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At least 109 records · Page 6Linked to original sources
PubMed1997

A novel monoclonal antibody which reacts with a high molecular weight neuronal cytoplasmic protein and myelin basic protein (MBP) in a patient with macroglobulinemia.

We report on the case of a 70-year-old man with primary macroglobulinemia who showed cranial polyneuropathy and extensive radiculoneuropathy. His serum contained an IgM lambda monoclonal antibody which reacted with both a high molecular weight protein in grey matter and purified myelin basic protein (MBP) on immunoblotting. In an immunohistochemical study, strong immunoreactivity was detected only in the cytoplasm of neurons and weak immunoreactivity was detected in myelin. These findings suggest that this antibody may be related to the pathogenesis of neuronal damage in patients with macroglobulinemia.

Aged↗
PubMed2002

Trisomy 4 as the sole cytogenetic abnormality in a Waldenström macroglobulinemia.

We report a case of Waldenström macroglobulinemia with trisomy 4 as the sole cytogenetic abnormality. Trisomy 4 has been reported previously in Waldenström macroglobulinemia, but only in conjunction with multiple chromosomal aberrations. Trisomy 4 has been reported in other hematologic malignancies including acute myeloid and lymphoid leukemias.

Aged↗
PubMed2003

Waldenström macroglobulinemia with a novel der(8;17)(q10;q10).

We report the occurrence of an unbalanced whole-arm translocation of der(8;17)(q10;q10) in an 80-year-old female patient with Waldenström macroglobulinemia. To our knowledge, der(8;17)(q10;q10) has not been described in Waldenström macroglobulinemia. Although this cytogenetic abnormality has been reported in a number of solid tumors, a literature review suggests also a possible association with B-cell chronic lymphoproliferative disorders.

Aged↗
PubMed1995

Concomitant partial tetrasomy 3q and trisomy 18 in Waldenström macroglobulinemia.

We report a 54-year-old female patient with Waldenström macroglobulinemia who shows concomitant partial tetrasomy 3q in the form of an additional isochromosome 3q, and trisomy 18. To our knowledge, isochromosome 3q has not been reported in chronic lymphoproliferative disorders. A review of the literature on karyotypic aberrations in Waldenström macroglobulinemia suggests that additional copies of the long arm of chromosome 3 and chromosome 18 may play a possible role in the pathogenesis of a subset of this disorder.

Aneuploidy↗
PubMed2001

An IgM anti-MBP Ab in a case of Waldenstrom's macroglobulinemia with polyneuropathy expressing an idiotype reactive with an MBP epitope immunodominant in MS and EAE.

In a previously described case of Waldenstrom's Macroglobulinemia, complicated by polyneuropathy, the IgM/lambda monoclonal antibody (mAb) was highly reactive with myelin basic protein (MBP). Given our demonstration that V lambda x, a recently described murine lambda variable region gene product, can itself bind MBP as well as confer MBP reactivity to an Ab, the possibility of a shared idiotypy between murine V lambda x and this human IgM/lambda anti-MBP was investigated. We characterized the epitope specificity of the macroglobulinemia patient's MBP-reactive IgM/lambda using indirect ELISA procedures with MBP, a citrullinated isomer of MBP termed C8, or peptide fragments of MBP as the coating antigens and monospecific Ab to V lambda x as the secondary Ab. The patient's MBP-reactive IgM/lambda was recognized by Ab specific for V lambda x and, like murine mAb containing V lambda x bound human MBP but not MBP-C8 nor other common autoantigens such as DNA, thyroglobulin, or actin. The anti-MBP reactivity was selective for MBP peptide 90-170 and preferentially recognized MBP peptide 84-96. Thus, the patient's macroglobulin and perhaps certain other human Ab with a 'V lambda x idiotype' bind to MBP peptide residues 84-96, an immunodominant peptide in multiple sclerosis patients. Such binding may be involved in the pathogenesis of neural damage in patients with neuroimmunologic disorders related to plasma cell dyscrasias or autoimmunity.

Animals↗
PubMed1987

Crossreacting idiotypes of kappa-monoclonal immunoglobulins M in sera of patients with Waldenström's macroglobulinemia.

Seventeen anti-idiotypic antisera (anti-Id) were prepared against kappa-monoclonal IgM from patients with Waldenström's macroglobulinemia. Their reactivity against homologous and heterologous IgM was tested using an ELISA. Crossreacting idiotypes were only found in two out of 289 investigated antigen-antibody reactions. One anti-Id IgG crossreacted with determinants on polyclonal IgM. These rare crossreactions were observed in Waldenström's macroglobulinemia patients with and without polyneuropathy. The scarcity of common idiotypes on different monoclonal IgM does, however, not constitute a decisive argument against common or related antibody specificities of such monoclonal IgM.

