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1/56. 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.
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ranking = 1
keywords = peripheral
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2/56. Neurolymphomatosis in Waldenstrom's macroglobulinaemia.

    We report four patients with Waldenstrom's macroglobulinaemia with an unusual neurologic complication, neurolymphomatosis, characterized by meningeal and root nerve infiltration by lymphoplasmacytic cells. patients presented with rapidly progressive leg proximal weakness. Examination of cerebrospinal fluid disclosed lymphoplasmacytic cells. magnetic resonance imaging of the lumbar spine was suggestive of a tissular infiltration of leptomeninges and nerve roots. Chemotherapy and irradiation of involved tissues led to a remarkable and sustained neurological improvement.
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ranking = 0.018080296832896
keywords = nerve
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3/56. 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.
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ranking = 4
keywords = peripheral
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4/56. 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.
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5/56. Rearrangement of immunoglobulin gene in metastatic waldenstrom macroglobulinemia to the vitreous.

    PURPOSE: To report metastatic waldenstrom macroglobulinemia cells with immunoglobulin heavy chain gene rearrangement in the vitreous and the blood. methods: A 58-year-old man with waldenstrom macroglobulinemia developed bilateral vitreitis. Diagnostic vitrectomy was performed on the left eye. The vitreous cells and the peripheral blood lymphocytes were analyzed using microdissection and polymerase chain reaction amplification. RESULTS: vitrectomy specimen of the left eye contained a few degenerated cells. Molecular analysis showed immunoglobulin heavy chain gene rearrangement at the third complementary determining region of the vitreal infiltrating cells and peripheral blood lymphocytes. CONCLUSIONS: waldenstrom macroglobulinemia rarely metastasizes to the vitreous. Molecular detection of the immunoglobulin heavy chain gene third complementary determining region rearrangement is helpful in the diagnosis of the malignancy. microdissection combined with polymerase chain reaction is a useful and innovative tool for molecular pathological investigation.
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keywords = peripheral
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6/56. The polyneuropathy associated with Waldenstrom's macroglobulinaemia can be treated effectively with chemotherapy and the anti-CD20 monoclonal antibody rituximab.

    A 50-year-old male developed headache, impaired balance, visual defects and severe deafness. Ten months later he presented with markedly reduced power and tremor of his right arm. Waldenstrom's macroglobulinaemia (WM) with accompanying polyneuropathy was diagnosed. The patient received chemotherapy, which resulted in a partial improvement of the arm tremor. Subsequently, he was treated with rituximab (4 x 375 mg/m2), leading to complete resolution of the tremor and the paresis of his arm. Additionally, his headache and imbalance disappeared. Fifteen months after rituximab therapy he remained free of any neurological symptoms. This is the first report showing that WM-associated polyneuropathy can be treated effectively with a combination of chemotherapy and the anti-CD20 monoclonal antibody rituximab.
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ranking = 1.1787874360847
keywords = neuropathy
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7/56. The tyranny of the anecdote: Waldenstrom's macroglobulinemia and scleritis.

    A 49-year-old male developed a bilateral scleritis that eventually involved the optic nerve and was associated with cotton wool spots. Investigation of a markedly elevated erythrocyte sedimentation rate revealed a diagnosis of Waldenstrom's macroglobulinemia. Although it was plausible to believe that the two rare diseases were causally related, the scleritis resolved while the gammopathy progressed.
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ranking = 0.0090401484164478
keywords = nerve
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8/56. 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.
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ranking = 1
keywords = peripheral
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9/56. Autoimmune thrombocytopenia in Waldenstrom's macroglobulinemia.

    Autoimmune phenomena are well-recognised complications of Waldenstrom's macroglobulinemia (WM) and IgM monoclonal gammopathy. Peripheral neuropathy and cold agglutinin hemolytic anemia are the most common reported and occur in 5-10% of patients. Autoimmune thrombocytopenia has been rarely reported in WM and its incidence is not known. In this study we report the case of a 67-year-old man who presented with autoimmune thrombocytopenia who was subsequently found to have WM. Laboratory investigation demonstrated that platelet-associated IgM (PAIgM) but not PAIgG was clearly elevated compared to normal controls. In addition the patient's serum reacted strongly with a panel of donor platelets analysed with an indirect platelet immunofluorescence assay utilising an anti-IgM secondary antibody. Glycoprotein specificity could not however be demonstrated by ELISA techniques for platelet glycoproteins IIbIIIa, IaIIa, IbIXa, and IV. We also reviewed the case records of 104 additional cases of WM diagnosed at our institution between 5/93 and 5/99. Three further cases with clinically significant autoimmune thrombocytopenia were identified. The overall incidence of autoimmune thrombocytopenia (4/105, 3.8%) in this cohort of patients was similar to the incidence of peripheral neuropathy (7/105, 6.7%) and cold agglutinins (3/105, 2.9%).
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ranking = 3.5855040439448
keywords = peripheral neuropathy, peripheral, neuropathy
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10/56. 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.
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ranking = 6.778078942528
keywords = peripheral neuropathy, peripheral, neuropathy
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