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The purpose of this study was to use multiparameter flow cytometry to detect occult marrow disease (OMD) in patients with solitary plasmacytoma of bone and assess its value in predicting outcome. Aberrant phenotype plasma cells were demonstrable in 34 of 50 (68%) patients and comprised a median of 0.52% of bone marrow leukocytes. With a median follow-up of 3.7 years, 28 of 50 patients have progressed with a median time to progression (TTP) of 18 months. Progression was documented in 72% of patients with OMD vs 12.5% without (median TTP, 26 months vs not reached; P = .003). Monoclonal urinary light chains (ULC) were similarly predictive of outcome because progression was documented in 91% vs 44% without (median TTP, 16 vs 82 months; P < .001). By using both parameters, it was possible to define patients with an excellent outcome (lacking both OMD and ULC, 7.7% progression) and high-risk patients (OMD and/or ULC, 75% progression; P = .001). Trials of systemic therapy are warranted in high-risk patients., (© 2014 by The American Society of Hematology.)

Background: Plasmacytoma of the bladder is an extremely rare tumor, with all information concerning this neoplasm derived from case reports. It can be a major diagnostic pitfall on both histology and urine cytology., Case: A 95-year-old woman presented with gross hematuria and a large bladder mass detected by ultrasound. The case was initially misdiagnosed as a high grade urothelial carcinoma. Since the urine cytology did not show the classical cytologic features of urothelial carcinoma, the histologic sections were reviewed and immunohistochemical staining performed. The final diagnosis was plasmacytoma of the bladder. Subsequently the patient underwent a skeletal survey and bone scan, which did not reveal any lesion suspicious for multiple myeloma. The patient was scheduled for radiotherapy., Conclusion: In this case of bladder plasmacytoma, urine cytology provided a clue to the diagnosis. Urine cytology can be a diagnostic tool to help make this diagnosis in the case of poorly differentiated bladder neoplasm, especially in a patient with a known history of multiple myeloma.

Exfoliative cytology specimens from patients with plasma cell dyscrasias are rarely diagnostic, though there are reports of neoplastic plasma cells observed in pleural fluid and urine. Immunoglobulin concretions or crystals associated with neoplastic plasma cell populations have been well described. These crystals may be found within plasma cell nuclei or cytoplasm, extracellularly, or within cells of histiocytic lineage where they are presumably derived by phagocytosis. To the best of our knowledge, this is the first report of immunoglobulin crystals within histiocytes in the urine of a patient with plasmacytoma. A 61-year-old male underwent routine cystoscopy and urine collection to evaluate hematuria. The bladder biopsy demonstrated IgA kappa-producing plasmacytoma. Urine cytopsin preparations did not contain plasma cells, but there were histiocytic cells with degenerative nuclei and eccentrically located cytoplasmic spherical refractile hyaline crystals thought to be immunoglobulin crystals. This was supported by immunocytochemical staining., (Copyright 2004 Wiley-Liss, Inc.)

Background: Plasmacytoma of the bladder is a rare but important entity. We report a case of plasmacytoma of the bladder that was diagnosed by urinary cytology., Case: A 71-year-old male with a history of multiple myeloma presented in renal failure. Renal ultrasound revealed right-sided, moderate hydronephrosis with a 4 x 4-cm, posterolateral, obstructing mass. Magnetic resonance imaging demonstrated a bladder mass involving the bladder base, right lateral wall and dome with extension into the perivesical tissues on the right. The mass showed a moderate degree of enhancement following intravenous gadolinium administration. Urine cytology was performed to evaluate for bladder carcinoma or other malignancies besides plasmacytoma. The specimen was signed out as multiple myeloma of the bladder. Cystoscopy and biopsy were subsequently performed on the bladder mass. The diagnosis of plasmocytoma was made, confirming the urine cytology diagnosis., Conclusion: Urinary cytology can be a diagnostic tool for plasmocytoma involving the bladder.

