RESUMEN
Toxic epidermal necrolysis (TEN) is a lethal entity, characterized by extensive epidermal necrosis and multiorgan failure. Hemophagocytic syndrome (HFS) is also a rare and lethal syndrome characterized by hyperinflammation that leads to the appearance of fever, pancytopenia, organomegaly and hemophagocytosis. The concomitance of these diseases is extremely uncommon. We report a 38 years old female, who during the course of a HFS secondary to Hodgkin Lymphoma (HL), presented a TEN secondary to antibiotics. She was admitted due to a consumptive syndrome, lymphadenopathy, visceromegaly and severe pancytopenia. Laboratory and bone marrow tests confirmed HFS. Due to constant fever, imipenem was indicated. On the third day she started with pain and skin rash. She evolved with positive Nikolsky sign. Cutaneous biopsy was concordant with extensive TEN, which was managed with intravenous immunoglobulin and dexamethasone. A complete response and normalization of the blood count were achieved. Finally, the lymph node biopsy showed HL of mixed cellularity type, which was managed with 8 cycles of ABVD chemotherapy, achieving complete remission.
Asunto(s)
Enfermedad de Hodgkin/complicaciones , Linfohistiocitosis Hemofagocítica/etiología , Síndrome de Stevens-Johnson/etiología , Adulto , Antibacterianos/efectos adversos , Protocolos de Quimioterapia Combinada Antineoplásica , Bleomicina , Dacarbazina , Doxorrubicina , Femenino , Enfermedad de Hodgkin/tratamiento farmacológico , Enfermedad de Hodgkin/patología , Humanos , Imipenem/efectos adversos , Linfohistiocitosis Hemofagocítica/tratamiento farmacológico , Linfohistiocitosis Hemofagocítica/patología , Síndrome de Stevens-Johnson/tratamiento farmacológico , Síndrome de Stevens-Johnson/patología , Resultado del Tratamiento , VinblastinaRESUMEN
BACKGROUND: International guidelines suggest a screening panel for monoclonal gammopathies that contains serum protein electrophoresis (SPE), free light chain (FLC) measurements and immunofixation. This combination provides the possibility of a timely accurate diagnosis. AIM: To evaluate the sensibility of a simple screening panel (SPE + FLC). MATERIAL AND METHODS: We analyzed 191 consecutive serum samples of patients with a suspected monoclonal gammopathy (MG). RESULTS: Seventy five patients were diagnosed with MG. The sensitivity and specificity of the combination of SPE + FLC for the diagnosis of monoclonal gammopathy were 95% (95% confidence intervals 89-99) and 99% (95% confidence intervals 96-100), respectively. CONCLUSIONS: We were able to validate the international recommendations on the diagnostic accuracy of this simple combination of two tests in serum for monoclonal gammopathy.
Asunto(s)
Electroforesis de las Proteínas Sanguíneas/métodos , Cadenas Ligeras de Inmunoglobulina/sangre , Paraproteinemias/diagnóstico , Biomarcadores/sangre , Humanos , Estudios Prospectivos , Reproducibilidad de los Resultados , Sensibilidad y EspecificidadRESUMEN
Toxic epidermal necrolysis (TEN) is a lethal entity, characterized by extensive epidermal necrosis and multiorgan failure. Hemophagocytic syndrome (HFS) is also a rare and lethal syndrome characterized by hyperinflammation that leads to the appearance of fever, pancytopenia, organomegaly and hemophagocytosis. The concomitance of these diseases is extremely uncommon. We report a 38 years old female, who during the course of a HFS secondary to Hodgkin Lymphoma (HL), presented a TEN secondary to antibiotics. She was admitted due to a consumptive syndrome, lymphadenopathy, visceromegaly and severe pancytopenia. Laboratory and bone marrow tests confirmed HFS. Due to constant fever, imipenem was indicated. On the third day she started with pain and skin rash. She evolved with positive Nikolsky sign. Cutaneous biopsy was concordant with extensive TEN, which was managed with intravenous immunoglobulin and dexamethasone. A complete response and normalization of the blood count were achieved. Finally, the lymph node biopsy showed HL of mixed cellularity type, which was managed with 8 cycles of ABVD chemotherapy, achieving complete remission.
Asunto(s)
Humanos , Femenino , Adulto , Enfermedad de Hodgkin/complicaciones , Síndrome de Stevens-Johnson/etiología , Linfohistiocitosis Hemofagocítica/etiología , Vinblastina , Bleomicina , Enfermedad de Hodgkin/patología , Enfermedad de Hodgkin/tratamiento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica , Doxorrubicina , Imipenem/efectos adversos , Síndrome de Stevens-Johnson/patología , Síndrome de Stevens-Johnson/tratamiento farmacológico , Resultado del Tratamiento , Dacarbazina , Linfohistiocitosis Hemofagocítica/patología , Linfohistiocitosis Hemofagocítica/tratamiento farmacológico , Antibacterianos/efectos adversosRESUMEN
Background: International guidelines suggest a screening panel for monoclonal gammopathies that contains serum protein electrophoresis (SPE), free light chain (FLC) measurements and immunofixation. This combination provides the possibility of a timely accurate diagnosis. Aim: To evaluate the sensibility of a simple screening panel (SPE + FLC). Material and Methods: We analyzed 191 consecutive serum samples of patients with a suspected monoclonal gammopathy (MG). Results: Seventy five patients were diagnosed with MG. The sensitivity and specificity of the combination of SPE + FLC for the diagnosis of monoclonal gammopathy were 95% (95% confidence intervals 89-99) and 99% (95% confidence intervals 96-100), respectively. Conclusions: We were able to validate the international recommendations on the diagnostic accuracy of this simple combination of two tests in serum for monoclonal gammopathy.