1/5. 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 = hypergammaglobulinemia (Clic here for more details about this article) |
2/5. Angioimmunoblastic lymphadenopathy with dysproteinemia: report of a case in infancy with review of literature.A case of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) in infancy is reported. The disease had a mild onset with generalized lymphadenopathy, hepatosplenomegaly, thrombocytopenia, polyclonal hypergammaglobulinemia, and T-cell deficiency. The AILD course lasted more than 100 months, alternating clinical remission to recurrent relapses. hepatitis b viral infection suddenly evolving to hepatic failure was the cause of death. From a rapid survey of the present knowledge, the nosology, immunological features, and therapy of AILD are discussed and a possible presumptive pathogenetic pathway is proposed.- - - - - - - - - - ranking = 0.5keywords = hypergammaglobulinemia (Clic here for more details about this article) |
3/5. Monoclonal hypergammaglobulinemia without malignant transformation in angioimmunoblastic lymphadenopathy with dysproteinemia.A patient with angioimmunoblastic lymphadenopathy with dysproteinemia had a monoclonal IgG hypergammaglobulinemia. In contrast to previous reports, the development of a monoclonal paraprotein in the setting of angioimmunoblastic lymphadenopathy with dysproteinemia did not herald a transformation to malignancy. The patient improved clinically and the monoclonal gammopathy disappeared when the patient was treated with prednisone. The relationship of these findings to current concepts regarding the cause of angioimmunoblastic lymphadenopathy with dysproteinemia is discussed.- - - - - - - - - - ranking = 2.5keywords = hypergammaglobulinemia (Clic here for more details about this article) |
4/5. Multicentric giant lymph node hyperplasia. A hyperimmune syndrome with a rapidly progressive course.A patient who had diffuse lymph node enlargement, fever, skin rashes, anemia and polyclonal hypergammaglobulinemia is described. Histologic examination of lymph nodes taken from different sites (cervical, axillary and inguinal) revealed the presence of giant lymph node hyperplasia. The liver and bone marrow showed a moderate lymphocytic and plasma cell infiltration. The clinical presentation of a multicentric variety of giant lymph node hyperplasia in the reported case is similar to the clinical features usually associated with angio-immunoblastic lymphadenopathy with dysproteinemia, indicating that these two disorders may be related and may affect the same organs and systems. Alternatively, this histologic reactive giant lymph node hyperplasia progressing with a rapid declivitous course can be considered distinctive of a separate entity.- - - - - - - - - - ranking = 0.5keywords = hypergammaglobulinemia (Clic here for more details about this article) |
5/5. Successful treatment of angioimmunoblastic lymphadenopathy with dysproteinemia by local irradiation.We report an 85-year-old man presenting with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) of the palatine tonsil with cervical and axillary lymph node enlargement, pleural effusion and hypergammaglobulinemia. The patient was treated in internal medicine with combined chemotherapy with steroids which resulted in a complete remission. However, he returned almost 1 year later with an ulcerative lesion in the left palatine tonsil, with no lymph node involvement. Considering that the patient was an elderly person, chemotherapy had been administered during the initial admission, tumor cells appeared to originate from T cells and relapse occurred only in the local lesion, the lesion was treated by local irradiation using 60Co at a dose of 40 Gy, which produced a complete resolution. radiotherapy appeared to be effective for the treatment of localized lesions of AILD.- - - - - - - - - - ranking = 0.5keywords = hypergammaglobulinemia (Clic here for more details about this article) |