1/131. Reactive angioendotheliomatosis in an infant.Reactive cutaneous angioendotheliomatosis (RCA) is an uncommon benign disease characterized by intravascular proliferation of endothelial cells. The observation of RCA in infants is exceedingly rare. We describe a case of RCA in a 3-month-old infant. The lesions were characterized by six small purpuric papules (1-2 mm in diameter), distributed on the thighs and neck. The general condition of the patient was good, with no lymphadenopathy, systemic involvement, or fever. The histopathologic features of a papule were characterized by the presence of cohesive aggregates of large mononucleated cells protruding into the lumina of dilated vessels and filling some of them completely. Neither an inflammatory infiltrate nor a proliferation of pericytes were present around blood vessels. Intravascular proliferating cells demonstrated positive staining for ulex europaeus agglutinin 1 (UEA-1) and for factor viii-RA and CD34 antigens. The course of the disease was unremarkable with persistence of the lesions for 8 months; no treatment was started.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/131. Cryopathic gangrene with an IgM lambda cryoprecipitating cold agglutinin.Immunochemical and serologic studies of cold agglutinis in patients with chronic cold agglutinin disease (CCAD) have shown the almost exclusive occurrence of IgM kappa antibodies with specificity for the I antigen of red cells. An unusual subgroup of patients has been delineated in which the cryoprotein is IgM lambda, frequently lacks I specificity and often cryoprecipitates. Studies of such a protein from a patient with an unusual array of immunoproliferative disorders including Grave's disease with exophthalmos and Waldenstrom's macroglobulinemia indicate that the cryoprecipitating and cold agglutinating properties probably derive from the sam protein. The occurrence of this type of antibody should suggest the presence of a more aggressive lymphoproliferative disorder than simple CCAD.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/131. autoimmunity and extranodal lymphocytic infiltrates in lymphoproliferative disorders.OBJECTIVE: To examine the relationship between autoimmunity and extranodal lymphocytic infiltrates in different lymphoproliferative disorders with immunoglobulin alterations. SUBJECTS AND DESIGN: A clinical review combined with a retrospective cohort study of 380 patients, 28 with monoclonal gammopathy of undetermined significance, three with common variable immunodeficiency, 147 with chronic lymphocytic leukaemia, 57 with Waldenstrom's macroglobulinaemia and 145 with non-Hodgkin's malignant lymphoma. SETTING: A university hospital and The State serum Institute in Copenhagen. INTERVENTION: Clinical examination of each patient with special attention to chronic inflammatory and autoimmune manifestations. Biopsies were taken from non-infectious infiltrates, some of which were additionally tested with PCR analysis for gene rearrangements. Serological screening with a test battery for various autoantibodies was used in combination with techniques for the detection of M-components and monoclonal B-cell proliferation. MAIN OUTCOME MEASURES: Clinical and/or serological autoimmune manifestations, M-component and other immunoglobulin alterations, and inflammatory tissue changes were studied in patients with chronic inflammatory, polyclonal or oligoclonal pseudolymphomas and in monoclonal, malignant extranodal lymphomas. RESULTS: In 380 consecutive patients, 49 (12.9%) had extranodal manifestations, of whom 47 also had autoimmune manifestations. Nearly half of the 47 patients had more than one autoimmune manifestation. There was a strong correlation between clinical signs and corresponding autoantibodies such as anti-SSA and -SSB antibodies in sjogren's syndrome (10 cases), antithyroid peroxidase antibodies in thyroiditis and Graves' disease (10 cases), and parietal cell antibodies in gastric ulcers with maltoma (12 cases). Clinical and serological signs of autoimmunity correlated strongly with female sex (34, 72% women; and 13, 28% men) and with immunoglobulin alterations. CONCLUSIONS: To our knowledge this is the first systematic review of B-lymphoproliferative and autoimmune disorders indicating that pseudolymphoma and malignant lymphomas, including maltomas, may develop in the context of a permanent autoantigenic drive.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/131. Pulmonary asbestosis associated to pleural non-Hodgkin lymphoma.We describe herein a case report of a patient affected by pulmonary asbestosis who developed a non-Hodgkin lymphoma originating in the pleura. The case is unusual for the uncommon site and because the chronic antigenic stimulation by asbestos bodies may have locally promoted an immunologic derangement.