatal. Objetivo: Describir un caso con este síndrome poco frecuente pero de alta mortalidad. Presentación del caso: Paciente de 10 meses, nacido de parto eutócico, a término, normopeso, con antecedentes de ingreso a los dos meses por sepsis, con aumento de las transaminasas y adenopatías cervicales. Se realizó biopsia del ganglio cervical y se diagnosticó adenitis granulomatosa. En esta ocasión, cuatro días antes del ingreso comenzó con fiebre y decaimiento; al examen físico presentó tiraje intercostal bajo, polipnea superficial, hepatomegalia y esplenomegalia. Exámenes complementarios, presentó anemia, transaminasas, albúmina y proteínas totales elevadas; orina con pigmentos biliares y cuerpos cetónicos positivos; plaquetas 100 x 109. Los especialistas de gastroenterología plantearon una colestasis del lactante. El paciente falleció y en la necropsia se constató una hepatoesplenomegalia, hígado amarillento, adenopatías mesentéricas y peripancreáticas, pulmones hemorrágicos con aumento de consistencia; en el estudio microscópico se encontró en hígado, bazo, médula ósea, y ganglios linfáticos, histiocitos con hemofagocítosis. Conclusiones: El síndrome hemofagocítico es una enfermedad poco frecuente que muchas veces no se sospecha y pasa inadvertido, por lo tanto hay que pensar en dicha entidad porque tiene implicaciones pronósticas graves para el paciente, como puede ser un desenlace fatal(AU)

Introduction: The hemophagocytic syndrome, also called hemophagocyticlymphohistiocytosis or macrophage activation´s syndrome is a serious disease characterized by exaggerated activation of the immune system and increased cytotoxic lymphocytic and macrophage activity, which can be potentially fatal. Objective: To describe a case with this rare syndrome with a high mortality rate, and the diagnosis was made postmortem in our hospital. Case presentation: A 10-month-old patient, born by natural delivery, with normal weight, with a history of admission at 2 months due to sepsis, with increased transaminases levels and cervical adenopathies. A cervical lymph node biopsy was performed, and granulomatous adenitis was diagnosed. On this occasion, four days before admission, he presented fever and weakness; physical examination revealed low intercostals retraction, superficial polypnea, hepatomegaly and splenomegaly. The complementary tests showed anemia, transaminases, albumin and total proteins with high levels; urine had bile pigments and positive ketone bodies; platelets 100 x 109. Gastroenterology specialists set out cholestasis of the infant. The patient died and at necropsy there were evidences of hepato splenomegaly, yellowish liver, mesenteric and peripancreatic adenopathies, hemorrhagic lungs with increased consistency. In the microscopic study, histiocytes with hemophagocytosis were found in liver, spleen, bone marrow, and lymph nodes. Conclusions: The hemophagocytic syndrome is a rare disease that often goes unnoticed. It has serious prognostic implications for the patient with a fatal outcome(AU)

[Haemophagocytic syndromes: The importance of early diagnosis and treatment]. / Síndromes hemofagocíticos: la importancia del diagnóstico y tratamiento precoces.

Haemophagocytic syndrome, or haemophagocytic lymphohistiocytosis (HLH), is a disorder with high mortality, typically recognised at paediatric age. Without proper treatment, HLH can be fatal. The risk of a rapid progression to multi-organ failure and central nervous system involvement leading to long-term sequelae, are the most feared consequences of a diagnostic delay. Therefore, HLH is a medical emergency that paediatricians should be able to identify in a patient with fever and progressive worsening of general condition. The application of the HLH diagnostic criteria, which include clinical and analytical data (as well as a bone marrow aspirate), and the search for a trigger (infectious, oncological, rheumatological, or metabolic). These are decisive for the establishment of a targeted treatment, which aims at neutralising the trigger and reducing the hyper-inflammation. The most relevant data for general paediatricians are presented in this review, including the physiopathology, diagnosis, and treatment of this serious disease.

Hemofagocitosis secundaria a dengue / Hemophagocytosis secondary to dengue fever

La infección por el virus del dengue constituye un problema de salud pública mundial. Causada por un virus de la familia Flaviviridae, presenta un amplio espectro clínico, desde formas asintomáticas frecuentes hasta las formas graves de fiebre hemorrágica y shock por fuga capilar. Existen cuatro serotipos; los serotipos 2 y 3 están asociados a las formas graves de la enfermedad. El diagnóstico definitivo de infección por dengue depende del aislamiento del virus en sangre, de la detección del antígeno viral o el ARN viral en suero o tejido, o detección de anticuerpos específicos. El síndrome hemofagocítico se produce ante la desregulación del sistema inmune que lleva a la activación macrofágica descontrolada, y se manifiesta con alteraciones clínicas, hematológicas, bioquímicas e histológicas. La asociación entre ambas entidades se encuentra descripta como una forma inusual y grave de presentación que responde a la tormenta de citocinas liberadas durante la enfermedad. Presentamos el caso de un paciente adulto con dengue, hemofagocitosis y valores inusualmente elevados de ferritina en sangre que evolucionó favorablemente con tratamiento de soporte.

