1/25. Cutaneous involvement by angioimmunoblastic T-cell lymphoma with remarkable heterogeneous Epstein-Barr virus expression.INTRODUCTION: Initially described as an abnormal immune reaction, most cases of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD)-like T-cell infiltrates are now regarded as a peripheral T-cell lymphoma (AILD T-NHL). AILD T-NHL is characterized clinically with constitutional symptoms, generalized lymphadenopathy, hepatosplenomegaly, skin rash, and polyclonal hypergammaglobulinemia. Epstein-Barr virus (EBV) is frequently detected in involved lymph nodes, but the presence of EBV in cutaneous infiltrates of AILD T-NHL has rarely been examined. We present a patient with AILD T-NHL with cutaneous involvement that shows marked heterogeneity of EBV expression in the lymph node and skin biopsies, and review the histological findings of AILD T-NHL in the skin. methods: Two skin biopsies of a diffuse maculopapular rash and a lymph node were examined and immunophenotyped. in situ hybridization for detection of EBV in the lymph node and skin biopsies was utilized. In order to attempt to delineate which lymphocytes were EBV positive, skin biopsies were dual labeled with CD3, CD45RO, CD20 and EBV. The skin biopsies and lymph node were submitted for gene rearrangement studies by polymerase chain reaction (PCR). Capillary electrophoresis of fluorescently labeled PCR products was utilized for PCR product quantitation. RESULTS: The histological features of the lymph node were diagnostic of AILD T-NHL and a T-cell clone was identified by PCR. The skin biopsies showed an atypical superficial and deep perivascular polymorphous infiltrate consistent with cutaneous involvement by AILD T-NHL. Both skin biopsies showed the same clonal T-cell receptor gene rearrangement as the lymph node. in situ hybridization of the lymph node and one skin biopsy showed a few scattered EBV-positive lymphocytes (<1% of the infiltrate). A second skin biopsy revealed 40-50% of the lymphocytes as EBV positive. Dual staining for CD20 and EBV identified a minority of EBV-infected lymphocytes as B-cells, but most of the EBV-positive cells lacked staining for CD3 and CD45RO. CONCLUSIONS: In our patient, the same T-cell receptor gene rearrangement was found by PCR in all three biopsy sites. Most cases of AILD T-NHL contain only a few EBV-positive cells, but in our patient the extent of EBV expression ranged from <1% to 40-50% of the AILD T-NHL cutaneous infiltrate. To our knowledge, this case is the most extensive and heterogeneous expression of EBV in cutaneous AILD T-NHL to date.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
2/25. Angioimmunoblastic lymphadenopathy (AILD) may respond to thalidomide treatment: two case reports.The histological morphology of AILD-type T-cell lymphoma shows proliferation of small, arborizing high endothelial venules. patients typically have generalized lymphadenopathy, fever, weight loss, skin rash, polyclonal hypergammaglobulinemia and autoimmune phenomena, and are susceptible to infections. The clinical course is usually aggressive. About 30% of patients have an initial remission on steroids alone, but most require some form of cytotoxic chemotherapy. Median survival ranges from 15 to 24 months, and it appears that the disease cannot be cured. We have used anti-angiogenetic therapy with thalidomide in two AILD patients and observed remarkable responses.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
3/25. Angioimmunoblastic lymphadenopathy following ciprofloxacin administration.Angioimmunoblastic lymphadenopathy (AILD) is a rare disorder characterised by generalised lymphadenopathy, fever, hepatosplenomegaly, immune hemolytic anemia and polyclonal hypergammaglobulinemia. We report the occurrence of histology-proven AILD in a patient who had received ciprofloxacin. We suggest that this drug may be added to the list of agents susceptible to elicit AILD.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
4/25. 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 = 2keywords = hypergammaglobulinemia (Clic here for more details about this article) |
5/25. Epstein-Barr virus-related lymph node lesion resembling autoimmune disease-like clinicopathological findings in elderly patients. Report of three cases.Three cases of Epstein-Barr virus (EBV)-related lymphoproliferative disorders in elderly patients showing autoimmune disease-associated lymphadenopathy-like clinicopathological findings have been reported. Clinically, they were characterized by systemic lymphadenopathy, "B" symptoms, polyclonal hypergammaglobulinemia, elevated serum LDH and transient presence of various autoantibodies, and absence of atypical lymphocytosis in peripheral blood. One case was associated with idiopathic thrombocytopenic purpura. The clinical course was self-limiting. Histologically, they exhibited numerous lymphoid follicles with hyperplastic germinal centers and atypical interfollicular widening with prominent vascular proliferation. In the paracortical area, there was a mixed infiltrate comprising small to medium-sized lymphocytes and plasma cells, and variable numbers of eosinophils and T- and B-immunoblasts. in situ hybridization demonstrated a varying number of EBV-infected lymphocytes in the germinal center as well as in the interfollicular area. polymerase chain reaction demonstrated that neither clonal rearrangement of T-cell receptor gamma-gene nor immunoglobulin heavy-chain rearrangement was detected in two of the cases examined. Although acute EBV infection rarely occurs in older adults, EBV related to reactive lymphoproliferative disorder should be added to the differential diagnosis of autoimmune disease-associated lymphadenopathy and node-based peripheral T-cell lymphoma in elderly patients.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
