11/97. Unusual immunogammopathy maculopathy.PURPOSE: To describe an unusual maculopathy in patients with serum immunogammopathies. DESIGN: Retrospective observational small case series. PARTICIPANTS: Three patients derived from the clinical retina practices of the authors were noted to have unusual maculopathy. methods: Each patient underwent fluorescein angiography and serum laboratory evaluation. MAIN OUTCOME MEASURE: Findings on fluorescein angiography. RESULTS: An unusual and atypical macular detachment with or without subretinal precipitates or fundus signs of serum hyperviscosity, such as retinal hemorrhages and dilated retinal veins, may be observed in patients with immunogammopathies such as multiple myeloma, Waldenstrom's macroglobulinemia, and benign polyclonal gammopathy. fluorescein angiography shows macular hypofluorescence with no evidence of retinal vascular or retinal pigment epithelial leakage within the macular elevation. CONCLUSIONS: patients with atypical unilateral or bilateral macular detachment may be afflicted with a serum immunogammopathy such as multiple myeloma or Waldenstrom's macroglobulinemia. Diagnostic serum protein electrophoresis and hematology consultation should be considered.- - - - - - - - - - ranking = 1keywords = viscosity (Clic here for more details about this article) |
12/97. 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 = 6keywords = viscosity (Clic here for more details about this article) |
13/97. Hepatocellular carcinoma followed by Waldenstrom's macroglobulinemia: a case report.A case of hepatocellular carcinoma associated with Waldenstrom's macroglobulinemia is reported. During treatment and follow-up of hepatocellular carcinoma, a 54-year-old man was found to have a progressive monoclonal increase in serum IgM level and subsequently was diagnosed as having Waldenstrom's macroglobulinemia. Chemotherapy was carried out to prevent hyperviscosity syndrome due to Waldenstrom's macroglobulinemia without any surgical management for hepatocellular carcinoma. The association of these diseases is quite rare; only 6 cases have been reported in the literature, and this is the first case in which hepatocellular carcinoma preceded Waldenstrom's macroglobulinemia.- - - - - - - - - - ranking = 1keywords = viscosity (Clic here for more details about this article) |
14/97. clonal evolution in waldenstrom macroglobulinemia highlights functional role of B-cell receptor.The course of clonal evolution of 2 related clones in the blood of a patient with waldenstrom macroglobulinemia (WM) indicates the functional importance for the expression of the B-cell receptor for the survival of these malignant cells. Protein and nucleotide sequencing of the paraproteins' variable regions revealed 2 predominant Vlambda and 2 VH sequences, each set comprised in the ratio 1:1.5. The 2 VH sequences and 2 Vlambda sequences shared the same VDJ and VJ junctional sequences, respectively, indicating that 2 malignant clones had evolved from a common ancestor. This is the first report on intraclonal heterogeneity in WM. Comparison of the Vlambda and VH sequences with the closest matching known germline genes showed that they contained approximately 10 somatic mutations each. The distribution and type of mutations demonstrate that mutations have continued to accumulate in the malignant clones and that selection has been operating to preserve immunoglobulin structure.- - - - - - - - - - ranking = 3.1166635046726E-5keywords = blood (Clic here for more details about this article) |
15/97. waldenstrom macroglobulinemia with karyotypic aberrations involving both homologous 6q.An 84-year-old female presenting with proptosis and hyperviscosity syndrome was found to have waldenstrom macroglobulinemia. Karyotypic analysis showed structural chromosomal abnormalities involving both homologous chromosomes 6 with a deleted 6q at q21-q23 and a complex three-break rearrangement in the t(6;13;21)?(q21;q14;q11). A literature review suggests that deletions of chromosome 6 at 6q21 are associated with lymphoplasmacytoid differentiation and IgM production in B-cell chronic lymphoproliferative disorders.- - - - - - - - - - ranking = 1keywords = viscosity (Clic here for more details about this article) |
16/97. Karyotypic abnormalities and clinical aspects of patients with multiple myeloma and related paraproteinemic disorders.Karyotypic abnormalities were detected in the malignant cells of 6 of 18 patients with multiple myeloma (MM). Six patients with benign monoclonal gammopathy, one with amyloidosis of immunoglobulin origin, and two with Waldenstrom's macroglobulinemia had normal karyotypes. All six MM patients with aneuploidy were in a group of 10 patients in an accelerated or relapse phase of their disease and four had high serum paraprotein levels (7.92, 6.24, 6.80, and 4.24 g/dl, respectively) when their abnormal karyotypes were detected. Five of the 6 MM patients with aneuploidy had received prior chemotherapy. aneuploidy was not observed in 8 stable MM patients. Abnormalities of chromosome 14 were present in all 6 patients, with a 14q marker in 5 and loss of No. 14 in 1. A translocation between Nos. 11 and 14 was found in aneuploid cells of 2 patients who had plasma cell leukemia (PCL). However, the break point in the long arm of No. 11 differed in the 2 patients. A gain of Nos. 5, 9, and 11 was seen in 3 patients, a gain of No 1 in 