Dengue virus infection constitutes a major public health problem worldwide. It is caused by a virus belonging to the Flaviviridae family. It produces a wide range of clinical presentations, from asymptomatic infection to severe forms of the disease with hemorrhagic fever or shock secondary to capillary leak syndrome. Four serotypes have been described; serotype 2 and serotype 3 are associated with the most severe forms of the disease. The diagnosis is based on laboratory tests aimed to detect antibodies, viral RNA, or antigens in serum. The hemophagocytic syndrome is generated by a dysfunction of the immune system with clinical, hematological, biochemical and histological manifestations. The association between these two entities is described as an unusual and severe presentation of dengue fever. We present a case of an adult patient with this association and very high blood levels of ferritin, who responded favorably to supportive care.

Síndrome hemofagocítico secundario a leishmaniasis visceral en un lactante mayor: reporte de caso / Hemophagocytic síndrome secondary to visceral leishmaniasis in a toddler: report of a case

El síndrome hemofagocítico está caracterizado por la activación anómala del sistema inmune. De etiología primaria o secundaria a enfermedades infecciosas, metabólicas, inmunitarias o neoplásicas. Con incidencia de 0,12 a 1 casos por cada 100 000 niños y una sobrevida de dos meses de no recibir tratamiento. Se diagnostica con 5 de 8 criterios propuestos por la Asociación Internacional de Histocitosis, y tratamiento según etiología. . Se presenta el caso de un paciente masculino de 15 meses de edad, procedente una zona endémica de leishmaniasis (Choluteca, Honduras), que ingresó al Hospital Escuela Universitario con cuadro clínico de fiebre prolongada y hepatoesplenomegalia. En los exámenes auxiliares de detectó: pancitopenia, serología positiva para leishmania, hipertrigliceridemia y valor de ferritina aumentada. Se hizo el diagnóstico de leishmaniasis visceral y síndrome hemofagocítico. Se trató con antimoniato de meglumina por 28 días, metilprednisolona y prednisona; con evolución favorable. El síndrome hem ofagocítico podría ocurrir secundario a leishmaniasis visceral por lo que su diagnóstico debería sospecharse tempranamente para un tratamiento oportuno y mejor pronóstico

The hemophagocytic syndrome is characterized by an abnormal activation of the immune system. This condition may be primary or secondary to infectious, metabolic, immunological, or malignant conditions. Its incidence is 0.12 to 1 case per 100,000 children, and the survival time is around two months with no therapy. The condition is diagnosed with the occurrence of 5 of 8 criteria proposed by the International Histiocytosis Association, and its therapy is according the original etiology. We present the case of a 15-month old male patient, who was brought from Choluteca (an endemic area for leishmaniasis), who was admitted to the University Hospital with prolonged fever and hepatosplenomegaly. Laboratory tests showed pancytopenia, positive serology for Leishmania, hypertriglyceridemia, and increased ferritin levels. Visceral leishmaniasis and hemophagocytic syndrome were diagnosed. The patient received meglumine antimoniate for 28 days, and also methylprednisolone and prednisone; and he had a favorable outcome. The hemophagocytic syndrome may be secondary to visceral leishmaniasis, and its diagnosis should be promptly suspected in order to have timely therapy and a better prognosis

Síndrome hemofagocítico: una rara complicación en el paciente con infección por el virus de inmunodeficiencia humana (VIH) / Hemophagocytic syndrome: a rare complication in patients with human immunodeficiency virus (HIV) infection

Resumen: El síndrome hemofagocítico es una enfermedad infrecuente y grave caracterizada por un estado de hiperinflamación sistémica con sobreproducción de citocinas. Puede responder a causas genéticas (primario) o desencadenarse por infecciones, fármacos, neoplasias o enfermedades autoinmunes. Existen criterios diagnósticos establecidos. El tratamiento consiste en el bloqueo de la respuesta inflamatoria sistémica, asociado al tratamiento de la causa desencadenante cuando se halla. La mortalidad es alta y usualmente está en relación a la causa que gatilla el fenómeno. Se presenta un caso de sindrome hemofagocítico en paciente infectada por el virus de la inmunodeficiencia humana.

Abstract: Hemophagocytic syndrome is a rare and serious disease characterized by a state of systemic hyperinflammation with overproduction of cytokines. Can respond to genetic causes (primary) or be triggered by infections, drugs, tumors or autoimmune diseases. There are established diagnostic criteria. Treatment consists in blocking the inflammatory response associated with the treatment of the underlying cause when it is. Mortality is high and usually is related to the cause that triggers the phenomenon. We report a case of hemophagocytic syndrome in HIV-infected patient.