Angiogenesis is a crucial process in the progression of multiple myeloma (MM). Vascular endothelial growth factor (VEGF) and hepatocyte growth factor (HGF) are multifunctional cytokines that potently stimulate angiogenesis including tumour neovascularization. Serum levels of VEGF and HGF were measured in 52 patients with MM by enzyme-linked immunosorbent assay (ELISA). Serum levels of VEGF and HGF were elevated in MM patients compared with healthy controls (VEGF: mean 0.31 ng/ml and 0.08 ng/ml respectively, P < 0.01; HGF: mean 2.17 ng/ml and 0.45 ng/ml, respectively, P < 0.001). In serial samples taken after chemotherapy, serum VEGF and HGF levels were correlated with M-protein levels. Serum levels of VEGF were higher in patients with extramedullary plasmacytomas than in patients without them (P < 0.05). They were also significantly higher in a group of patients who showed poor response to chemotherapy (P < 0.01). Serum levels of HGF were higher in patients with complications such as anaemia, hypercalcaemia and amyloidosis than in patients without these complications (P < 0.01, P < 0.05, P < 0.05 respectively). Both serum VEGF and HGF levels were significant predictors of mortality (P = 0.01, P = 0.02, respectively, log-rank test). The present study demonstrated that serum levels of VEGF and HGF are significantly elevated and dependent on the severity of MM, suggesting that measurement of VEGF and HGF may be useful for assessing disease progression and for predicting the response to chemotherapy in MM patients.

Thalidomide is active in patients with refractory myeloma. Seventeen patients (nine men/eight women, median age 73 years) with multiple myeloma (MM) were treated with thalidomide. Fifteen patients had refractory disease and two untested relapse. The median dose of thalidomide was 500 mg (range, 200-800 mg). Nine of the 17 patients (53%) responded. The response rate was significantly higher in patients with no extramedullary disease than in those with soft tissue masses (75% CI: 43-95% versus 0%; P = 0.01)). Of note, no decrease in the size of soft tissue plasmacytomas was observed in all the five patients who had extramedullary involvement. This data suggests that the mechanism of action and effectiveness of thalidomide might depend on the site of the tumour cells.

We present a case of primary nodal plasmacytoma in an elderly Chinese woman that was associated with significant paraproteinaemia and paraproteinuria. Clinical and laboratory features of the patient satisfied Durie's criteria for the diagnosis of multiple myeloma. The present case was unusual in two aspects. Firstly, there was no evidence of clonal plasma cell proliferation elsewhere in the body after extensive radiological investigations, repeated bone marrow examinations, and polymerase chain reaction for immunoglobulin gene rearrangement study. Secondly, the clinical behaviour was indolent despite the large amount of paraprotein production, and showed satisfactory disease control with local radiotherapy. The differential diagnoses of plasmacytosis in the lymph node are also discussed.

Double (kappa + lambda), Bence Jones (BJ) proteinuria was detected in a male patient of 73 years. The disease started as a solitary plasmacytoma in bone and transformed into multiple myeloma. At the beginning, isolated BJ lambda proteinuria was observed which was soon accompanied by transient excretion of BJ kappa protein.

It is compared an electrophoretic study between unconcentrated and concentrated urine samples on cellulose acetate and immunoelectrophoresis. The study shows that the findings carried out on cellulose acetate are overlapped with the immunochemical methods therefore the cellulose acetate may be preferred an useful medium on the electrophoretic assay of the unconcentrated urinary proteins without concentration.

We report two unusual manifestations of multiple myeloma in a 71-year-old man. Late in the course of his disease, myeloma cells were observed in the patient's urinary sediment. At post mortem, the source of the cells was found to be a plasmacytoma of the urinary bladder. To our knowledge, this appears to be the first report of a clearly demonstrable anatomic source for myeloma cells in the urine.

Large-amplitude circadian rhythms were observed in the urinary excretion of polyamines by rats bearing an immunocytoma. Control animals excreted polyamines at a lower rate but also with marked circadian variation. In confirmation of earlier observations, light-chain excretion by the tumor-bearing rats also exhibited a circadian rhythm, superimposed on an increasing trend. The potential of these rhythms as markers for the chronotherapy of cancer is noted.