Antibodies, Anti-Idiotypic↗
PubMed1978

Lymphosarcoma of the mandible associated with macroglobulinemia of Waldenström.

Waldenström macroglobulinemia is a rare progressive immunoproliferative disorder involving lymphocytes and occasionally plasma cells. Unlike the multiple myeloma, this disease has minimal osseous lesions. Lesions in the jaws are very rare. This paper presents a case of lymphosarcoma in the mandible in a 68-year-old woman, who was diagnosed as suffering from Waldenström macroglobulinemia. The possibility of immunosuppression by Leukeran as an aetiologic factor causing the bone lesions is suggested.

Aged↗
PubMed1997

B-cell lymphoma accompanying monoclonal macroglobulinemia with features suggesting marginal zone B-cell lymphoma.

Waldenstroem's macroglobulinemia is usually closely related to a histopathologic subtype called lymphoplasmacytoid lymphoma in the Revised European-American Lymphoma (REAL) classification. Here, we report a case of B-cell lymphoma accompanied by monoclonal macroglobulinemia and pathologically compatible with marginal zone B-cell lymphoma (MZBCL) rather than lymphoplasmacytoid lymphoma. The patient was a 74-year-old man with lymphomatous lesions that were recognized as submandibular lymphadenopathy, subcutaneous tumor and bilateral orbital masses, but with no bone marrow involvement. The atypical lymphoid cells that occupied the lymph node were varied such as small cells harboring irregular nuclei, monocytoid B-cells, large cells with vesicular nuclei, and plasma cells. The tumor cells expressed CD19, CD20, IgM and kappa on their cell surfaces and cytoplasmic IgM, but not CD5 or CD10. These findings suggest that the clinical and histopathologic findings of this case are compatible with those of MZBCL rather than lymphoplasmacytoid lymphoma. Serum IgM was elevated up to 4080 mg/dl and M-proteinemia of IgM-kappa type was confirmed by immunoelectrophoresis. Hyperviscosity syndrome caused by monoclonal IgM was not apparent. Immunohistochemical study confirmed that monoclonal IgM-kappa was produced and secreted from the tumor cells of MZBCL. This case suggests a close relationship between MZBCL and lymphoplasmacytoid lymphoma, from the perspective of the cellular origin during B-cell differentiation.

Aged↗
PubMed1999

Rituximab therapy in Waldenstrom's macroglobulinemia: preliminary evidence of clinical activity.

To assess the preliminary efficacy of rituximab therapy in Waldenstrom's macroglobulinemia (WM), we examined the clinical and laboratory data for all patients with WM treated on IDEC Pharmaceuticals sponsored trials and one patient treated at Walter Reed Army Medical Center. Seven symptomatic patients with WM were treated with four (n = 6) or eight (n = 1) weekly infusions of rituximab (375 mg/m2). Patients had received a median of three prior therapies (range 1-4) which included alkylator therapy in all (five patients refractory) and fludarabine in four (all refractory). Therapy was tolerated well in all patients without decrement in cellular immune function or significant infectious morbidity. Partial responses were noted in three of these patients, including two with fludarabine-refractory disease. The median progression-free survival for these patients was 6.6 months (range 2.2-29+ months). These data suggest that rituximab has clinical activity in heavily pre-treated patients with Waldenstrom's macroglobulinemia. Based on these data, clinical studies of Rituximab in previously untreated and treated WM appear indicated.

Aged↗
PubMed2004

t(1;14) and t(11;18) in the differential diagnosis of Waldenström's macroglobulinemia.

