1/12. 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) |
2/12. A male with angioimmunoblastic T-cell lymphoma and proliferative glomerulonephritis.In this article we present the case report of a 67-year-old male with a nephrotic syndrome due to a proliferative glomerulonephritis, associated with an angioimmunoblastic T-cell lymphoma. diagnosis was made on an axillary lymph node biopsy and showed expanded T-cell areas with multiple blood vessels, small mature lymphocytes, eosinophils, and plasma cells. A kidney biopsy was suggestive for a proliferative glomerulonephritis with intra- and extracapillary proliferation. Hypercellular glomeruli were seen, as well as multiple floride crescents. Interstitial edema and fibrosis were absent. Immunohistochemical reactions were negative; there was some mesangial reaction with IgM in the glomeruli. Treatment with high-dose corticosteroids was initiated, with clinical improvement, and was immediately followed by therapy with cyclophosphamide, hydroxydaunomycin, vincristine, and prednisone (CHOP), which induced complete remission with a follow-up of 1 year. To our knowledge, the association of angioimmunoblastic T-cell lymphoma and proliferative glomerulonephritis has only been described twice. It concerned elderly men who developed acute renal failure a couple of months after the diagnosis of an angioimmunoblastic T-cell lymphoma. In both, immunoglobulin-containing dense deposits within glomeruli were observed, which was not the case in our patient, where only some mesangial colorization of the IgM in the glomeruli was seen.- - - - - - - - - - ranking = 18.396683034201keywords = blood vessel, vessel (Clic here for more details about this article) |
3/12. Atypical lymphoplasmacytic and immunoblastic proliferation from systemic lupus erythematosus. A case report.A case of atypical lymphoplasmacytic and immunoblastic proliferation (ALPIBP) in the lymph nodes associated with well-documented systemic lupus erythematosus (SLE) is presented. A 30-year-old Japanese female with an 18-year history of SLE presented with right neck lymphadenopathy of 3 months duration. A biopsy specimen showed a diffuse effaced lymph node architecture without follicles and minimal sinuses. At high power field, a polymorphous population of small- to medium-sized lymphocytes, plasma cells, plasmacytoid cells, as well as large, basophilic transformed lymphocytes and immunoblasts diffusely infiltrated the paracortical area. Interestingly, the immunohistochemical study demonstrated large, irregularly shaped accumulations of follicular dendritic cells (FDCs) surrounding the small vessels, which is an immunohistochemical finding characteristic of angioimmunoblastic T-cell lymphoma (AILT). However, the present lesion showed the following differences to AILT: (a) absence of CD3 , CD4 and CD10 clear cells, which are tumor cells of AILT; (b) absence of pronounced arborizing vascular proliferation; (c) on molecular analysis, the present case demonstrated a polyclonal pattern converse to the monoclonal T-cell receptor gamma chain gene rearrangement in most AILTs (d) absence of EBV infected lymphoid cells, which are frequently detected AILT. As previously suggested, the present case indicates that a clinical correlation as well as immunohistologic and genotypic studies may be necessary to discriminate between ALPIBPs and AILT.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/12. association of Epstein-Barr virus with an angioimmunoblastic lymphadenopathy-like lymphoproliferative syndrome.A patient presented with fever, night sweats, generalized lymphadenopathy, cold agglutinin hemolytic anemia, and thrombocytopenia. Lymph node biopsy revealed a lymphoplasmacytic infiltrate sparing the cortical sinuses but associated with arborizing blood vessels. Epstein-Barr virus (EBV) dna was demonstrated in tissue from an involved lymph node. These data suggest an association of EBV with a lymphoproliferative process that resembles angioimmunoblastic lymphadenopathy with dysproteinemia.- - - - - - - - - - ranking = 18.396683034201keywords = blood vessel, vessel (Clic here for more details about this article) |
5/12. Peripheral neuropathy associated with angioimmunoblastic lymphadenopathy.A 49-year-old man developed mononeuritis multiplex associated with angioimmunoblastic lymphadenopathy. The biopsy of the sural nerve revealed focal reduction of myelinated fibres and axonal degeneration, as well as perivascular inflammatory infiltrates composed of lymphocytes and plasma cells, exhibiting policlonal immunoglobulin expression, proliferation of blood vessels, thickening of the vessel wall and endothelial hyperplasia. These latter changes are similar to those commonly encountered in the lymph nodes, as well as in other organs, in patients suffering from angioimmunoblastic lymphadenopathy.- - - - - - - - - - ranking = 19.396683034201keywords = blood vessel, vessel (Clic here for more details about this article) |
