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

Lymph node infiltration by monoclonal plasma cells can occur either in aggressive forms of myeloma or may represent regional extension of extramedullary plasmacytomas, whereas lymph node plasmacytoma presenting as a solitary extramedullary plasmacytoma is very unusual. We report two cases of lymph node plasmacytomas without systemic disease diagnosed after surgical excision. Clinical remission was achieved after local radiotherapy although one patient relapsed with multifocal extramedullary plasmacytomas 20 months after radiotherapy.
 ...
PMID:Extramedullary plasmacytoma: report of two cases with uncommon presentation. 1504 15

Epidural spinal cord compression is a neurologic emergency requiring immediate attention. The therapy instituted depends on several factors, including the patient's condition at the time of presentation, the nature of the underlying malignancy, the extent of systemic disease burden, and patient prognosis. The most essential aspect of treatment is the establishment of the diagnosis. If one suspects malignant cord compression an emergency, magnetic resonance imaging of the entire spinal axis is indicated. If magnetic resonance imaging is unavailable, post-myelographic computed tomography is an alternative. However, treatment should not be delayed until imaging is performed, particularly if neurologic deficits are present. Pain should be adequately addressed and opioids administered if necessary. Steroids should be given. If significant neurologic deficits are present, a high-dose corticosteroid bolus, followed by standing doses, should be given. However, if pain is the predominant symptom, steroids can be withheld pending immediate imaging or lower doses can be given without a bolus. Neurosurgical consultation should be obtained, and surgery should be considered if the patient's condition permits. This is particularly true if spinal instability or significant kyphosis is present or compression is secondary to bony fragments. Other indications include patients with limited systemic disease burden in whom better survival is predicted and possibly those with radioresistant tumors. The type of surgery performed should be tailored to the distribution of disease within the spine and accessibility through anterior body cavities. Radiation therapy, an effective noninvasive treatment that can be delivered quickly and safely, is an appropriate option as well. This is particularly true in radio-responsive tumors, such as myeloma and lymphoma, in which surgery may be avoided entirely. Chemotherapy may play a role as adjuvant therapy in some tumors.
 ...
PMID:Epidural Spinal Cord Compression. 1515 6

AL amyloidosis is a rare disorder characterised by tissue deposition of a fibrillary proteinaceous material, formed from monoclonal immunoglobulin light (or exceptionally heavy) chains. Although it may complicate multiple myeloma or B-cell lymphomas, AL amyloidosis is often associated with a low burden of clonal plasma cells ("primitive" AL amyloidosis). The mechanisms involved in the formation of AL amyloid deposits remain unclear, but are probably related to structural peculiarities of monoclonal immunoglobulin light chains. AL amyloidosis is usually a systemic disease, often revealed by renal involvement, the most common complication of the disease. The longterm prognosis of AL amyloidosis is poor, mainly related to amyloid restrictive cardiomyopathy leading to congestive heart failure. Oral melphalan and prednisone is considered the standard treatment for AL amyloidosis, but with limited increase in the median survival. High-dose intra-venous melphalan with autologous stem cell transplantation is an effective treatment, aimed at eliminating the clonaly expanded plasma cells, which has been shown to induce complete hematologic remissions and to prolong survival. However, the tolerability of such treatment is low, limiting its use to selected patients. The development of new drugs, able to interfere with amyloid fibril deposition, may provide a new therapeutic approach.
 ...
PMID:[Immunoglobulin light chain amyloidosis: recent molecular, clinical and therapeutic approach]. 1529 Nov 38

A case of solitary extramedullary plasmacytoma of the left pterygoid fossa is presented, which is an unusual location for such tumors. A 54-year-old male patient was admitted for retro-orbital and left-sided facial pain which had persisted for several months. Physical, nasal endoscopic and optic examinations showed no abnormalities. No palpable cervical lymph nodes were found. MRI revealed a 2-cm mass in the left pterygoid fossa. A biopsy was carried out and the resulting histological evaluation of the mass confirmed a plasmacytoma. A comprehensive work-up, including bone marrow biopsy, total-body skeletal survey, technetium scintigrams, determination of urine Bence-Jones protein and serum myeloma protein, chest radiograph, total blood count and urinalysis, was carried out in order to rule out multiple myeloma. All of these studies were unremarkable in terms of a systemic disease and therefore the patient was diagnosed as having a solitary extramedullary plasmacytoma of the pterygoid fossa. He was referred for radiation therapy, receiving irradiation of 50 Gy for 6 weeks. The clinical manifestations, imaging findings, pathology and treatment procedures of extramedullary plasmacytoma are described, together with a review of the literature.
 ...
PMID:Solitary extramedullary plasmacytoma arising in the pterygoid fossa. 1551 2

