1/230. Increased whole blood viscosity on cooling in a patient with cold hemoagglutinin disease.The whole blood and plasma viscosities have been evaluated in a patient with cold hemoagglutinin disease at different temperatures and at different shear rates. At 37 and 42 degrees C, whole blood viscosity values, regardless of the shear rate applied, resulted to be correspondent to the hematocrit value (31%). The values observed were similar to those noted in a patient with chronic bleeding anemia and an approximately equivalent hematocrit (33%). The same was true for plasma viscosity. At 32 degrees C, whole blood viscosity, regardless of the shear rate, resulted to be higher than expected. The values observed were similar to those noted in a normal subject with a clearly higher hematocrit value (44%) and definitely higher than those noted in the chronic anemia patient. On the contrary, plasma viscosity remained unchanged. These studies indicate that in cold hemoagglutinin disease, red cell aggregation and piling are capable of increasing blood viscosity.- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
2/230. poems syndrome, steroid-dependent diabetes mellitus, erythema elevatum diutinum, and rheumatoid arthritis as extramedullary manifestations of plasma cell dyscrasia.poems syndrome is a rare synopsis of different multisystemic disorders (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy, and skin lesions) associated with plasma cell dyscrasia. We herein report the atypical case of a 44-year-old white man presenting with glomerulopathy, poems syndrome, and erythema elevatum diutinum with a few-year history of non-insulin-dependent diabetes mellitus (NIDDM) and seronegative rheumatoid arthritis (RA) as early manifestations of IgAlambda multiple myeloma. The prescription of 1 mg/kg/day prednisone improved the patient's features dramatically. skin lesions improved by the association of glucocorticoids and plasma exchange, recurred when plasmapheresis ceased, and remitted when plasma exchange was reintroduced. NIDDM requiring insulinotherapy recurred when corticoids were discontinued and remitted when prednisone was reintroduced. However, prednisone and plasmapheresis had no effect on polyneuropathy, M-paraprotein, and plasma cell dyscrasia in our patient, who developed indolent multiple myeloma a few years later. We thus concluded that poems syndrome, steroid-dependent diabetes mellitus, rheumatoid arthritis, RA, and skin vasculitis in our patient were triggered by plasma cell dyscrasia.- - - - - - - - - - ranking = 98.226400944725keywords = plasma cell, plasma (Clic here for more details about this article) |
3/230. intestinal obstruction and gastrointestinal bleeding due to systemic amyloidosis in a woman with occult plasma cell dyscrasia.A 60-year-old woman presented to our hospital with repeated vomiting. Upper gastrointestinal endoscopy revealed a 1 cm diameter ulcer with clean base on the roof of the gastric antrum. Histological examination of gastric biopsies revealed abundant amorphous eosinophilic deposits in the submucosa. congo red stain for amyloid was positive. A barium follow-through study revealed a mass in the jejunum causing incomplete obstruction. urine for bence jones protein was negative. serum protein electrophoresis did not reveal any abnormal band and serum immunoelectrophoresis did not detect any monoclonal immunoglobulin. bone marrow examination, however, revealed an increased proportion of plasma cells. Subsequent immunohistochemical staining demonstrated monoclonal lambda light chains in the marrow plasma cells, thereby confirming a plasma cell dyscrasia. amyloidosis involving the gastrointestinal tract can produce a wide variety of non-specific symptoms and signs. A high index of suspicion is necessary to arrive at an early diagnosis. Management consists of supportive therapy for the gastrointestinal tract as well as treatment of the underlying condition.- - - - - - - - - - ranking = 96.893067611392keywords = plasma cell, plasma (Clic here for more details about this article) |
4/230. Rapid onset monoclonal gammopathy in cutaneous lupus erythematosus: interference with complement c3 and C4 measurement.Accurate nephelometric immunoassay requires both the analyte and the calibration standard to have the same molecular mass. Alteration in the size of the analyte can affect light scatter and yield erroneous results. We report a case where an autoantibody, a monoclonal IgM with immunoconglutinin activity, interfered with the nephelometric quantitation of plasma complement components C3 and C4.- - - - - - - - - - ranking = 0.33333333333333keywords = plasma (Clic here for more details about this article) |
5/230. 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.- - - - - - - - - - ranking = 138.41866801627keywords = plasma cell, plasma (Clic here for more details about this article) |
6/230. Schnitzler's syndrome: report of a case with progression to Waldenstrom's macroglobulinaemia.Schnitzler's syndrome is a rare but distinct entity in which chronic urticaria is a prominent feature. Our patient presented with an urticarial rash associated with intermittent fevers, lethargy, a migratory polyarthralgia and loss of weight. skin biopsy revealed a neutrophilic urticaria. An IgM kappa paraprotein was detected on serum protein electrophoresis at a level of 8 g/L (0.6-2.5 g/L). Bone marrow biopsy was normal. Marked improvement of the arthralgia, fever and lethargy was obtained with oral prednisolone. The urticarial rash, however, was only partly responsive. For the next 10 years the patient remained stable but corticosteroid dependent. Over a period of 10 years the IgM paraprotein had slowly risen to 47 g/L. Repeat bone aspirate and trephine revealed a diagnosis of a low grade lymphoplasmacytic lymphoma consistent with Waldenstrom's macroglobulinaemia. To date, despite six cycles of chemotherapy with oral chlorambucil, his disease remains persistent but stable.- - - - - - - - - - ranking = 0.33333333333333keywords = plasma (Clic here for more details about this article) |