2, and rearrangements of No. 1 in 5 MM patients, including all 4 who had a 14q marker chromosome initially. A deletion of chromosome 6 at band q25 was detected in 2 MM patients and a pericentric inversion of No. 6 (6p21 to 6q13) was seen in the patient with PCL. Three of 4 MM patients had a nonrandom loss of one chromosome 8. Two other MM patients, who were treated with melphalan and prednisone, developed acute nonlymphocytic leukemia (ANLL) 2 and 4 years after the diagnosis of MM. Marrow cells of one patient showed a 5q- chromosome and a constitutional translocation involving Nos. 13 and 14 during the preleukemic stage; during the leukemic phase, the karyotype evolved to 50 chromosomes including extra chromosomes 1, 6, 8, 10, and 21 and a missing 7, in addition to the originally detected 5q- and the 13/14 translocation. The peripheral blood from the other patient was hypodiploid, with a missing chromosome 7 and a translocation between 3q and 9p. These patterns of chromosome change resemble those of ANLL rather than MM and are similar to the changes seen in ANLL after treated malignant lymphoma.- - - - - - - - - - ranking = 3.1166635046726E-5keywords = blood (Clic here for more details about this article) |
17/97. Primary lung involvement in Waldenstrom's macroglobulinaemia: report of two cases and review of the literature.Pulmonary involvement in Waldenstrom's macroglobulinaemia (WM) occurs in 3-5% of cases, but lung involvement without bone marrow infiltration is extremely rare. We report 2 patients who presented with bilateral consolidations on chest X-ray and non-specific symptoms and were treated for a long period of time for pulmonary infections until the diagnosis was made by open lung biopsy. Both patients presented high monoclonal IgM in the serum and one also had blood lymphoplasmacytosis. Trephine bone biopsy and bone marrow smears were normal and there was no other site of involvement. Along with the presentation of our patients, we review the literature, discuss some of the possible underlying mechanisms and raise the attention of clinicians to this rare manifestation of the disease.- - - - - - - - - - ranking = 3.1166635046726E-5keywords = blood (Clic here for more details about this article) |
18/97. Waldenstrom's macroglobulinemia and sensorineural hearing loss.Waldenstrom's macroglobulinemia, (WM) first described in 1944, is an uncommon disease caused by the abnormal production of immunoglobulin m monoclonal macroglobulin. Presenting signs and symptoms most frequently include fatigue or generalized weakness; tendency to bleed from mucosal surfaces; characteristic retinal lesions, including dilated and tortuous retinal veins, retinal hemorrhages, and exudates; lymphadenopathy; hepatosplenomegaly; sensory motor peripheral neuropathy; worsening normochromic anemia; increased sedimentation rate; and extremely high serum viscosity. hearing loss is an unusual presenting symptom of WM; only 6 cases are reported in literature. The etiology of hearing loss is unclear; however, hyperviscosity and thrombus formations are the most likely causes. We present a patient diagnosed with WM whose initial presenting symptoms were hearing loss and progressive sensory peripheral neuropathy with subsequent development of lymphadenopathy and hyperviscosity syndrome. Treatment with Fludarabine lead to improvement of her hearing and neurological deficits, as well as resolution of her other hyperviscosity-related symptoms.- - - - - - - - - - ranking = 4keywords = viscosity (Clic here for more details about this article) |
19/97. Successful non-myeloablative allogeneic peripheral blood stem cell transplantation (PBSCT) for Waldenstrom'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 Waldenstrom'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 Waldenstrom's macroglobulinemia.- - - - - - - - - - ranking = 0.00015583317523363keywords = blood (Clic here for more details about this article) |
20/97. Waldenstrom's macroglobulinaemia presenting as reticulate purpura and bullae in a patient with hepatitis b virus infection.Cutaneous manifestations of Waldenstrom's macroglobulinaemia (WM) include purpura, ulcers, urticaria, leukocytoclastic vasculitis, and immunobullous dermatoses. No association has been reported previously of WM and hepatitis b virus (HBV) infection. A 40-year-old female HBV carrier was admitted to hospital because of generalized oedema, oliguria, haematuria, hypertension, fever and blood-tinged sputum. Cutaneous manifestations included generalized petechiae, palpable purpura mainly on the legs, multiple necrotic ulcerations and gangrenous changes on the toes, and necrotic, giant confluent reticulate purpura on the trunk surmounted by several tense bullae. Laboratory investigations revealed monoclonal gammopathy of IgM kappa type (6.7 g/L), membranoproliferative glomerulonephritis associated with HBV infection, Bence Jones proteinuria, and an increased number of abnormal plasmacytoid cells in the bone marrow. Pathologic examination demonstrated immune complex-mediated vasculitis with deposits of IgM in the walls of dermal vessels and secondary subepidermal bulla formation. HBV infection may have caused WM or modified the clinical course in this fatal case.- - - - - - - - - - ranking = 3.1166635046726E-5keywords = blood (Clic here for more details about this article) |
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