A young female with Fisher-Evans' syndrome and a previous melanoma developed acute renal failure with generalized lymphoadenopathy and fever. The appearance of renal lesions is common in the course of several hematological disorders, but is unusual in Fisher-Evans' syndrome. Fisher-Evans' syndrome, defined as Coombs' positive hemolytic anemia and immune thrombocytopenia, is more frequently associated with the other autoimmune diseases, but not with renal involvement. In our case report, having excluded amyloidosis, myeloma, interstitial nephritis and sepsis, the rapid involvement of renal function with enlarged renal size seemed to suggest renal lymphoma. However, the lack of a monoclonal T-lymphocyte population in the renal tissue and peripheral blood, along with a clinical course characterized by a rapid reversibility of acute renal failure made this diagnosis rather an unlikely one. Polyclonal lymphocyte infiltration in a patient with a persistent autoimmune disease made us suspect a hyperimmune reaction. This syndrome is a non-neoplastic proliferation of B-cells involving an exaggeration of lymphocyte transformation. However, the clinical course is progressive and fatal, and can trigger a lymphoproliferative systemic disease. In our patient, two elements led us to suspect it was not a typical hyperimmune syndrome: first, polyclonal lymphocytes had massively infiltrated the kidney and, secondly, the clinical outcome was extremely favorable. Therefore, we were faced with an "atypical" and "singular" hyperimmune reaction with renal involvement, polyclonal proliferation of T-lymphocytes that had exhausted itself over time. Infective or toxic agents or drugs such as cyprofloxacin could have triggered the phenomenon, in the presence of a favorable condition such as Fisher-Evans' syndrome.
 ...
PMID:Acute renal failure in a young woman with Fisher-Evans' syndrome. 1559 44

Amyloidosis is a systemic disease in which clinical manifestations are caused by the replacement of normal tissue with insoluble amyloid fibrils. Cardiac involvement causes a restrictive cardiomyopathy and is associated with poor functional outcomes. Cardiac magnetic resonance imaging and measurement of B-type natriuretic peptide are particularly helpful in distinguishing restrictive cardiomyopathy from constrictive pericarditis, but a tissue biopsy is required to make the diagnosis of amyloidosis. Although standard treatment options for congestive heart failure may provide symptomatic relief in cardiac amyloidosis, prognosis remains dismal. Judicious diuretic use remains the mainstay of therapy, but achieving optimal fluid balance is difficult because patients are usually "preload dependent." Angiotensin-converting enzyme inhibitors in low doses are often helpful but may lead to orthostatic hypotension, particularly in patients who also have involvement of the autonomic nervous system. beta Blockers may be useful if given relatively early in the disease process, but should be used with caution in patients with advanced disease because they may exacerbate symptoms. Therapy aimed at the underlying disease process in primary systemic amyloidosis is based on treatment regimens used in multiple myeloma, such as melphalan and prednisone. These offer limited benefit when cardiac involvement is significant, but newer treatments, including the novel anthracycline 4'-iodo-4'-deoxydoxorubicin, potentially combined with autologous stem cell transplantation, offer some hope for the future.
 ...
PMID:Current therapeutic strategies in cardiac amyloidosis. 1628 71

Less than 5% of patients with a plasma cell dyscrasia present with a single bone (SBP) or extramedullary plasmacytoma (EMP) without evidence of systemic disease (normocalcemia, absence of anemia, preservation of uninvolved immunoglobulins, or renal disease attributable to myeloma). Diagnosis requires biopsy confirmation of a monoclonal plasma cell infiltrate from a single site. The treatment of choice for both entities is radiotherapy given with curative intent (> 4000 cGy) resulting in long term disease-free survival in approximately 30% of patients with SBP and 65% of patients with EMP.
 ...
PMID:Solitary bone and extramedullary plasmacytoma. 1630 6

The authors describe the case of a 72-year-old woman with dural plasmacytoma revealing an immunoglobulin (Ig) G-kappa multiple myeloma (MM). She presented with headaches and left hemiparesis. Magnetic resonance imaging demonstrated a right frontal extraaxial lesion arising from the dura mater, and biological studies revealed hypercalcemia, hyperproteinemia, and a serum gamma globulin peak. A diagnosis of IgG-kappa MM was based on microscopic examination and immunohistochemical analysis of the dural plasmacytoma as well as on signs of systemic myeloma after surgery. The patient died 3 years after the first symptoms of MM despite systemic chemotherapy and no recurrence of the dural plasmacytoma. Myelomatous involvement of the dura mater is a rare occurrence given that only three cases have been reported to date. Nevertheless, this pathological entity should be differentiated from solitary dural plasmacytoma (SDP) because the prognosis is radically different. Progression seems to be correlated with systemic disease in contrast to the long-term survival associated with SDP. Careful systemic evaluation should be made in such a presentation to rule out MM, which would require different management and has a different prognosis.
 ...
PMID:Dural plasmacytoma revealing multiple myeloma. Case report. 1661 66

We report a case of a 64-year-old patient in whom limitation of mouth opening was the presenting symptom of plasmacytoma. Intra-oral biopsy confirmed a plasma-cell tumour, with no sign of extension on imaging. He was treated with radiotherapy (4000 cGy) and followed up. The diagnosis of solitary plasmacytoma of bone can be confirmed only when the presence of systemic disease has been excluded by clinical, biological, and radiological investigations. We think that the treatment should consist only of radiotherapy. Long-term follow-up is necessary because of the high risk of development of multiple myeloma, which may be delayed.
 ...
PMID:Plasmacytoma in the temporomandibular joint: a case report. 1769 64

Amyloidosis is a broad and complex class of diseases that comprises several etiologies, many manifestations, and a diversity of outcomes. We discuss a patient with primary systemic amyloidosis associated with multiple myeloma that illustrates many of the typical and atypical features of the disease process. Despite more in-depth assessment and accurate classification, survival for patients with primary systemic disease remains poor.
 ...
PMID:Primary systemic amyloidosis associated with multiple myeloma: a case report and review of the literature. 1795 7


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>