A report is given on frequency investigations in the plasmocytoma in a dissection material of 36,985 cases of the years 1951 to 1971. 107 observations of multiple, nearly exclusively osseous plasmocytomas are evaluated concerning frequency of age and sex, affection of the skeleton as well as correlations between the degree of the histological maturity of the tumour cells and the produced para-proteins as well as the existence of the Bence-Jones-protein. The findings were compared with those of the literature.

Sixty-six consecutive patients exhibiting isolated urinary excretion of monoclonal free light chains, i.e. Bence Jones protein (BJP), on screening investigation for serum and urine monoclonal immunoglobulins were studied in order to better define the spectrum of immunoproliferative disorders associated with such a protein abnormality. The typical plasma cell neoplasms accounted for only one third of the cases, multiple myeloma (MM) and systemic amyloidosis (AL) being diagnosed in 18% and 15% of the patients, respectively. Eighteen (27%) of the patients were recognized as having malignant nonHodgkin's lymphomas (NHL), 21 (32%) had chronic lymphocytic leukemia (CLL), and 2 (3%) had hairy cell leukemia (HCL). Three patients (5%) without apparent evidence of any malignant immunoproliferative disease were classified as having a monoclonal gammopathy of undetermined significance (MGUS). The greatest urinary concentrations of BJP were found in plasmacytic neoplasms, the daily excretion of MM patients being significantly higher than that of AL patients. Considerably lower BJP outputs were recorded in the other diseases, the lowest ones being associated with MGUS. NHL patients had a daily excretion four times higher as compared with that of CLL patients. The distribution of NHL by histologic type was: follicular center cell lymphomas (FCCL) 39%, small lymphocytic lymphoma (SLL) 33%, immunoblastic lymphoma (IBL) 17%, and plasmacytoid lymphocytic lymphoma (PLL) 11%. The highest BJP levels were found in PLL, and the lowest ones in FCCL. In CLL patients the amount of urinary BJP correlated significantly with the tumor load, as estimated by the number of enlarged lymphoid areas. The study suggests that detection and measurement of isolated urinary BJP may provide useful data for the clinical evaluation of a wide spectrum of immunoproliferative disorders.

Urinary proteins from 50 patients with multiple myeloma (37 Ig G, 6 Ig A, 7 Bence Jones) were investigated by discelectrophoresis in polyacrylamidgels containing sodium dodecylsulfat. All samples were also characterized by immunelectrophoresis. Quantitatively and qualitatively normal proteinuria was found in 13 patients (26%). 22 patients (44%) had monoclonal free light chains in the urine, kappachains were eliminated mainly in the monomeric form, lambdachains in all samples in the dimeric form. In 2 patients were found to exist light chains as monomers and dimers. 11 other patients (22%) had peaks of monoclonal Ig G or Ig A in the urine, always associated with the elimination of other nonmonoclonal proteins. Non-specific proteinuria was found in the remaining 4 patients. The clinical validity of the findings is discussed.

A 33-year-old male presented with right side pleural plasmacytoma with secretion of Bence Jones (BJ) protein and deposition of amyloid. The pleural effusion contained a prominent peak of kappa-type BJ protein on the background of polyclonal gammopathy. Pleural biopsy disclosed features of plasmacytoma with amyloid deposition. A small peak of BJ protein was also present in serum electrophoretogram on the background of increased polyclonal IgG and IgA. Kidney function was impaired and urine protein ranged from 7.8 to 19.1 g/day with 92.5% bimodal BJ protein. There were no bone lesions by systemic bone survey, and repeated bone biopsies and marrow aspirations showed plasma cells of less than 5%. The clinical course worsened progressively; over the next five months the pleurisy became bilateral and peritoneal involvement supervened. This is believed to be the first report of primary plasmacytoma involving successively pleurae and peritoneum.

A case of plasma cell myeloma initially producing Bence Jones protein of lambda type was presented. BJ protein disappeared within two months of cyclophosphamide and betamethasone administration. A complete remission continued for the following 2 years and 8 months with no medication, although the patient insidiously developed osteolytic lesions. The discrepancy of long-term negative BJP and clinical remission without medication and progressive bone destruction was reported.