1/29. Signet-ring cells in a Waldenstrom's Macroglobulinaemia.A case of signet-ring cell lymphoma affecting bone marrow is reported. The patient was diagnosed as Waldenstrom's Macroglobulinaemia on the basis of clinical and laboratory features including morphology, immunohistochemistry and gene rearrangement studies. light microscopy examination showed cells contained large globular inclusions (signet-ring cells) that stained for kappa immunoglobulin light chain by immunohistochemistry. In addition, the neoplastic cells expressed the common leukocyte antigen CD45 and the B cell marker CD19. This to the best of our knowledge is the first report of a patient with Waldenstrom's Macroglobulinaemia with the presence of vacuolated signet ring- cells in the bone marrow. Differential diagnosis arises with non-haemopoietic tumours and this needs to be based on specific immunostaining. Tumours and this need to be based on specific immunostaining.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/29. 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) |
3/29. 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) |
4/29. A case of acquired autoimmune bullous disease associated with IgM macroglobulinaemia.A variety of autoimmune bullous dermatoses have been reported to develop in association with lymphoproliferative disorders. We report a patient with IgM macroglobulinaemia, who presented with a skin fragility similar to but somewhat milder than that seen in epidermolysis bullosa acquisita. Immunofluorescence detected circulating IgM autoantibodies reacting with the basement membrane zone, which reacted predominantly with dermal side of 1 M NaCl-split skin. immunoblotting of the epidermal and dermal extracts with the patient's serum showed no specific reactivity. Further studies are needed to identify the antigenic molecules responsible for the IgM deposition.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/29. Treatment of plasma cell dyscrasias by antibody-mediated immunotherapy.The use of serotherapy to treat patients with plasma cell dyscrasias (PCDs) has been sought by us and others. Candidate antigens that have been targeted or proposed for targeting in PCDs include the immunoglobulin idiotype, CD19, CD38, CD54, CD126, HM1.24, and Muc-1 core protein. Unfortunately, many of these antigens are not ideal for use in serotherapy since they are not selectively expressed, are either shed or secreted, or have not been fully characterized. Serotherapy with an anti-CD19 monoclonal antibody (B4) conjugated to a blocked ricin toxin had no significant activity in patients with multiple myeloma (MM). Circulating CD20 clonotypic B cells have been detected in the circulation of most MM and Waldenstrom's macroglobulinemia (WM) patients. plasma cells from most WM patients express CD20, but most MM patient plasma cells either lack CD20 or express it weakly. In view of recent successes with anti-CD20-directed serotherapy in other B-cell malignancies, we initiated a phase II trial to study the anti-CD20 monoclonal antibody rituximab (Rituxan; IDEC Pharmaceuticals, San Diego, CA, and Genentech, Inc, san francisco, CA) in patients with MM. We describe two PCD patients (one with WM and one with MM) who responded to therapy. By flow cytometric analysis, CD20 plasma cells and B cells present in the bone marrow and peripheral blood of a patient with MM disappeared with response to rituximab therapy. However, residual CD20- tumor cells remained in the bone marrow following rituximab therapy, and after 6 months this patient progressed with CD20- myeloma cells. As a potential strategy to overcome this limitation, we demonstrated that interferon-gamma at pharmacologically achievable levels induced CD20 expression on these CD20- plasma cells, consistent with our recent findings that interferon-gamma is a potent inducer of CD20 expression on MM patient plasma cells and B cells. We also characterize a response to rituximab with a decrease in paraprotein and resolution of anemia in a patient with WM whose response to rituximab is ongoing after 19 months. This preliminary experience supports the potential use of serotherapy targeting CD20 in PCDs. Our studies further suggest that interferon-gamma may enhance CD20 expression on MM plasma cells, thereby increasing their susceptibility to anti-CD20 monoclonal antibody therapies.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
6/29. Hyperviscosity syndrome secondary to a myeloma-associated IgG(1)kappa paraprotein strongly reactive against the hiv-1 p24 gag antigen.Hyperviscosity syndrome secondary to hypergammaglobulinemia is a rare and potentially fatal complication in patients with human immunodeficiency virus type-1 (hiv-1) infection. We studied an hiv-1-positive patient with symptomatic hyperviscosity attributable to IgG(1)kappa multiple myeloma. The patient initially responded to plasmapheresis and was subsequently treated with cytotoxic immunosuppressive chemotherapy. The patient remained asymptomatic during a 3-year follow-up period. The monoclonal IgG(1)kappa gammopathy evolved to a biclonal variant of the same subtype with an expansion of marrow plasma cell population. Western blot analysis demonstrated that this myeloma-associated paraprotein was strongly reactive against the hiv-1 p24 gag antigen.- - - - - - - - - - ranking = 5keywords = antigen (Clic here for more details about this article) |