[Hemophagocytic lymphohistiocytosis: Analysis of 18 cases]. / Linfohistiocitosis hemofagocítica: análisis de 18 casos.

BACKGROUND AND OBJECTIVE: Hemophagocytic lymphohistiocytosis (HLH) is a serious condition, caused by an improper regulation of the immune response to different stimuli of the immune system. Early diagnosis and treatment are a challenge for the clinician. PATIENTS AND METHOD: We conducted a retrospective study at our institution between 2010 and 2015, of adult patients diagnosed with HLH, in accordance with the criteria of the Histiocyte Society, analyzing their clinical characteristics, diagnostic and etiological studies and the outcome. RESULTS: Eighteen patients were analyzed. Median time to diagnosis was 24 days. We found neoplastic etiology in 8 cases (7 hematologic), while it was infection-related in 6 (4 visceral leishmaniasis), and an inflammatory disease in one. In the remaining 3, an underlying cause for the HLH was not found. Course of treatment was corticosteroids in 16 patients, associated with cyclosporine in 2 of them, one received immunoglobulins, while another received etoposide with tacrolimus. CONCLUSIONS: We emphasize the scarce use of etoposide therapy, the currently recommended treatment. Overall mortality was 44%, mainly associated with neoplastic etiology (67 compared to 16.6% mortality in infection-related etiology, P<.05).

Síndrome hemofagocítico asociado a tuberculosis en una paciente con inmunodeficiencia adquirida / Hemophagocytic syndrome associated with tuberculosis in a patient with acquired immunodeficiency

El síndrome hemofagocítico secundario es infrecuente en pediatría y, más aun, asociado a tuberculosis. Presentamos el caso de una paciente con síndrome de inmunodeficiencia adquirida, tuberculosis diseminada y síndrome hemofagocítico. Niña de 8 años, condiagnóstico de síndrome deinmunodeficiencia adquirida, que se internó por fiebre, vómitos y dolor abdominal. Presentaba distensión abdominal, deshidratación, taquipnea, rales subcrepitantes y sibilancias en ambos pulmones, anemia, plaquetopenia y alteración de la coagulación. Recibió antibióticos de amplio espectro y se realizó una laparotomía exploradora con apendicectomia y biopsia ganglionar. A las 72 horas, presentó convulsión tónico clónica, deterioro del sensorio, fiebre, hipoxemia, hepatoesplenomegalia, ascitis y edema periférico. Presentaba bicitopenia, hiperferritinemia y examen microscópico de médula ósea con hemofagocitosis. Recibió gammaglobulina intravenosa, corticoides y transfusiones sanguíneas. Se aisló Mycobacterium tuberculosis del aspirado gástrico, médula ósea y biopsia ganglionar abdominal. Se trató con isoniacida, rifampicina, estreptomicina y etambutol, y presentó franca mejoría.

The secondary hemophagocytic syndrome is rare in children and even rarer associated with tuberculosis. e report the case of a patient with acquired immunodeficiency syndrome, disseminated tuberculosis and hemophagocytic syndrome. An 8-year-old girl, diagnosed with acquired immunodeficiency syndrome, was admitted due to fever, vomiting and abdominal pain. She presented abdominal distension, dehydration, tachypnea, crackles and wheezing in both lungs, anemia, thrombocytopenia and coagulopathy. She received broad-spectrum antibiotics and exploratory laparotomy was performed with appendectomy and lymph node biopsy. After 72 hours the patient presented tonic clonic seizure, impaired sensory, fever, hypoxemia, hepatosplenomegaly, ascites and peripheral edema. She developed bicytopenia, hyperferritinemia and bone marrow microscopic examination with hemophagocytosis. She received intravenous gammaglobulin, steroids and blood transfusions. Mycobacterium tuberculosis was cultured in gastric aspirate, bone marrow and abdominal lymph node biopsy. She was treated with isoniazid, rifampicin, streptomycin and ethambutol, showing marked improvement.

Otitis externa maligna con ectima gangrenoso en un paciente con infección por VIH / Malignant otitis externa with ecthyma gangrenosum in an hiv-positive patient

La otitis externa maligna es considerada una infección invasiva del conducto auditivo externo, infrecuente y sumamente agresiva, que llega, en ocasiones, a comprometer la base del cráneo. Se presenta habitualmente en inmunocomprometidos, incluidos aquellos con infección por VIH. El ectima gangrenoso es una infección cutánea localizada, inusual, causada, en la mayoría de los casos por Pseudomonas aeruginosa, con compromiso multisistémico y evolución tórpida. La asociación entre ambas entidades es aún más rara. A continuación, presentamos el caso de una mujer de 37 años, con el antecedente principal de infección por VIH desde hace 10 años, adherente al tratamiento antirretroviral y, a pesar de mantener una adecuada respuesta inmunológica y virológica, se presenta a la urgencia de nuestro hospital en shock séptico por otitis externa maligna, con ectima gangrenoso y posible síndrome hemofagocítico como complicación, aislándose Pseudomonas aeruginosa como agente causal.