Waldenström's macroglobulinemia is caused by several B-cell proliferative disorders including lymphoplasmacytic lymphoma, marginal zone B-cell lymphoma, B-cell chronic lymphocytic leukemia and multiple myeloma. Differential diagnosis between lymphoplasmacytic lymphoma and extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue is particularly difficult as there is a considerable overlap in histological presentation. We report a case of Waldenström's macroglobulinemia with involvement of the peripheral blood, bone marrow and stomach. Serum chemistry revealed an IgM of 5.4 g/dl, but Bence-Jones protein in urine was negative. Abnormal lymphoid cells were detected in both blood and the bone marrow. Flow cytometry of the bone marrow aspirate showed that majority of cells were CD20(+), CD38(+), expressing immunoglobulin lambda light chain, but CD5(-) and CD10(-). Gastric biopsies revealed infiltration of the gastric mucosa by small lymphoid cells showing plasmacytoid differentiation and occasional Dutcher bodies. Lymphoepithelial lesions and Helicobacter pylori were not seen. Thus, the differential diagnosis between lymphoplasmacytic lymphoma and mucosa-associated lymphoid tissue lymphoma was raised. To resolve this, we performed BCL10 immunohistochemistry and reverse transcriptional polymerase chain reaction (RT-PCR) for the API2-MALT1 fusion transcript of t(11;18)(q21;q21). Both bone marrow and gastric biopsies showed strong BCL10 nuclear staining, similar to that seen in t(1;14)(p22;q32) positive mucosa-associated lymphoid tissue lymphoma, but absence of the API2-MALT1 fusion transcript. To further ascertain whether the detection of t(1;14)(p22;q32) and t(11;18)(q21;q21) can be reliably used for the differential diagnosis between lymphoplasmacytic lymphoma and mucosa-associated lymphoid tissue lymphoma, we screened for these translocations by BCL10 immunohistochemistry in 58 lymphoplasmacytic lymphomas and RT-PCR for t(11;18)(q21;q21) in 40 lymphoplasmacytic lymphomas, respectively. None of the lymphoplasmacytic lymphomas studied harbored these translocations. Thus, detection of t(1;14)(p22;q32) and t(11;18)(q21;q21) is useful in the differential diagnosis between lymphoplasmacytic lymphoma and mucosa-associated lymphoid tissue lymphoma.

ADP-ribosyl Cyclase↗
PubMed1999

Autologous peripheral blood stem cell transplantation for Waldenstrom's macroglobulinemia.

We report a 50-year-old male patient with Waldenstrom's macroglobulinemia poorly responsive to conventional chemotherapy, who went on to receive high-dose melphalan as conditioning before autologous PBSC infusion. Autologous hematopoiesis was reconstituted 5 weeks after transplant. Complete remission was confirmed by blood and marrow examination. The patient remains well at 12 months, with no detectable monoclonal IgM in his serum and absence of plasmacytoid lymphocytes in his marrow. Our results suggest that, at least in this case, high-dose chemotherapy with autologous PBSC support was an effective therapeutic approach for Waldenstrom's macroglobulinemia.

Hematopoietic Stem Cell Transplantation↗
PubMed2001

Successful non-myeloablative allogeneic peripheral blood stem cell transplantation (PBSCT) for Waldenström's macroglobulinemia with severe pancytopenia.

We report a 62-year-old male who underwent non-myeloablative allogeneic peripheral blood stem cell transplantation (PBSCT) because of his life-threatening severe pancytopenia due to refractory Waldenström's macroglobulinemia. This therapy was performed safely and he made a marked recovery from his cytopenia that had not been improved with any other therapy. Bone marrow aspirates showed post-transplant mixed chimerism during engraftment, and became completely donor-derived after a series of GVHD symptoms, without subsequent donor lymphocyte infusion. Our results suggest that non-myeloablative allogeneic PBSCT could be a good alternative for patients suffering from multi-drug resistant Waldenström's macroglobulinemia.

Critical Illness↗
PubMed1994

Waldenström's macroglobulinemia with an IgM paraprotein that is both a cold agglutinin and a cryoglobulin and has a suppressive effect on progenitor cell growth.

BACKGROUND: A patient with Waldenström's macroglobulinemia was admitted to the hospital with fever, leg pain, and dyspnea. The patient had gas gangrene of the left leg that required above-the-knee amputation. Plasmapheresis was instituted to treat hyperviscosity. STUDY DESIGN AND METHODS: The patient's serum contained an IgM-kappa paraprotein, a cryoglobulin, and a cold agglutinin. The serum was studied. RESULTS: The patient's red cells typed as A1, Rh-positive. The direct antiglobulin test was negative. The serum contained a cold agglutinin with anti-Pr cold agglutinin specificity (titer 4096). Maximal thermal range was 30 degrees C. Following dithiothreitol treatment, the cold agglutinin activity disappeared. The serum IgM concentration in the tested sample was 62.3 g per L. The cold agglutinin titer in the supernatant after removal of the cryoglobulin was 256, and the IgM level was 0.31 g per L. Redissolving the cryoglobulin in a equivalent volume of saline resulted in a cold agglutinin titer of 4096 and an IgM level of 68.4 g per L. These results indicate that the cryoglobulin and the cold agglutinin are the same paraprotein. Serum protein electrophoresis using agarose gel and immunofixation of the serum revealed an IgM-kappa monoclonal band. Progenitor cell assays were performed by adding the patient's serum at final concentrations of 0, 1, 5 and 10 percent (vol/vol) to patient's and normal donor's peripheral blood mononuclear cells. Inhibition of burst-forming units-erythroid and colony-forming units-granulocyte/macrophage by the patient's serum was demonstrated. Appropriate controls and the use of the serum of another patient with Waldenström's macroglobulinemia did not suppress progenitor cell growth. The patient's serum inhibited colony formation in a dose-response fashion. CONCLUSION: Reports of cryoprecipitable cold agglutinins are rare. This case is unusual because the IgM-kappa paraprotein was also a cold agglutinin with anti-Pr specificity and erythroid and granulocyte-macrophage progenitor cell-suppressive properties.