7/29. Lymphoplasmacytic lymphoma/waldenstrom macroglobulinemia associated with Hodgkin disease. A report of two cases.Although the clinical course of lymphoplasmacytic lymphoma (LPL)/waldenstrom macroglobulinemia (WM) is usually indolent, high-grade non-Hodgkin lymphoma may develop in a small subset of patients. We have not found any patients with LPL/WM associated with hodgkin disease (HD) described in the literature, prompting us to report 2 cases. In case 1, the patient had LPL/WM involving bone marrow diagnosed 1 week before left supraclavicular lymph node biopsy revealed LPL/WM and classical HD. In case 2, the patient had a 15-year history of LPL/WM before classical HD developed involving bone marrow, liver, and lymph node. Both cases were positive for IgM, monotypic immunoglobulin light chain, and B-cell antigens and were CD3-. The neoplastic Hodgkin cells were CD15 , CD20 (case 1), CD30 , CD3-, and CD45- and were negative for Epstein-Barr virus rna. Both patients were treated with chemotherapy for HD. In case 1, clinical response was excellent with no histologic evidence of HD in subsequent biopsy specimens. In case 2, HD was progressive at last follow-up, despite therapy. patients with LPL/WM, similar to patients with other types of low-grade B-cell lymphoma, can develop HD that may respond to chemotherapy.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/29. Transfusion-associated graft-versus-host disease in a patient with Waldenstrom's macroglobulinaemia.BACKGROUND AND OBJECTIVES: Routine irradiation of cellular blood products is not presently recommended for patients with non-Hodgkin's lymphoma (NHL). MATERIALS AND methods: We report the case of a 72-year-old-man with Waldenstrom's macroglobulinaemia who developed transfusion-associated graft-versus-host disease (TA-GvHD) 13 days following a non-irradiated red cell transfusion. RESULTS: The patient had not previously received purine analogues and none of the donors was homozygous for a human leucocyte antigen (HLA) haplotype that was shared by the recipient. Therefore, his only apparent risk factor was lymphoplasmacytoid NHL. CONCLUSIONS: This case further strengthens the argument that NHL per se is a risk factor for TA-GvHD and supports the proposal that the guidelines for prophylactic irradiation of cellular blood products be extended to include all cases of NHL.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/29. Lymphoplasmacytic lymphoma with monoclonal gammopathy-related pseudo-Gaucher cell infiltration in bone marrow and spleen--diagnostic and therapeutic dilemmas.Gaucher-like cells have occasionally been described in various haematological malignancies including Hodgkin's disease, non-Hodgkin's lymphoma, multiple myeloma (MM) and chronic myelogenous leukaemia (CML). A special type of this phenomenon is crystal-storing histocytosis or the so-called pseudo-pseudo Gaucher cells (PPGC) in which crystalline protein storage in macrophages is induced by paraproteinemia. Here we describe a 54-year-old man with an initial suspicion of gaucher disease and monoclonal IgA gammopathy in whom a correct diagnosis of lymphoplasmacytic lymphoma (LPL) with massive infiltration of bone marrow and spleen by PPGC was confirmed by immunological, ultrastructural and molecular characterisation. The activity of leukocyte beta-glucocerebrosidase was only slightly elevated (7.3 nmol/mg protein/1 h) which ruled out the diagnosis of classic Gaucher's disease. The patient received two courses of CHOP without improvement and anti-CD20 monoclonal antibody (rituximab) with only temporary stabilisation. Subsequently, he underwent splenectomy because of prolonged severe pancytopenia and a suspicion of hypersplenism. After splenectomy significant haematological improvement was observed. Following anti-CD20 therapy, changes in immunoprofile and morphology of tumour cells were evident. Before treatment the population of LPL was more divergent, with expression of LCA, CD20, CD38 and CD138. However, after the treatment, there were more mature plasma cells which no longer expressed CD20 antigen-this picture was more consistent with the diagnosis of plasma cell myeloma. Similarly, in the spleen there were no CD-20-positive cells evident. Finally, the patient received two courses of VAD vincristine, doxorubicin, dexamethasone) with further haematological improvement but complete response was not achieved.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/29. A new serum lipoprotein associated erythrocyte antigen which reacts with a monoclonal IgM. The stored human red blood cell SHRBC antigen.A monoclonal IgM (IgM) was found to react with stored human red blood cells (SHRBC) and human low density lipoprotein (LDL). IgM Re Fab fragment was reactive. SHRBC antigen was present, in a hidden state, in all fresh human ABO erythrocytes studies; it was not present in fresh or stored animal RBC. Cross reactivity of SHRBC and LDL antigens against IgM Re and IgM Re Fab was demonstrated. In contrast with SHRBC antigen, LDL antigen has no species specificity and was also found in rabbit and rat LDL. Furthermore, cross-reacting soluble inhibitors were also found in human saliva, and in a small molecule fraction issued from human and rabbit serum. The IgM Re also reacted with human and animal glomerular membrane. These findings are suggestive of two closely related but not identical antigens: a human specific SHRBC antigen and a LDL-associated, SHRBC-like antigen, which is a secreted, non-human specific substance. The former is part of the human RBC structure, the latter is a soluble molecule which may be found in saliva and in serum where it may be free or bound to LDL; it also binds to glomerular membranes.- - - - - - - - - - ranking = 15keywords = antigen (Clic here for more details about this article) |
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