Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical, haematological and biochemical correlations of 224 consecutive unselected examples of monoclonal gammopathy have been studied. The paraprotein frequency detedted was IgG 62 percent, IgA 15.2 percent, IgM 10.3 percent, Bence Jones protein 8.9 percent and in 3.6 percent the paraproteins were not identified. In half the monoclonal gammopathy was associated with an immunocytoma (myeloma in 82 and lymphoma in 30). In three cases the associated clinical disease was amyloidosis. In 36 cases (16.1 percent) the associated clincial disease was a nonlymphoproliferative malignant tumour. Monoclonal gammopathy may be a significant marker of malignancy in such cases. In 73 cases (32.6 percent) the associated clinical conditions were unrelated to the gammopathy although only 55 of these cases were sufficiently investigated to warrant classification as examples of benign monoclonal gammopathy. There was a strong correlation between Bence Jones proteinuria and malignancy. Sixty-five patients demonstrated Bence Jones proteinuria and in 59 of these a malignancy was detected. The association was strong between hypercalcaemia and malignancy as this was present in all 27 of the cases who had hypercalcaemia. The relationship between Bence Jones proteinuria and hypercalcaemia was also strong and Bence Jones proteinuria was detected in 73 percent of the hypercalcaemic patients as opposed to 36.7 percent in the whole series. Hypercalcaemia and Bence Jones proteinuria, when found in a patient with monoclonal gammopathy have a grave clinical connotation.
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PMID:Immunocytoma, cancer and other associations of monoclonal gammopathy: a review of 224 cases. 105 67

Monoclonal gammopathy of type IgG-lambda (IgG concentration 27.8 g/l) was discovered by chance in a 66-year-old woman with aortic and mitral valve disease. The patient declined any further diagnostic procedures. Three months later she experienced severe pain in the lumbar spine and developed decompensated cardiac failure with pulmonary and ankle edema. The IgG concentration had risen to 50.5 g/l. Echocardiography showed a large pericardial effusion and 600 ml of bloodstained fluid containing numerous plasma cells was aspirated (total protein 81.8 g/l, gamma-globulin 38.9%). Iliac crest biopsy showed diffuse infiltration with polymorphic plasma cells, but the differential count in peripheral blood was unremarkable. Multiple myeloma of Stage IIa was diagnosed and she was given cytostatic therapy with 17.5 mg melphalan and 112 mg methylprednisolone daily by mouth (for 4 days at intervals of 6 weeks). Though at first the IgG concentration fell, it later rose again. The beta 2-microglobulin level was raised at 30 mg/l. After three cycles of chemotherapy the patient complained of severe pain in the hips and thighs. The blood film now showed numerous, predominantly immature plasma cells. A few days later, having been ill for four months in all, she died, showing all the signs and symptoms of plasma cell leukaemia.
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PMID:[IgG-lambda-type multiple myeloma with plasma-cell pericardial effusion and terminal plasma-cell leukemia]. 142 69

We reviewed 32 cases of necrobiotic xanthogranuloma and 16 cases from the world literature. Necrobiotic xanthogranuloma is a destructive dermal and subcutaneous xanthogranuloma that most frequently involves the face (periorbital region) and trunk. Monoclonal gammopathy is common (IgG-kappa in 23 cases and IgG-lambda in nine cases), and cryoglobulins have also been found. Low complement levels, anemia, and leukopenia are frequently present. Bone marrow examination shows plasma cell proliferation and, rarely, true myeloma. We describe mucosal, muscle, and systemic lesions, including xanthogranulomatous myocardial lesions. The clinical course is chronic and often progressive. Low-dose chlorambucil treatment is safe and effective, but individual patients have responded to treatment with corticosteroids, melphalan, local radiation, and plasma exchange.
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PMID:Necrobiotic xanthogranuloma. 173 94

A 60-year-old woman was referred to us because of tumors on the occipital and the bilateral submaxillary areas. Biopsy proved them to be well-differentiated lymphosarcoma. On admission, systemic lymphadenopathy was noted and there was 26% of plasmacytoid cells in the bone marrow of the sternum. Monoclonal gammopathy of IgM,K type was found; her disease was diagnosed as a macroglobulinemia (IgM: 8,460 mg/dl). VENP-therapy consisted of vincristine 1 mg/w, cyclophosphamide 50 mg/d procarbazine 50 mg/d and prednisolone 30 mg/d was applied for about four weeks, but in vain. Transaminase levels were elevated (GOT 575 U, GPT 480 U) and the superficial lymphnodes did hardly diminish. Therefore, after improvement of the liver dysfunctions, 5 courses of AAAP-therapy, which was consisted of ACNU 50 mg/d (IV drip over 4 hrs), adriamycin 20 mg/d (IV push), methotrexate 25 mg/d (IV push) and prednisolone 60 mg/d (IV push) once a week or three were employed with excellent clinical effects. The superficial lymphnodes disappeared, M-protein and plasmacytoid cells in the bone marrow markedly decreased. An interval of the initial remission reached to 17 months. As previously reported, AAAP-therapy was also effective to multiple myeloma and acute lymphocytic leukemia of B-cell type. Therefore, AAAP-therapy would be one of the best chemotherapies for B-cell malignancy including macroglobulinemia.
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PMID:[A case report of macroglobulinemia responded to AAAP-therapy]. 682 Sep 7

