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1/401. 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. ( info)

2/401. 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. ( info)

3/401. Lymphoid lesions of the conjunctiva: relation of histopathology to clinical outcome.

    A retrospective clinicopathologic study of 40 patients with lymphoid lesions of the conjunctiva demonstrated the validity of current histologic criteria in predicting clinical outcome. overall histologic architecture as well as cytologic detail must be used to differentiate benign reactive lymphoid hyperplasia from lymphoma. Lesions verified clinically as being malignant had obvious malignant cytologic features. Clinical signs of surface follicularity, multifocality, and minimal elevation suggest benignancy. All the benign lesions, on histopathologic examination, were either follicular in architecture or composed of mature lymphocytes, and were generally restricted to the substantia propria. Bilaterality and clinical recurrence do not necessarily imply a malignant disease. ( info)

4/401. 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. ( info)

5/401. Cutaneous waldenstrom macroglobulinemia in transformation.

    waldenstrom macroglobulinemia is a low-grade B-cell lymphoproliferative disorder of the elderly with characteristic monoclonal IgM-producing neoplastic infiltrates of the bone marrow, lymph node, and spleen. Cutaneous manifestations are usually nonspecific such as purpura, ulcers, and urticarial lesions. These lesions are caused by hyperviscosity of the blood, immune complex-mediated vascular damage, paraprotein deposition, and amyloid deposition. Specific skin lesions occur rarely and generally consist of translucent, flesh-colored papules composed of monoclonal IgM deposits. Rarely, there may be violaceous lesions composed of low-grade lymphoplasmacytic infiltrates characteristic of waldenstrom macroglobulinemia. Both cutaneous manifestations of the disease, as well as disease transformation to high-grade, large cell lymphoma are rare. We report two very unusual cases of waldenstrom macroglobulinemia with documented skin disease that demonstrated transformation to high-grade lymphoma. Both patients were elderly men with long-standing waldenstrom macroglobulinemia involving the bone marrow, who subsequently developed skin involvement by the disease. waldenstrom macroglobulinemia can rarely manifest as cutaneous disease, sometimes as a high-grade transformation of low-grade waldenstrom macroglobulinemia elsewhere. Distinction of cases of transformed waldenstrom macroglobulinemia from de novo cutaneous large cell lymphoma may be important, because the two entities are likely biologically different. ( info)

6/401. Allogeneic bone marrow transplantation for advanced Waldenstrom's macroglobulinemia.

    Waldenstrom's disease is a lymphoproliferative disorder that is typically treated with plasmapheresis and/or alkylating agents. In young patients, other lymphoproliferative disorders have been treated with allogeneic transplantation. Two patients with aggressive Waldenstrom's disease, who progressed in spite of multi-agent chemotherapy and autologous stem cell transplantation, in one case, underwent allogeneic transplantation from their HLA-identical donors. Both remain alive with event-free survivals of more than 3, and more than 9 years, respectively. Allogeneic transplantation should be considered for young patients with Waldenstrom's disease. ( info)

7/401. Increased risk of lymphoma in sicca syndrome.

    The risk of cancer was ascertained in 136 women with sicca syndrome followed at the National Institutes of health (NIH). Seven patients developed non-Hodgkin's lymphoma from 6 months to 13 years after their first admission to NIH. This was 43.8 times (P less than 0.01) the incidence expected from the rates of cancer prevailing among women of the same age range in the general population during this time. In addition, three cases of Waldenstrom's macroglobulinemia occurred in this study group. Eight patients developed cancers other than lymphoma, similar to the number expected based on the rates prevailing in the general population. patients with a history of parotid enlargement, splenomegaly, and lymphadenopahy had an increased risk of lymphoma. These clinical conditions did not appear to be early manifestations of undiagnosed lymphoma but rather seemed to identify a subgroup of patients with sicca syndrome with marked lymphoid reactivity, who had a particularly high risk of subsequently developing lymphoma. ( info)

8/401. Waldenstrom's macroglobulinaemia with Ig E M-component.

    A case of Waldenstrom's macroglobulinaemia with Ig E M-component was presented. A 76-year-old Japanese woman had a history of the operation for mammary carcinoma. Although neither lymphadenopathy nor hepatosplenomegaly was present, the immunohaematological findings typical for Waldenstrom's macroglobulinaemia were observed in this patient. Coexistence of the Ig M and Ig E M-components was recognized in the patient's serum protein. Positive reaction for FITC-conjugated anti-epsilon or rhodamine-conjugated anti-mu was noted in proliferating lymphoplasmacytic cells of the patient's bone marrow, but these reactions were not simultaneously observed in a single cell. The finding suggests that two separate populations of M-component-producing cells were present. ( info)

9/401. Waldenstrom's macroglobulinaemia in South African Negroes.

    Waldenstrom's or primary macroglobulinaemia has rarely been documented in Black Africans. Six South African Negro patients with Waldenstroms macroglobulinaemia were assessed and followed during a 10-year period from 1966 to 1976. The hyperviscosity syndrome was the presenting feature in four patients and one patient each presented with the nephrotic syndrome and a lacrimal mass respectively. On initial evaluation of the six patients, the serum IgM varied from 1.3 to 4.8 g% and in two patients cryoglobulinaemia was found. The patients received supportive treatment, chemotherapy, and when necessary, plasmaphoresis. One patient was lost to follow-up, one patient died 7 years after the onset of his illness, but the remaining four patients are alive 18 months to 5 years after presenting with their disease. Although the disease occurred in a younger age group (mean age 43 years) than in Caucasians it did not differ in clinical features or response to treatment from other parts of the world. ( info)

10/401. Multiple karyotypic aberrations in a polymorphous variant of waldenstrom macroglobulinemia.

    A 71-year-old woman presented with malaise, skin bruising, epistaxis, and gingival bleeding of recent and prompt onset. There was no adenopathy. The liver and spleen were not enlarged. Bone marrow aspirate showed a polymorphous infiltration with lymphocytes (22%), typical Marschalko plasma cells (16%), plasmacytoid lymphocytes (29%), lymphoblasts (8%), and immunoblasts (13%). The immunoblasts morphologically resembled lymphosarcoma cells with a frequent "clover-leaf" appearance. An IgM paraprotein concentration in serum was 38.5 g/L. The bone marrow histopathology confirmed the presence of heterogenous cell infiltration, with 30% of the population being comprised of lymphoblasts and immunoblasts. In order to differentiate a polymorphous variant of waldenstrom macroglobulinemia (WM) from the more common small cell lymphocytic lymphoma (SLL) in anaplastic metamorphosis, flow cytometric studies were performed on marrow specimens. A typically bright surface IgM (lambda) was demonstrated with a less bright CD38. Further immunophenotype was HLA-DR , CD19 , CD20 and CD10-, CD22-, T-Ag- and kappa light chain- expression. This corroborated the diagnosis of an extremely rare, polymorphous variant of WM. The marrow cytogenetics disclosed 50% (10/20) pathologic metaphases 48,X,dup(X)(p21p22),der(2), 5,del(6)(q11q21), 12,inv(16)(p13q22), del(17) (p12), and 50% normal metaphases. The patient was treated with a LOPP protocol. She failed to respond and died 5 months after the diagnosis with myocardial and renal insufficiency complicating a pronounced pancytopenia in the peripheral blood. ( info)
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