1/3. Angioimmunoblastic lymphadenopathy with dysproteinemia following doxycycline administration.Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a primary lymphoproliferative T-cell disorder, currently classified as a peripheral T-cell non-Hodgkin's lymphoma. AILD is characterized by generalized lymphadenopathy, hepatosplenomegaly, immunological abnormalities, polyclonal hypergammaglobulinemia and anemia. We report a case of AILD in an 80-year-old male who presented with a generalized pruritic maculopapular eruption and fever following doxycycline administration. The maculopapular rash progressed to formation of confluent nodules, plaques and finally erythroderma with lymphadenopathy and hepatosplenomegaly. Blood analysis revealed an elevated erythrocyte sedimentation rate and polyclonal hypergammaglobulinemia. Lymph node biopsy showed almost complete effacement of the nodal architecture with diffuse proliferation of small vessels forming an arborizing network, surrounded by atypical lymphocytes, usually CD3 CD4 and occasionally CD3 CD8 . There were also larger cells (immunoblastic shape) that displayed CD20 positively, some scattered plasma cells, and eosinophils. histology of a cutaneous lesion showed spongiosis and infiltration of the epidermis by atypical lymphocytes with large hyperchromatic nuclei, perivascular dermal lymphocytic infiltrate (CD3 ) mixed with plasma cells and occasional large immunoblasts (CD20 ). During hospitalization the patient developed hemolytic anemia (Coombs positive) and lung metastases. The prognosis of AILD is generally poor, with a median survival of less than 20 months. Our patient died two and a half months after the diagnosis was made due to sepsis caused by staphylococcus aureus isolated in hemoculture.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/3. Angio-immunoblastic lymphadenopathy. Report of a case with pleural effusion.A 78-year old man is presented with a characteristic case history and physical and laboratory findings typical of angio-immunoblastic lymphadenopathy with dysproteinemia (AILD). The disease had an acute onset with constitutional symptoms, generalized lymphadenopathy and hepato-splenomegaly. The presence of a large pleural effusion was of particular interest. Histologically the distinctive feature was a pronounced proliferation of small blood vessels and immunoblasts in the lymph nodes. Management of patient with AILD is problematic. Since AILD is a non-neoplastic process, symptomatic treatment with small doses of steroids, if necessary, would seem to be the best therapeutic approach. However, in our patient, as well as in other cases reported in the literature, a rapid and long standing (18 months so far in our case) complete remission was obtained with a short cycle of chemiotherapy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/3. Chronic ulcerative colitis, skin necrosis, and cryofibrinogenemia.Necrotizing skin lesions developed in a man with chronic ulcerative colitis. No evidence of intrinsic disease of medium or small-sized vessels was found. A circulating cryofibrinogen was thought to be responsible for in situ thrombosis leading to skin infarctions. sodium warfarin in a daily dose of 2.5 to 5 mg appears to have thwarted progression of developing lesions and the occurrence of new ones.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |