Résumé
There have been few reports on chromosomal abnormalities in secondary hemophagocytic lym-phohistiocytosis (HLH) including Epstein-Barr virus-associated HLH (EBV-HLH). Clonality of HLH can be determined by karyotypic analysis, T-cell receptor or IgH rearrangement studies, and EBV genome terminal repeat investigation. Chromosome analysis in EBV-HLH is the most important tool currently available for assessment of case-by-case prognosis. We report a case of fatal EBV-HLH with clonal karyotype abnormality. A 57-year-old man was admitted with persistent high fever and hepatosplenomegaly. Laboratory data revealed pancytopenia, hypofibrinogenemia, coagulation abnormalities, and marked abnormalities of liver function tests. EBV-DNA was detected in the bone marrow by PCR. The bone marrow studies showed mature, benign-looking histiocytic hyperplasia with prominent hemophagocytosis and clonal chromosomal abnormality. Although the patient was treated with immunochemotherapy and antibiotics, he died of disseminated intravascular coagu-lopathy and sepsis. Considering this fatal clinical course, it is important to take intensive therapeutic measures if karyotype abnormality is noted in the treatment of EBV-HLH cases.
Sujets)
Humains , Adulte d'âge moyen , Antibactériens , Moelle osseuse , Aberrations des chromosomes , Fièvre , Génome , Herpèsvirus humain de type 4 , Hyperplasie , Caryotype , Tests de la fonction hépatique , Lymphohistiocytose hémophagocytaire , Pancytopénie , Réaction de polymérisation en chaîne , Pronostic , Récepteurs aux antigènes des cellules T , Sepsie , Séquences répétées terminalesRésumé
Splenic marginal zone lymphoma (SMZL) is a rare B-cell neoplasm characterized by massive splenomegaly, moderate lymphocytosis, bone marrow intrasinusoidal involvement of lymphocytes and a relatively indolent course. We report a case of SMZL diagnosed by bone marrow studies using immunophenotyping and immunohistochemical stain, and confirmed by splenectomy. The patient was a 61-year old male, who showed mild lymphocytosis in peripheral blood and bone marrow aspirates. Immunophenotyping of bone marrow aspirates showed lymphocytes positive for CD19, CD20, CD22 (dim), CD23 (dim) and negativie for CD5 and CD10. The immunohistochemistry of bone marrow and spleen also showed lymphocytes positive for CD20 and negative at for cyclin D1. Now he is being treated for chronic obstructive pulmonary disease and will receive chemotherapy.
Sujets)
Humains , Mâle , Lymphocytes B , Moelle osseuse , Cycline D1 , Traitement médicamenteux , Immunohistochimie , Immunophénotypage , Lymphocytes , Hyperlymphocytose , Lymphomes , Broncho-pneumopathie chronique obstructive , Rate , Splénectomie , SplénomégalieRésumé
Myelofibrosis results from stimulation of bone marrow stromal fibroblasts by fibrogenic cytokines elaborated by neoplastic or reactive cells in the marrow. Chronic idiopathic myelofibrosis should be differentiated from secondary myelofibrosis resulting from bone marrow involvement of malignant lymphoma because these diseases have different therapeutic strategies. Myelofibrosis in systemic lupus erythematosus is an uncommon but well-recognized complication, and identifying an autoimmune myelofibrosis is important in diagnosing this benign cause of myelofibrosis. We report two cases of myelofibrosis presenting the clinical and radiologic findings that mimicked malignant lymphoma -a case of autoimmune myelofibrosis associated with systemic lupus erythematosus showing extensive lymphadenopathy and a case of chronic idiopathic myelofibrosis with focal intrasplenic extramedullary hematopoiesis- and discuss the importance of the clinical information and radiologic findings for the pathologic diagnosis of myelofibrosis.