6/25. immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma in a child.We report the case of a 14-year-old Japanese boy with peripheral T-cell malignant lymphoma, showing progression from immunoblastic lymphadenopathy (IBL) to overt malignant lymphoma. He suffered recurrent fever, generalized lymphadenopathy, hepatosplenomegaly and maculopapular exanthema. leukocytosis with eosinophilia and polyclonal hypergammaglobulinemia were observed during the aggressive course of the disease. In the early phase, human immunoglobulin and steroids improved the symptoms but did not induce complete remission, and the patient died one year after the onset of the illness. Four biopsies of lymph nodes revealed progression from IBL to CD4 positive T-cell lymphoma through IBL-like T-cell lymphoma. Though IBL-like T-cell lymphoma is defined as IBL with neoplastic features and overt T-cell malignant lymphoma progressed from IBL-like T-cell lymphoma is excluded from the definition, it may be preferable that such malignant lymphoma as our case should also be included in IBL-like T-cell lymphoma.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
7/25. IBL-like T cell lymphoma expressing monoclonal gammopathy (macroglobulinemia) in the serum.A case of IBL-like T cell lymphoma with serum monoclonal gammopathy was reported. A 58-year-old woman, who had suffered from heart failure, was admitted because of asthma attack, fever and lymphadenopathy. Leucopenia with a small amount of atypical lymphocytes was detected. serum analysis showed monoclonal elevation of IgM-kappa (M-protein) and hyperviscosity. Urinary Bence-Jones protein was detected. Lymph node biopsy revealed the disappearance of normal structure and proliferation of T cells with pale cells which characterized IBL-like T cell lymphoma. Immunocytochemistry revealed the pale cells to bear T cell markers (MT-1, CD 5, CD 8 or CD 4) and IgM-positive cell distribution. Tonsilar biopsy showed the infiltration of atypical lymphoids and pale cells. bone marrow biopsy showed moderate lymphoplasmacytoid proliferation with lymph follicles. Clinical data and serum analysis suggested macroglobulinemia. Additional lymph node biopsy was performed and revealed IBL-like T cell lymphoma. IBL-like T cell lymphoma is characterized by polyclonal hypergammaglobulinemia. The present case probably occurred initially as IBL-like T cell lymphoma and lymphoplasmacytoid cell proliferation might have followed due to an excess of CD 4 cells.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
8/25. 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 = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
9/25. immunoblastic lymphadenopathy-like T-cell lymphoma with multiple cutaneous and visceral involvements.Morphologic and immunological findings found in a patient with immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma (IBL-T) are presented. Though the initial clinical features were suggestive of IBL, multiple cutaneous and visceral tumors appeared later in his course. The cutaneous lesion is considered to be unique, because the neoplastic T cells with suppressor/cytotoxic (S/C) phenotype showed focal epidermotropism, resulting in necrosis and ulceration of the overlying epidermis. An interesting feature in IBL-T is the frequent association of polyclonal hypergammaglobulinemia, yet the neoplastic T cells show S/C phenotype. Since Ia-like antigen was expressed on the neoplastic T cells, it is stressed that antigen-presenting and contrasuppressor cells should also be included in the cell populations which have a possibility to be a normal counterpart of IBL-T.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
10/25. Sinus histiocytosis with massive cervical lymphadenopathy. Case report and literature review.Sinus histiocytosis with massive cervical lymphadenopathy (SHML) was originally described in 1969 as a benign clinicopathologic entity characterized by massive bilateral cervical lymphadenopathy, fever, leukocytosis, elevated ESR, and hypergammaglobulinemia, usually occurring within the first two decades of life. We present an illustrated case of an elderly patient with polyclonal hypergammaglobulinemia and a 2-year history of multilobulated cervical and submandibular lymphadenopathy. The etiology and pathogenesis of SHML are not known. diagnosis requires lymph node biopsy to exclude other causes of cervical lymphadenopathy such as malignant lymphoma, malignant histiocytosis, metastatic carcinoma, and tuberculous lymphadenitis. Histologic examination shows marked dilatation of subcapsular and medullary lymph node sinuses containing large, foamy or vacuolated histiocytes. Although no curative treatment is known, corticosteroids, radiation therapy, vinblastine and oral cyclophosphamide, and surgery have been used to palliate constitutional symptoms and mechanical obstruction from massive lymphadenopathy. Since one third of SHML patients have evidence of disease for 5 years, and a mortality rate of 7% exists with benign histologic disease, all patients with SHML should be carefully screened for evidence of immunodeficiencies that may precipitate a fatal outcome.- - - - - - - - - - ranking = 2keywords = hypergammaglobulinemia (Clic here for more details about this article) |
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