RESUMEN
Objective:To study the incidence, clinical features, diagnosis, and treatment of angioimmunoblastic T-cell Lymphoma (AITL). Methods:We retrospectively analyzed the clinical data of 31 patients with AITL treated in the tumor hospital of Harbin Medical University between January 1995 and January 2008. We also analyzed the incidence, clinical features, diagnosis, and treatment of AITL. Results:The complete response rate and partial response rate of the entire group were 54.8%and 16.1%, respectively. The overall response rate was 70.9%, and the median survival time was 27.3 months. The five-year survival rate of the entire group was 35%. The response rate was 76.5%in the CHOP group and 75%in the COP group. No significant differences were detected between two groups (P>0.05). Con-clusion:The symptoms and signs of ATIL differ in different sites. The COP and CHOP regimens have similar efficacy and tolerable side effects.
RESUMEN
Angioimmunoblastic lymphadenopathy with dysproteinemia(AILD), first described at early seventies, is a clinico-pathologic syndrome which has common features of lymphadenopathy, hepatosplenomegaly, fever, rash and dysproteinemia. It is a lymphoproliferative disease, differs from malignant lymphoma by the high frequency of constitutional symptoms and by the presence of generalized lymph node enlargement, hepatosplenomegaly, cutaneous menifestations and polyclonal hypergammaglobulinemia at the time of clinical onset. Peripheral T cell lymphomas(PTCL) are morphologically and immunologically heterogeneous group of lymphoproliferative disorders that are composed of postthymic lymphocytes. There are group of cases showing features similar to AILD, and these cases are classified as AILD like PTCL. We report a rare case of AILD like PTCL in an 8 year old boy, who visited Seoul National University Hospital with fever, hepatosplenomegaly and cervical lymphadenopathy, and diagnosed as AILD like PTCL by lymph node biopsy. He did not respond to conventional chemotherapy and died 1 year after clinical onset during chemotherapy, showing feature of multiple brain infarction of unknown etiology.
Asunto(s)
Niño , Humanos , Masculino , Biopsia , Infarto Encefálico , Quimioterapia , Exantema , Fiebre , Hipergammaglobulinemia , Linfadenopatía Inmunoblástica , Ganglios Linfáticos , Enfermedades Linfáticas , Linfocitos , Linfoma , Linfoma de Células T Periférico , Trastornos Linfoproliferativos , Paraproteinemias , SeúlRESUMEN
We report here a case of angioimmunoblastic lyinphadenopathy with dysprote inemia(AILD) in a 65-year-old male associated with skin nodules on the face. Physical examina.tion showed generalized lymphadenopathy, hepatosplenomegaly, erythematous maculopapular eruptions on the trunk and extremities, and er the matous nodules on the face. Lahoratory findings showed hemolytic anernia, arid polyclonal gammopathy. Histopathological findings of lymph node revealed typical features of AILD. I-iistopathological findings of maculopapules revealed lymphohistiocytic infiltra tion and extravasated erythrocytes around the blocked vessels, whereas histological picture of nodules on the face mirrorred that of the lymph node. After taking systemic chemotherapy, his general condition and skin lesions were improved.
Asunto(s)
Anciano , Humanos , Masculino , Quimioterapia , Eritrocitos , Extremidades , Linfadenopatía Inmunoblástica , Ganglios Linfáticos , Enfermedades Linfáticas , PielRESUMEN
We present a case of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) in a 57-year-old male who had suffered from fever and general weakness of one month's duration. He showed generalized lymphadenopathy, hepatomegaly and purpuric maculopapules on the whole body surface. Lahoratory findings show anemia, polyclonal gammaglobulinemia and thrombocytopenia. The lymph node biopsy specimens show morphologic triad of AILD except lack of interstitial deposits of eosinophilic materials and the skin biopsy specimen shows lymphohistiocytic vasculitis. Bone marrow biopsy specimen shows similar findings in lymph node, suggesting bone marrow involvement. He have been treated with COPP chemotherapy.
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Anemia , Biopsia , Médula Ósea , Quimioterapia , Eosinófilos , Fiebre , Hepatomegalia , Linfadenopatía Inmunoblástica , Ganglios Linfáticos , Enfermedades Linfáticas , Piel , Trombocitopenia , VasculitisRESUMEN
Herein we report a 51-year-old male with angioimmunoblastic lymphadenopathy who had developed rnaculopapular eruptions after the administration of crystalline penicillin, acetylsalicylic acid and sulpyrine. Skin biopsy showed mild lyrnphohistiocytic infiltration and extravasated RBCs around the blood vessels in the upper dermis. He had run a progressively downhill course in spite of vigorous chemotherapy and ultimately died.