High-resolution protein electrophoresis of serum, urine, and cerebrospinal fluid (CSF) can aid in the diagnosis of multiple myeloma, amyloidosis, macroglobulinemia, multiple sclerosis, and other diseases. Electrophoresis-Tutor is a personal computer program based on approximately 150 digital images that teaches the clinical interpretation of agarose gel electrophoretic patterns. The program is divided into the following sections: introduction, CSF, serum, urine, review of disease states, program navigator, and final exam. The CSF section describes normal and abnormal CSF findings with emphasis on oligoclonal banding, as seen in the CSF of patients with multiple sclerosis. The serum section emphasizes monoclonal gammopathy patterns but also has detailed descriptions of inflammation, liver disease, protein-losing disorders, genetic deficiencies, and other patterns. Monoclonal gammopathy is described in the context of specific associated clinical conditions (e.g., myeloma, amyloidosis). For each monoclonal gammopathy example, results of standard electrophoresis, densitometry, and immunofixation are presented. The review of disease states uses animation to illustrate the development and remission of a variety of pathological patterns. The program navigator allows the user to jump quickly to any place in the program. The optional exam contains 20 questions, and detailed feedback is given after each question. Electrophoresis-Tutor can be used as a stand-alone teaching tool, a companion to traditional instruction, or a reference source.
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PMID:Electrophoresis-tutor: an image-based personal computer program that teaches clinical interpretation of protein electrophoresis patterns of serum, urine, and cerebrospinal fluid. 765 48

We report six patients affected by POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes), a peculiar multiorgan disease frequently associated with osteosclerotic myeloma or other plasma cell disorders. Sensorimotor polyneuropathy was associated with multisystem involvement in all of the patients, with osteosclerotic myeloma in 2 cases, monoclonal gammopathy of undetermined significance in 2 cases and Castleman's disease in the final two. In all of the patients, sural nerve biopsy findings were consistent with a mixed, axonal and demyelinating neuropathy. Increased levels of Interleukin-6 were found in two cases, but the pathogenesis of the disease is far from established.
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PMID:The POEMS syndrome: report of six cases. 769 93

We review the concept fo Monoclonal Gammopathy emphasizing that the so called benign forms, with or without polyneuropathy can have an unpredictable evolution. Polyneuropathies associated to monoclonal gammopathy-IgM, IgG, IgA, Waldestrom's disease, Multiple myeloma, Osteosclerotic myeloma (POEMS), Amyloidosis and Cryoglobulinemias are reviewed. They generally are solely sensory, sensorimotor or rarely, solely motor. The importance of neuro-physiological studies and nerve biopsy is emphasized. For progressive forms, treatment with plasmapheresis, immunotherapy, corticoids or intravenous gammaglobulin is suggested.
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PMID:[Polyneuropathies and dysglobulinemias]. 780 41

Although studies of patients with rheumatoid arthritis have found no increases in overall cancer rates, significant elevations in rates of lymphoma and myeloma have been reported, suggesting a causal relationship between autoimmune disorders and lymphoid malignancies. Nevertheless, only 22 cases of concomitant rheumatoid arthritis and multiple myeloma were identified during a retrospective national multicenter study carried out in France. Neither disease exhibited unusual features. In every case, rheumatoid arthritis preceded multiple myeloma. Monoclonal gammopathy preceded multiple myeloma in two patients, by 3 and 7 years respectively. No patient had amyloid arthritis. Analysis of data from the cancer registry of the Somme district in northern France did not suggest a significantly increased risk of multiple myeloma in rheumatoid arthritis patients (relative risk 2.3). Use of interferon alpha to treat myeloma has been reported to exacerbate concomitant autoimmune disorders.
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PMID:[Rheumatoid arthritis and multiple myeloma. Apropos of 22 cases. Results of a multicenter national survey]. 816 23

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.
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PMID:Biclonal gammopathy in multiple myeloma: a case report. 1089 8

The revised guideline 'Monoclonal gammopathy (paraproteinaemia)' of the Dutch Institute for Health Care Improvement (CBO) describes the most recent clinically relevant developments in the field of monoclonal proteins (M-proteins). Criteria with both prognostic and therapeutic significance are established for 'monoclonal gammopathy of undetermined significance' (MGUS) and (smouldering) multiple myeloma. If an M-protein is found incidentally, the therapeutic consequences will be determined mainly by the probability that the M-protein fits in with the diagnosis or the clinical findings in the patient in question, as well as with the type and extent of the monoclonal gammopathy. A myeloma risk score enables the treating physician to judge whether a bone marrow examination and determination of the skeletal status are required. The revised guideline contains specific clinical questions and indications that can be used on the laboratory order forms and which facilitate the interpretation of the test results. The investigation of skeletal abnormalities must fulfill established criteria in order to be able to discriminate between MGUS and multiple myeloma, and for the staging of multiple myeloma. There is no indication for routine MRI, but MRI must be performed urgently in patients with radicular symptoms or a (threatened) transverse lesion. Both cytology and histology of the bone marrow are an essential part of the initial diagnosis and classification of multiple myeloma. Cytogenetic studies of the bone marrow are recommended as well. In the case of unexplained polyneuropathy, an M-protein should be looked for.
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PMID:[The CBO professional guideline 'monoclonal gammopathy' (paraproteinemia) (revision)]. 1217 32


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