ABSTRACT
Hemophagocytic syndrome is a severe condition of excessive immune activation that has a high mortality in the absence of treatment. The syndrome is classified as primary if associated with congenital or hereditary problems, or secondary/acquired if associated with infectious, autoimmune or oncology diseases. We report four adult cases of the syndrome, one with viral, two with autoimmune and one with idiopathic causes who were successfully treated with HLH 94-04 chemotherapy protocol. Our experience shows that a high index of suspicion, early diagnosis and an opportune therapy are essential in the treatment of this disease.
Subject(s)
Adult , Female , Humans , Male , Young Adult , Cyclosporine/therapeutic use , Dexamethasone/therapeutic use , Etoposide/therapeutic use , Immunosuppressive Agents/therapeutic use , Lymphohistiocytosis, Hemophagocytic/drug therapy , Methotrexate/therapeutic use , Drug Therapy, Combination/methods , Early Diagnosis , Ferritins/blood , Lymphohistiocytosis, Hemophagocytic/diagnosis , Lymphohistiocytosis, Hemophagocytic/physiopathologyABSTRACT
Natural killer cell malignancy is a rare and aggressive lymphoid neoplasm encompassing extra-nodal NK/T-cell lymphoma, nasal-type (ENKLN) and aggressive NK-cell lymphoma/leukemia (ANKL). A case of cutaneous ENKLN and a case of ANKL in Thai patients are reported Both patients developed hemophagocytic syndrome and shortly succumbed to death. The cells in cutaneous ENKLN are small to medium in size with minimal cytoplasm, round nuclei, irregular nuclear membrane, andfine chromatin with inconspicuous nucleoli. While that of ANKL are medium to large-sized mononuclear cells with moderate cytoplasm. Their nuclei are elongated to embryo-like with irregularly thickened nuclear membrane, fine chromatin, and small to occasional prominent nucleolus. Ancillary techniques studied on paraffin embedded tissues of both cases demonstrated that the neoplastic cells exhibit cytoplasmic CD3+, CD56+ and cytotoxic granules + by immunohistochemistry, absence of T cell receptor gene rearrangement by PCR, and presence of Epstein-Barr virus mRNA (EBER) transcripts by in situ hybridization. The authors reviewed the literature on natural killer cell neoplasm and compared the clinical characteristics, natural history, and association of Epstein-Barr virus infection with hemophagocytic syndrome.
Subject(s)
Adult , Epstein-Barr Virus Infections/physiopathology , Female , Humans , Killer Cells, Natural/pathology , Leukemia/pathology , Lymphohistiocytosis, Hemophagocytic/physiopathology , Lymphoma/pathology , Risk FactorsABSTRACT
El síndrome hemofagocítico reactivo, o linfohistiocitosis hemofagocítica secundaria, comprende un grupo numeroso de enfermedades, muchas de ellas de causa infecciosa, caracterizado por hemofagocitosis con citopenia de al menos dos de las tres series sanguíneas, aumento de los niveles de citoquinas y de la ferritina sérica. El cuadro clínico comprende manifestaciones inflamatorias sistémicas semejantes a la sepsis, entidad muy frecuente en las unidades de Terapia Intensiva, y posee elementos fisiopatológicos en común con ella. Proponemos mediante la presentación de cuatro casos clínicos, considerar al síndrome hemofagocítico reactivo como una entidad frecuente en los pacientes graves, con pruebas sencillas para orientar a qué pacientes realizar un procedimiento diagnóstico. Por último actualizamos los tratamientos específicos conocidos.
Reactive hemophagocytic syndrome or hemophagocytic lymphohistiocytosis comprises a variety of disorders, many of them associated with infection. It is characterized by hemophagocytosis, with cytopenia involving at least two cellular lines, increase in cytokines and serum ferritin. The clinical course resembles sepsis, sharing similar physiopathological features. We propose that hemophagocytic syndrome is an underdiagnosed entity in the critical care setting; simple tests aid to identify which patients should undergo diagnostic procedures. We discuss current therapeutic approaches.