RESUMEN
INTRODUCTION: Immune thrombocytopenia (ITP) is an acquired hemorrhagic disease due to antiplatelet antibodies, that will become a chronic disease in 70% of adults. Most of chronic ITP patients display clonal restriction of antiplatelet antibodies. To date, there is no biomarker able to predict the evolution of the disease. The objective of the study is to determine whether Hevylite® and/or Freelite® assays are prognostic factors for progression to chronic ITP. METHODS: This is a retrospective, monocentric, prognostic study of a biomarker, performed using frozen samples stored in a serum library. Freelite® and a Hevylite® assays were performed on the samples collected at diagnosis for adult patients with newly diagnosed ITP at the University Hospital of Poitiers between 2014/01/01 and 2017/05/01. To predict the evolution into a chronic disease, a ROC curve analysis was performed on four variables: IgGκ, IgGκ/IgGλ ratio, IgGκ - IgGλ, and κ/λ ratio. RESULTS: Thirty-two patients were included and analyzed. No patient had an abnormal κ/λ ratio. Three patients had an abnormal IgGκ/IgGλ ratio. The following variables IgGκ, IgGκ/IgGλ, IgGκ - IgGλ, and κ/λ ratio were not able to predict progression to chronic ITP in our study. CONCLUSION: This study did not reveal any prognostic value of the Freelite® and Hevylite® tests on the evolution of ITP into a chronic disease.
Asunto(s)
Inmunoensayo , Inmunoglobulina G/sangre , Cadenas Pesadas de Inmunoglobulina/sangre , Cadenas Ligeras de Inmunoglobulina/sangre , Púrpura Trombocitopénica Idiopática/sangre , Biomarcadores/sangre , Progresión de la Enfermedad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Estudios RetrospectivosRESUMEN
INTRODUCTION: Myalgia is a classical sign in invasive meningococcal diseases (IMD), but severe and persistent myalgia following an IMD have never been reported to date. CASE REPORT: A 20-year-old man presented with purpura fulminans and meningitis caused by Neisseria meningitidis serogroup Y, revealing properdin deficiency. Although meningitis symptoms improved after antibiotherapy, initial myalgia of the lower limbs increased, associated with mild rhabdomyolysis. Magnetic resonance imaging (MRI) revealed an increased STIR (Short TI inversion recovery) signal of both quadriceps muscles, without abscess. After exclusion of other causes of myopathy, a post-infectious myositis was diagnosed. A four-week course of corticosteroids led to dramatic improvement. CONCLUSION: Post-infectious inflammatory myopathy should be suspected in case of severe and persistent myalgia associated with rhabdomyolysis following an IMD, after exclusion of pyomyositis especially. A short course of corticosteroids seems to be effective.
Asunto(s)
Meningitis Meningocócica/complicaciones , Mialgia/microbiología , Miositis/microbiología , Properdina/deficiencia , Rabdomiólisis/microbiología , Humanos , Masculino , Neisseria meningitidis , Púrpura Fulminante/complicaciones , Adulto JovenRESUMEN
Serum free light chains (sFLC) assay is an important marker in plasma cell dyscrasia. It is thus recommended for the diagnosis of monoclonal gammopathy, together with serum protein electrophoresis and immunofixation. sFLC assay has also a prognostic value and is a criterion for treatment response and relapse of some monoclonal gammopathies. Three assays are currently available in France, the gold standard being the Freelite® assay, the two others requiring further validation. These three assays are not interchangeable during patient's follow-up. The Freelite® assay is integrated in the myeloma diagnostic criteria from the International Myeloma Working Group 2014, and has a higher sensitivity than Bence Jones protein test for diagnosis, treatment response and follow-up of light chain myeloma. The Freelite® assay is the main marker of therapeutic response in light chain amyloidosis and allows to stratify the risk for progression in monoclonal gammopathy of undetermined significance. Some studies have also shown its value in diagnosis of multiple sclerosis and in screening for monoclonal gammopathy in the cases of acute renal failure. The Freelite® assay is then currently essential in myeloma or amyloidosis, and could be soon extended to the management of autoimmune diseases.
Asunto(s)
Cadenas Ligeras de Inmunoglobulina/análisis , Paraproteinemias/diagnóstico , Pruebas Serológicas , Humanos , Inmunoensayo/métodos , Cadenas Ligeras de Inmunoglobulina/sangre , Mieloma Múltiple/sangre , Mieloma Múltiple/diagnóstico , Paraproteinemias/sangre , Paraproteinemias/etiología , Pronóstico , Pruebas Serológicas/métodos , Pruebas Serológicas/normasRESUMEN
INTRODUCTION: Auto-immune hemolytic anemia (AIHA) is a rare cause of anemia, characterized by autoantibodies directed against self red blood cells. It can be primary or secondary, in particular due to lymphoproliferative diseases. CASE REPORT: We report the case of a 24-year-old woman who presented with a severe macrocytic anemia associated with an ovarian teratoma. CONCLUSION: Ovarian teratoma is a rare cause of secondary AIHA, with only few cases reported. Its treatment differs from primary AIHA as steroids may be ineffective. Indeed, complete response can only be achieved with surgical excision of the tumor.