Xantoma diseminado / Disseminated xanthoma

Paciente de sexo femenino, de 22 años de edad, que consulta por poliuria, fiebre, desorientación témpo-espacial y pápulas pardo-rojizas en párpados, surco nasogenianos, pliegues y raíz de miembros. Se solicitan exámenes complementarios y biopsia confirmando el diagnóstico de xantoma diseminado. El interés del caso radica en una patología poco frecuente, generalmente de evolución benigna que en nuestra paciente tuvo desenlace fatal.

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.

Hemophagocytic syndrome associated with visceral leishmaniasis.

Hemophagocytosis, either primary (familial) or secondary (reactive), is a life threatening condition in childhood. Etiology should be vigorously searched to avoid a diagnosis of primary hemophagocytosis and treatment with cytotoxic drugs. A child with visceral leishmaniasis causing hemophagocytosis is presented.

Hemophagocytic Syndrome Associated with Kikuchi's Disease

A 13-yr-old female was admitted to our hospital with fever, seizure, and cervical lym-phadenopathy. Laboratory data showed pancytopenia, elevation of serum transaminase, lactate dehydrogenase, triglyceride, and ferritin levels. Lymph node biopsy revealed features of Kikuchi's disease and there were signs of histiocytosis and hemophagocytic phenomenon in bone marrow. She recovered after treatment with intravenous immunoglobulin and corticosteroids therapy. Hemophagocytic syndrome can be associated with Kikuchi's disease especially in childhood and seems to have a less aggressive clinical course and better prognosis.

Hepatosplenic B-cell lymphoma associated with hemophagocytic syndrome: a case report

While T-cell non-Hodgkin's lymphoma (NHL) associated with hemophagocytic syndrome (HPS) has been frequently observed, B-cell NHL associated with HPS has been rarely reported. We report a case of hepatosplenic B-cell lymphoma associated with HPS in a 41-year-old woman who presented with fever of unknown origin. An abdominal CT scan revealed splenomegaly with focal splenic infarction. Splenectomy and a liver wedge biopsy showed sinusoidal-pattern infiltration of medium to large tumor cells with positive reaction to a B-lymphocyte marker. Findings on bone marrow examination showed proliferation of histiocytes with avid hemophagocytosis.

Nasal angiocentric lymphoma with hemophagocytic syndrome

OBJECTIVES: Hemophagocytic syndrome (HS) is a fatal complication of nasal angiocentric lymphoma (AL) and difficult to distinguish from malignant histiocyosis. Epstein-Barr virus (EBV)-associated HS is frequently observed in lymphoma of T-cell lineage and EBV is highly associated with nasal AL. Clinicopathologic features of 10 nasal ALs with HS were reviewed to determine the clinical significance and the pathogenetic association with EBV. METHODS: Ten patients of HS were identified from a retrospective analysis of 42 nasal ALs diagnosed from 1987 to 1996. Immunohistochemical study and in situ hybridization were performed on the paraffin-embedded tumor specimens obtained from 10 patients. Serologic study of EBV-Ab was performed in 3 available patients. RESULTS: Five patients had HS as initial manifestation, 3 at the time of relapse and 2 during the clinical remission of AL. Four patients were treated by combination chemotherapy (CHOP) and others had only supportive care. The median survival of all patients with HS was 4.1 months (range 2 days-36.5 months) and all had fatal outcome regardless of the treatment-modality. All cases were positive for UCHL1 (CD45RO) and EBV by EBER in situ hybridization. The data of serologic tests indicated the active EBV infection. CONCLUSIONS: HS is a fatal complication of nasal AL and has a high association with EBV. Reactivation of EBV may contribute to HS and further investigation of predictive factors and effective treatment of HS should be pursued in the future.

Infected-associated hemophagocytic syndrome.

An epidemic of an infection associated with circulating hemophagocytes (HP) and activated monocytes (AM) was seen in Bombay. Although certain features overlapped with the well-defined entity of virus-associated hemophagocytic syndrome and familial hemophagocytic lymphohistiocytosis, it was distinct enough to place it in a separate category. Affected children were predominantly two days to two years of age. They had fever, altered sensorium, neurological symptoms, dyspnea, and/or diarrhea, and significant bleeding. Laboratory tests showed neutrophilia, AM and HP's in every blood smear, coagulopathy, normal cerebrospinal fluid, normal liver transaminases, hypertriglyceridemia, and hypoalbuminemia. Surgical cases were remarkable in that they had small bowel malformations. These cases were subdivided into four distinct groups based on age of presentation, neonates, infants, children and a surgical group. The clinical differences in each group are described.

Hepatopathy-thrombocytopenia vs infection-induced hemophagocytic syndrome in Wilms' tumor: a case report.

A 3-year-old boy with Wilms' tumor, post operative left nephrectomy stage, had HTS on day 99 of the combined chemotherapy which lasted for more than 20 days. He had severe respiratory distress due to a moderate amount of ascites and marked pleural effusion. Because of high fever, thrombocytopenia and marked hemphagocytosis in the bone marrow, he received IVIG for 2 days. Normal platelet count and markedly decreased pleural fluid were attained within 3 days. He subsequently tolerated full doses of combined chemotherapeutic agents with an additional one (doxorubicin). In cases of HTS, IAHS should be suspected. The bone marrow should be done and treatment accordingly so that there is no need to decrease, the dosage of chemotherapeutic agents afterwards.