Linfohistiocitosis hemofagocítica asociada a infecciones virales: reto diagnóstico y dilema terapéutico / Hemophagocytic lymphohistiocytosis associated with viral infections: diagnostic challenges and therapeutic dilemmas

La linfohistiocitosis hemofagocítica es un síndrome clinicopatológico de evolución potencialmente fatal, en el que una respuesta inmune no controlada e ineficaz conduce a hiperinflamación. Puede aparecer como una enfermedad familiar o esporádica, asociado a diferentes factores desencadenantes: infecciones, neoplasias, enfermedades autoinmunes o inmunodeficiencias adquiridas, pero la asociación más consistente es con infecciones virales, especialmente el virus de Epstein-Barr. Las principales características clínicas son fiebre, disfunción hepática, coagulopatía y pancitopenia. El diagnóstico es difícil debido a la rareza de este síndrome y a la falta de especificidad de los hallazgos clínicos, sin embargo, un diagnóstico y tratamiento precoces son importantes para disminuir la mortalidad. El tratamiento debe ser dirigido al control de la enfermedad subyacente y a suprimir la respuesta inflamatoria exagerada mediante el uso de inmunosupresores (AU)

Hemophagocytic lymphohistiocytosis is a frequently fatal clinicopathologic syndrome in which an uncontrolled and ineffective immune response leads to severe hyperinflammation. It may occur as either a familial disorder or a sporadic condition in association with a variety of triggers: infections, malignancies, autoimmune diseases, and acquired immune deficiencies. However, the most consistent association is with viral infections, especially Epstein-Barr virus. The main clinical features are fever, liver dysfunction, coagulation abnormalities and pancytopenia. Early diagnosis and treatment are important to reducing mortality, but the diagnosis is difficult because of the rarity of the syndrome and the lack of specificity of the clinical findings. Treatment should be directed towards treating the underlying disease and to suppressing the exaggerated inflammatory response through the use of immunosuppressive agents (AU)

Hemophagocytic lymphohistiocytosis associated with viral infections: Diagnostic challenges and therapeutic dilemmas. / Linfohistiocitosis hemofagocítica asociada a infecciones virales: reto diagnóstico y dilema terapéutico.

Hemophagocytic lymphohistiocytosis is a frequently fatal clinicopathologic syndrome in which an uncontrolled and ineffective immune response leads to severe hyperinflammation. It may occur as either a familial disorder or a sporadic condition in association with a variety of triggers: infections, malignancies, autoimmune diseases, and acquired immune deficiencies. However, the most consistent association is with viral infections, especially Epstein-Barr virus. The main clinical features are fever, liver dysfunction, coagulation abnormalities and pancytopenia. Early diagnosis and treatment are important to reducing mortality, but the diagnosis is difficult because of the rarity of the syndrome and the lack of specificity of the clinical findings. Treatment should be directed towards treating the underlying disease and to suppressing the exaggerated inflammatory response through the use of immunosuppressive agents.

[Extragonadal germ cell tumor in HIV+ female]. / Tumor de células germinales extragonadal en mujer VIH+.

Extragodanal germ cell tumors (EGCT) are not common, especially among women. Although there is no evidence of relationship between this sort of tumor and HIV infection they can appear at the same time in a patient, because in both cases the maximum incidence occurs in patients in the same age group. We present the case of a 27 years old woman, poly-drug user, with a recently diagnosis of HIV infection, who was admitted to clinic because of infection and shortage of breath, and develops during her hospitalization diarrhoea, generalized tonic-clonic seizure and left hemiparesis. Complementary tests showed us diffuse interstitial pulmonary pattern, mediastinal mass with intrathoracic adenopathies, cerebral tumor and diffuse intestinal enlargement. The breath infection got better with a wide-ranging antibiotic treatment, which included cotrimoxazol and levofloxacin, but the brain tumor didnt get better with the antitoxoplasma treatment. The clinical presentation simulated in the beginning a disseminated lymphoma, in a HIV+ patient; nevertheless, after receiving the result of the biopsy of a supraclavicular adenopathy and a b-HCG, an extragodanal germ cell tumor was diagnosed. We haven't found any case of EGCT in young women infected with HIV in our bibliographical review (MEDLINE).