6/12. Angio-immunoblastic lymphadenopathy (AIL) or T-cell malignant lymphoma of AIL-type. A histopathological, immunohistochemical and ultrastructural study of 8 cases.Eight cases of AIL-type T-cell malignant lymphoma are reported. The clinical symptoms are the same as those described in AIL: fever, malaise, weight loss, skin rashes, polyadenopathy, and splenomegaly. However, some differences can be noted: the absence of hepatomegaly in all cases but one, the absence of polyclonal hypergammapathy in all cases but one, and predominance in females. The lymph node modifications comprise diffuse infiltrations of lymphoid cells with irregular nuclei and pale cytoplasm, associated with a large number of immunoblasts and plasma cells. Some eosinophilic granulocytes and epithelioid cells can be seen. hyperplasia of the vessels and remnants of follicles, sometimes with proliferation of follicular dendritic cells, are prominent features. The immunolabelling study demonstrates the presence of an important T-cell population all expressing a high predominance of CD 4 phenotype. These findings are in accordance with those published in europe and in contrast with those of some of the Japanese cases, particularly the first patients published by Shimoyama et al. The differential diagnosis with AIL is based on the presence of clusters of mainly large cells with a pale cytoplasm, on the loss of expression of one T cell marker, as in 3 cases of our series, and on the presence of a high percentage of lymphoid cells engaged in the mitotic cycle as demonstrated with the Ki 67 monoclonal antibody. However, to draw a clear cut difference between AIL-type T-cell lymphoma and AIL considered as a prelymphomatous dysimmune lymphadenopathy, only the demonstration of cytogenetic abnormalities, as in one of our cases or of rearrangement of the genes coding for beta and/or gamma chain of the antigen receptor of T-cell are valuable criteria. The follow-up of our series is not long enough to appreciate the prognosis. Three patients died, one from a glioma. All the other cases, treated with polychemotherapy show total remission with an evolution of 10 to 39 months.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/12. A case report of the immunodysplasia syndrome and heavy chain disease associated with subacute bacterial endocarditis.A 36-year-old man was admitted to Saitama Medical School Hospital, because of a remittent fever which had continued for approximately 6 months, hepatosplenomegaly and lymphadenopathy. He had direct Coombs' test positive auto-immune hemolytic anemia associted with subacute bacterial endocarditis (SBE). The lymphnode demonstrated focal diffuse proliferation of immunoblasts and arborizing vessels with a few small germinal centers, which resembled histological features of the immunoblastic lymphadenopathy. The immunochemical analysis revealed the presence of free IgG Fc fragments in serum. From the above results the patient was diagnosed as immunodysplasia syndrome (IDS) and heavy chain disease (HCD) associated with SBE. It was suggested that the chronic antigenic stimulation due to SBE might have some role in the mechanism of the development of the IDS and HCD in our patient.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/12. Angioimmunoblastic lymphadenopathy after infectious mononucleosis.Angioimmunoblastic lymphadenopathy occurred in a 46-year-old man 16 months after an episode of infectious mononucleosis induced by Epstein-Barr (EB) virus. The features of infectious mononucleosis included fever, pharyngitis, lymph gland enlargement, hepatosplenomegaly, hyperbasophilic mononuclear cells, and IgM antibodies to EB virus, although heterophile antibodies were not detected. The illness was severe and prolonged and included an asymptomatic measles virus infection. Over a year later massive enlargement of the lymph nodes led to a biopsy, which showed a diffuse infiltration with lymphoid cells and a proliferation of arborising small vessels typical of angioimmunoblastic lymphadenopathy. In spite of corticosteroids, levamisole, chlorambucil, and radiotherapy, no remission occurred, and serious infections led to death 18 months after the onset. Viral infections with EB virus and measles virus associated with pre-existing or subsequent immunological changes probably resulted in the appearance of angioimmunoblastic lymphadenopathy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/12. 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 = 18.396683034201keywords = blood vessel, vessel (Clic here for more details about this article) |
10/12. immunoblastic lymphadenopathy in a five-month-old girl: successful treatment with immunosuppressants.A five-month-old girl developed high fever, erythema, hepatosplenomegaly and generalized lymphadenopathy. Laboratory examinations revealed elevated peripheral leukocyte counts, c-reactive protein, lactate dehydrogenase and serum ferritin level. Pathologic examination of the lymph nodes revealed immunoblastic lymphadenopathy (IBL) on the basis of the complete effacement of the normal architecture, replacement by a diffuse infiltrate composed of immunoblasts, plasmacytoid cells and small lymphocytes, and an abortive proliferation of blood vessels. B-cells and T-cells were nearly equally mixed throughout the lymph nodes. No rearrangements of the B-cell immunoglobulin and T-cell receptor genes were detected. The patient was initially treated with alpha-interferon with dramatic efficacy. After relapse, however, the disease was well controlled with cyclosporin A (CyA) and subsequently with combination regimens of CyA, deoxyspagarin and azathioprine with fair success. An alternating regimen of 6-mercaptopurine, cyclophosphamide and methotrexate was then instituted which continued the complete remission for 12 months. The effects of immunosuppressants in the treatment of IBL merit investigation.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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