Agglutinins↗
PubMed2003

Smouldering Waldenstrom's macroglobulinemia: factors predicting evolution to symptomatic disease.

Factors predicting evolution to symptomatic disease were investigated in 27 patients with smouldering Waldenstrom's macroglobulinemia (SWM) among 172 patients with Waldenstom's macroglobulinemia (WM), selected on the basis of the following criteria: (1) IgM paraprotein > 3 g/dL, and/or (2) bone marrow (BM) lymphoplasmacytoid (LPC) infiltration >or= 30%, and/or (3) diffuse infiltration pattern on BM biopsy, and (4) no treatment requirement for at least 12 months. Cumulative probability of survival was calculated by means of Kaplan-Meier. The Mantel and Haenszel test and multivariate Cox model were used to identify possible predictors for evolution. At a median follow-up of 79 months (range, 14 to 204), 11 patients (40.7%) showed progression to symptomatic disease, with the median interval from diagnosis being 46 months (range, 12 to 154). Event-free survival (EFS) at 5 and 10 years was 65% (95% confidence interval [CI], 45% to 85%) and 53% (95% CI, 31% to 75%), respectively. At multivariate analysis, paraprotein > 3 g/dL (hazard ratio [HR], 15.1; 95% CI, 3.01 to 75.64; P <.0009) and hemoglobin 3 g/dL and hemoglobin levels <or= 12.5 gr/dL are likely to predict SWM transformation into active disease requiring treatment.

Adult↗
PubMed2003

Treatment of Waldenstrom's macroglobulinemia with single-agent thalidomide or with the combination of clarithromycin, thalidomide and dexamethasone.

To evaluate the activity of thalidomide in Waldenstrom's macroglobulinemia (WM), 20 patients were treated on a dose schedule that escalated from 200 mg/d to 600 mg/d. On an intention-to-treat basis, five (25%) patients achieved a partial response, which was noted within 3 months of treatment. Adverse effects were common and prevented dose escalation of thalidomide in 75% of patients and led to premature discontinuation of treatment in 35%. We subsequently evaluated the oral combination of clarithromycin (500 mg twice per day), low-dose thalidomide (200 mg once daily), and dexamethasone (40 mg once per week). Our preliminary analysis on 12 previously treated patients indicate activity of this regimen in WM: three patients achieved a partial response and two patients demonstrated monoclonal protein reduction of greater than 25%. This combination was associated with a variety of side effects due not only to thalidomide, but also to corticosteroids and to clarithromycin. Our preliminary data indicate that this combination may be a useful salvage regimen for some patients with heavily pretreated macroglobulinemia.

Angiogenesis Inhibitors↗
PubMed2001

Cutaneous Waldenström's macroglobulinemia: report of a case and overview of the spectrum of cutaneous disease.

Specific infiltration of the skin is a rare complication of Waldenström's macroglobulinemia. Cutaneous disease may also develop as a direct result of paraproteinemia and paraprotein specific antiepidermal autoimmune disease. We report a patient with Waldenström's macroglobulinemia in whom cutaneous infiltration developed with a clonal population of lymphoplasmacytoid B cells, summarize previously reported cases, and review the spectrum of cutaneous disease associated with this hematologic malignancy.

Diagnosis, Differential↗
PubMed2002

Waldenström's macroglobulinemia: a review of therapy.

Waldenstrom's macroglobulinemia is a lymphoplasmacytic disorder characterized by a monoclonal IgM protein, anemia, hepatosplenomegaly, and hyperviscosity. With the increasing use of screening chemistry evaluations, many patients are diagnosed without symptoms and are candidates for observation with no therapeutic intervention until symptoms develop. Plasma exchange can be useful to manage hyperviscosity but does not address the infiltrative process in the bone marrow, which requires cytoreductive therapy. This review covers current regimens that have been used to manage Waldenstrom's macroglobulinemia, including alkylating agents, purine nucleoside analogs, and rituximab. The value of steroids, radiotherapy, stem cell transplantation, and splenectomy is also reviewed. The lack of phase III studies does not permit an algorithm that would be appropriate for all patients. Treatment needs to be individualized based on patient age, the clinical manifestations of Waldenström's, and the patient's potential for developing toxic side effects of the selected treatment regimen.

Adrenal Cortex Hormones↗