Malignant otitis externa is an aggressive, infrequent and invasive infection of the external auditory canal. In some cases it leads to skull base compromise, particularly in immunocompromised individuals, including HIV-positive patients. Ecthyma gangrenosum is an unusual, localized cutaneous infection, caused mostly by Pseudomonas aeruginosa, with systemic compromise and a torpid evolution. An association between these 2 entities is even more rare. We present the case of a 37-year old woman with a history of 10 years’ HIV infection and good adherence to antiretroviral therapy. Although she had an adequate immunological and virological response, she presented at our emergency department in septic shock due to malignant otitis externa with ecthyma gangrenosum and possible haemophagocytic syndrome as a complication, with Pseudomonas aeruginosa determined to be the causative agent.

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis: response to HLH-04 treatment protocol / Linfohistiocitosis hemofagocítica asociada con el virus de Epstein-Barr: respuesta al tratamiento con el protocolo HLH-04

Abstract Introduction: Hemophagocytic syndrome, macrophage activation syndrome, reactive histiocytosis or hemophagocytic lymphohistiocytosis (HLH) represent a group of diseases whose common thread is reactive or neoplastic mononuclear phagocytic system cells and dendritic cell proliferation. Clinical case: We present a case of an HLH probably associated with Epstein-Barr virus (EBV) in a 4-year-old male patient treated with HLH-04 protocol. Viral etiology in HLH is well accepted. In this case, clinical picture of HLH was assumed secondary to EBV infection because IgM serology at the time of clinical presentation was the only positive factor in the viral panel. Conclusions: Diagnosis of HLH is the critical first step to successful treatment. The earlier it is identified, the less the tissue damage and reduced risk of multiple organ failure, which favors treatment response.

Resumen Introducción: El síndrome hemofagocítico, síndrome de activación de macrófagos, histiocitosis reactiva o linfohistiocitosis hemofagocítica (HLH) representan un grupo de enfermedades cuyo factor común es la proliferación reactiva o neoplásica de las células mononucleares fagocíticas y del sistema de células dendríticas. Caso clínico: Se presenta un caso de HLH sugestivo de tener una asociación con el virus del Epstein Barr (VEB) de un paciente masculino de 4 años de edad, tratado con el protocolo HLH-04. La etiología viral en HLH es reconocida. En este caso se asumió un cuadro de HLH secundario a una infección por VEB, ya que la serología de IgM en el momento de la presentación clínica fue la única positiva en el panel viral. Conclusiones: El diagnóstico de la HLH es el primer paso crítico para el éxito del tratamiento. Entre más temprano se identifique, existe menor daño tisular y menor riesgo de falla orgánica múltiple, lo que favorece la respuesta al tratamiento.

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis: response to HLH-04 treatment protocol.

INTRODUCTION: Hemophagocytic syndrome, macrophage activation syndrome, reactive histiocytosis or hemophagocytic lymphohistiocytosis (HLH) represent a group of diseases whose common thread is reactive or neoplastic mononuclear phagocytic system cells and dendritic cell proliferation. CLINICAL CASE: We present a case of an HLH probably associated with Epstein-Barr virus (EBV) in a 4-year-old male patient treated with HLH-04 protocol. Viral etiology in HLH is well accepted. In this case, clinical picture of HLH was assumed secondary to EBV infection because IgM serology at the time of clinical presentation was the only positive factor in the viral panel. CONCLUSIONS: Diagnosis of HLH is the critical first step to successful treatment. The earlier it is identified, the less the tissue damage and reduced risk of multiple organ failure, which favors treatment response.

Pancitopenia en paciente con infección por VIH

Se expone el caso de una mujer joven con diagnóstico de infección por el virus de inmunodeficiencia humana (VIH) y abandono de tratamiento antiretroviral, ingresada por cuadro febril y tos, con sospecha de tuberculosis miliar. Evoluciona durante su internación con pancitopenia y esplenomegalia, iniciándose investigación y diagnóstico precoz de síndrome hemofagocítico (SHF). Presenta evolución desfavorable y óbito debido a múltiples comorbilidades.

This is the case of a young woman who was diagnosed with a human immunodeficiency virus (HIV) infection and had stopped the antiretroviral therapy. She was admitted for fever and cough with suspicion of miliary tuberculosis. The patient evolved during her hospitalization to splenomegalic pancytopenia. An investigation started and there was an early diagnosis of hemophagocytic syndrome (HPS). The patients evolved unfavorably and died due to multiple co-morbilities.