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/131. Reactive angioendotheliomatosis or intravascular histiocytosis? An immunohistochemical and ultrastructural study in two cases of intravascular histiocytic cell proliferation.Two elderly women with complex medical histories presented with erythematous patches, in one case involving the face and forearms, and in the other both elbows. Punch biopsies from both patients revealed intravascular proliferations of medium-sized and large cells with luminal occlusion typical of angioendotheliomatosis. Immunostaining did not show either lymphocytic or endothelial cell antigens but was consistent with a histiocytic differentiation of the intravascular cells in both cases, and was further substantiated by ultrastructural examination in one case. One patient received a course of cyclophosphamide therapy over 15 days. skin lesions faded but did not disappear. The patient died 10 months later from cardiac and renal failure, which was most probably unrelated to the skin lesions. In the other case, lesions diminished but did not entirely resolve with treatment with low doses of oral prednisone. Angioendotheliomatosis can be divided into a malignant variant, which is an angiotropic lymphoma mostly of B-cell phenotype, and a benign, reactive variant, which is characterized by a proliferation of cells expressing endothelial cell markers. Only one case of angioendotheliomatosis with cells of histiocytic differentiation has been published previously under the name of intravascular histiocytosis. Our cases are very similar to the latter. The question arises as to whether intravascular histiocytic cell proliferation is a neoplastic proliferation of histiocytes or an early stage of classic reactive angioendotheliomatosis representing the residual cells associated with organization of microthrombi, which will be later followed by endothelial cell proliferation.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/131. Nasal and nasal-type T/NK-cell lymphoma with cutaneous involvement.Natural killer (NK) cells are a third lymphocyte lineage, in addition to B- and T-cells, that mediate cytotoxicity without prior sensitization. NK cells also have phenotypic and genotypic characteristics; they express the NK-related antigen CD56 and T-cell markers such as CD2 and CD3 epsilon, but their T-cell receptor (TCR) locus is not rearranged. Non-Hodgkin's lymphomas are divided into B- and T-cell neoplasms and NK-cell lymphomas. We describe 2 Japanese patients with nasal and nasal-type T/NK-cell lymphoma in which the skin, nasal/nasopharyngeal region, bone marrow, and lymph node were the sites of involvement. The clinical and histopathologic findings were recorded. In addition, immunophenotyping, TCR gene rearrangement, and the existence of Epstein-Barr virus (EBV) dna by polymerase chain reaction amplification were determined. Clinically, the cutaneous eruptions were purplish, hard, multiple nodules. Histologically, angiocentric proliferation of small-to medium-sized, pleomorphic, lymphoid cells were observed. They revealed hand-mirror-shaped lymphocytes with azurophilic granules with the use of Giemsa staining by touch smear. These lymphocytes were found to be positive to immunophenotyping for CD2 (Leu5b), CD3 epsilon (DAKO), CD4 (Leu3a), and CD56 (Leu 19). No clonal rearrangement of TCR-beta, -gamma, and -delta genes and immunoglobulin gene markers were found, and no positive results of identification of EBV dna were shown. The patients underwent cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy with complete remission; however, both had recurrence of disease. Because NK-cell lymphomas express some T-cell markers, they may be mistakenly diagnosed as peripheral T-cell lymphomas if they are not investigated for the NK-cell-specific marker, CD56. Therefore the importance of immunophenotypic investigations of CD56 should be stressed. Also, the importance of clinical investigation of nasal/nasopharyngeal lymphomas should be stressed when NK-cell lymphoma is diagnosed involving the skin, because NK-cell lymphomas are often associated with the nasal and nasopharyngeal region.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/131. Primary cutaneous Ki-1(CD30) positive anaplastic large cell lymphoma in childhood.Primary cutaneous Ki-1(CD30) positive anaplastic large cell lymphoma (ALCL) is an unusual tumor in the pediatric population. However, the nodal-based form of the disease compared with other histologic subsets of childhood non-Hodgkin's lymphomas (NHL) more frequently involves skin, soft tissue, and bone. The objective of this article is to determine the histologic and immunologic characteristics of childhood primary cutaneous Ki-1(CD30) positive ALCL and its prognosis. The clinical data, histologic features and immunohistochemical profiles of skin biopsy specimens from 3 children with cutaneous Ki-1(CD30) positive lymphoma were reviewed. A literature search was performed and disclosed information on 5 childhood cases. The 3 