7/230. Primary lymphoplasmacytoid lymphoma of the trachea with immunoglobulin g paraprotein.A primary tracheal lymphoma with immunoglobulin g (IgG)-associated monoclonal serum paraprotein treated with surgery and chemotherapy is reported. As far as we know this is the first lymphoplasmacytoid lymphoma reported in the tracheobronchial tree and the first with a serum and tissue IgG monoclonal paraprotein. Differential diagnosis must be made essentially with extramedullary plasmacytoma and mucosa-associated lymphoid tissue lymphoma. CD-45RB strong positivity and the absence of lymphoepithelial lesions may help to differentiate lymphoplasmacytoid lymphoma from them. We expand the spectrum of lymphoid lesions with plasmacytoid features that can occur in the tracheobronchial tract.- - - - - - - - - - ranking = 6.3575724327766keywords = plasmacytoma, plasma (Clic here for more details about this article) |
8/230. Crystal-storing histiocytosis: a disorder occurring in plasmacytic tumors expressing immunoglobulin kappa light chain.Intracellular immunoglobulin crystal formation within plasma cells is an uncommon finding in multiple myeloma and other lymphoplasmacytic tumors. We present 12 cases of plasmacytic tumors with prominent crystal formation, including myeloma (5 cases), lymphoplasmacytic lymphoma (6 cases), and a nonneoplastic plasma cell proliferation. In all cases, crystal formation was associated with the proliferation of variable numbers of histiocytes containing similar inclusions. These cases showed a variety of appearances, sometimes obscuring the underlying plasma cell tumor and raising the differential diagnosis of a storage disorder, hemophagocytosis, or a mesenchymal lesion. In cases of lymphoplasmacytic lymphoma, patients typically presented with marked paraproteinemia and symptoms of hyperviscosity. Crystal-storing histiocytosis was not associated with other immunoglobulin deposition disorders, including amyloidosis, Mott cell tumors, or kappa-light chain deposition. In our cases and those previously reported, we found an overwhelming association of crystal-storing histiocytosis (CSH) with tumors expressing immunoglobulin kappa light chain with no consistent association with a particular heavy chain. These results suggest that CSH results from the ingestion of crystals produced by plasma cell tumors that either overproduce kappa light chain or express a structurally aberrant molecule. CSH persists in the marrow and other sites throughout the course of the disease and in our series was not highly associated with development of the adult fanconi syndrome or rapid clinical deterioration.- - - - - - - - - - ranking = 58.034133873176keywords = plasma cell, plasma (Clic here for more details about this article) |
9/230. Monoclonal cryoglobulinemia with extensive gangrene of all four extremities--a case report.The monoclonal immunoglobulin products of plasma cell neoplasm can give rise to a variety of manifestations including hyperviscosity, amyloidosis, cryoglobulinemia, neuropathy, and renal failure. A 50-year-old woman with monoclonal cryoglobulinemia developed extensive gangrene and ulceration of both hands and feet over several weeks requiring bilateral below-elbow and below-knee amputations. This case illustrates the importance of qualitative properties of paraprotein.- - - - - - - - - - ranking = 13.841866801627keywords = plasma cell, plasma (Clic here for more details about this article) |
10/230. Biclonal gammopathy in multiple myeloma: a case report.Monoclonal gammopathy is a group of B-cell disorders which result in the production of a specific and unique monoclonal immunoglobulin (M-component). Biclonal gammopathy is characterized by the simultaneous appearance of two different M-components. The incidence is about 1% of all monoclonal gammopathy. This paper reports on a 48-year-old male who had a chief complaint of back-pain beginning 7 months earlier. A physical examination was unremarkable, except for anemia and tenderness in the back. Hemoglobin was 5.4 g/dl, white blood cells 4.5 x 10(3)/microliter, platelets 157 x 10(3)/microliter, and reticulocytes 0.9%. serum iron was 79 mg/dl, and total iron-binding capacity was 210 mg/dl. A blood smear showed the formation of rouleaux, but no plasma cells were found. serum creatinine was 5.4 mg/dl, with a creatinine clearance of 18.1 ml/min. serum electrolytes were normal except for serum calcium which was 14.4 mg/dl. The urinalysis showed strongly positive proteinuria ( 2), with negative bence jones protein. serum protein electrophoresis showed an increase and a spike pattern of beta-2 globulin (2.8 g/dl) and alpha-2 globulin (1.5 g/dl), with normal gamma globulin. By nephelometry technique, serum IgG was normal (1388 mg/dl), IgA was high (900 mg/dl), and IgM was also high (517 mg/dl). Advanced and extensive osteolytic lesions were found in the clavicle, ribs, skull, humerus, femur, and columna vertebralis. plasma cells (myeloma cells) in bone marrow were 32%. The clinical diagnosis was multiple myeloma (biclonal gammopathy) stage IIIB (Durie and salmon staging system). Clinical response was good after two series of conventional chemotherapy, with normal serum electrophoresis, decreasing serum creatinine and serum calcium. Based on the above data, the diagnosis of multiple myeloma with biclonal gammopathy was confirmed. This is a rare case with a combination of IgA and IgM M-components.- - - - - - - - - - ranking = 13.841866801627keywords = plasma cell, plasma (Clic here for more details about this article) |
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