patients with primary cutaneous Ki-1(CD30) positive ALCL all presented similarly as rapidly growing masses initially and clinically believed to be infectious/reactive processes. The diagnosis was established on the basis of histopathologic examination and immunohistochemical studies. Histologic sections revealed an extensive infiltrate of tumor cells extending throughout the entire dermis into the subcutaneous fat with frank ulceration in 1 patient. No significant epidermotropism was noted. Tumor cells exhibited striking cellular pleomorphism and a high mitotic rate with numerous atypical mitoses. Inflammatory cells were present in all patients. The tumor cells stained positively for Ki-1 antigen (CD30), epithelial membrane antigen, and for T-cell markers (UCHL-1, CD3). One of 3 cases, however, failed to stain for leukocyte common antigen (LCA). No clinically apparent adenopathy was observed in any of the patients. In all instances the patients developed recurrent disease in the skin at sites separate from the primary location. None of the patients demonstrated any involvement of lymph nodes, bone marrow, or other organ systems. All patients were treated with chemotherapy with good response. Primary cutaneous Ki-1(CD30) positive lymphoma is rare in children and is characterized by recurrences. The prognosis seems to be favorable.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
8/131. Primary malignant lymphoma of brain. A review of four cases diagnosed by means of CT-guided stereotactic biopsy.In a series of 66 CT-guided stereotactic biopsies (SB) of the brain performed in 1995-1998, four were found to be the primary non-Hodgkin's lymphomas (PCNSL). All cases were studied with immunohistochemistry (broad panel of antibodies; reactions performed mainly on cytological smears) and with the use of an electron microscopy. In an immunophenotyping all cases were positive for leukocyte common antigen (LCA) and 3/4 showed B-cell phenotype. Since the PCNSL are typically located in central, periventricular region of brain and the surgical removal does not bring any benefit, the stereotactic biopsy is a method of choice to make a definite diagnosis that opens the chances for implementation of chemo- and radiotherapy. The diagnostic difficulties that are derivatives of an extremely small amount of the available biopsy material were discussed and the role of the immunophenotyping and of the electron microscopy for avoiding the possible diagnostic mistakes was stressed. Cytological smears stained with H&E and the smears and histological slides with immunohistochemical reaction against glial fibrillary acidic protein (GFAP) showing sometimes extremely dense network of astrocytes mingled with neoplastic lymphoma cells are especially interesting and seem to suggest the involvement of astroglia in the pathogenesis of PCNSL.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/131. Fine needle aspiration cytology of primary non-Hodgkin's lymphoma of the tongue. A case report.BACKGROUND: Fine needle aspiration cytology (FNAC) of extranodal non-Hodgkin's lymphoma of the tongue has rarely been described. CASE: A 47-year-old male was referred to the cytology laboratory for FNAC of a 3-cm-diameter swelling on the dorsum of the tongue, with a primary clinical diagnosis of soft tissue tumor. FNAC smears showed discrete, monomorphic, round to oval cells with scanty, deep blue cytoplasm. The nuclear margin was regular, with occasional prominent nucleoli and fine nuclear chromatin. The background showed many lymphoglandular bodies. The cells were strongly positive for leukocyte common antigen. A cytologic diagnosis of high grade non-Hodgkin's (NHL) was offered and subsequently confirmed by histopathology. CONCLUSION: Primary NHL of the tongue is relatively rare. As there are no characteristic clinical features of extranodal NHL of the tongue, FNAC may be useful for rapid diagnosis and management of such cases.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/131. Therapy-related megakaryoblastic leukemia with pituitary involvement following treatment for non-Hodgkin's lymphoma.A case of a 66-year-old Japanese man developed therapy-related megakaryoblastic leukemia with pituitary involvement after chemotherapy for non-Hodgkin's lymphoma. alkylating agents had been administered for the treatment of non-Hodgkin's lymphoma and 6 years later, megakaryoblastic leukemia with myelofibrosis and myelodysplasia developed. The blast cells expressed CD41, and immature antigens also. These findings were compatible with therapy-related megakaryoblastic leukemia. An autopsy revealed blast-cell infiltration into multiple organs including the posterior pituitary lobe. Therapy-related megakaryoblastic leukemia is very rare, and pituitary involvement may be associated with immaturity of blast cells.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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