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)

Immunoglobulin D (IgD) myeloma is a rare disease accounting for about 2% of all myelomas. The distinctive features are the predominant occurrence in males and young patients, short survival time, uncertain appearance of M-component in serum electrophoresis, predominance of lambda light chains, frequent renal impairment, hypercalcemia and amyloidosis. The aim of the present study was to show diagnostic difficulties resulting from a variety of non-specific initial symptoms and laboratory findings as well as to compare the staging system proposed by Durie and Salmon with the new risk grouping by Shimamoto. Case histories of 7 patients were analyzed retrospectively. Five of them were diagnosed as IgD multiple myeloma (IgD MM), 1 as non-secretory IgD myeloma and 1 as solitary bone IgD plasmocytoma that evolved to an IgD MM. All patients were staged according to the Durie and Salmon classification and the new risk grouping by Shimamoto. We report diagnostic problems with IgD myeloma in our patients, with special emphasis on non-specific rheumatoidal and neurological symptoms in 1 case. There was a very good correlation of the Japanese classification with the severity of the disease and the risk of death. In conclusion, the initial symptoms of IgD myeloma can be very misleading. Wide differential diagnosis, including autoimmunological disorders of the connective tissue, is necessary. The new Japanese risk grouping seems to be of greater prognostic significance for IgD myeloma than the Durie and Salmon staging system.
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PMID:Immunoglobulin D myeloma--problems with diagnosing and staging (own experience and literature review). 1601 54

Staging and monitoring of multiple myeloma (MM) is mainly based on monoclonal component quantification; the absence of such a parameter renders difficult follow up of patients with nonsecretory MM (nsMM). In this study our aims were to determine the specificity and sensitivity of Tc99m-sestaMIBI scintigraphy at diagnosis and during follow up of nsMM patients. Nine nsMM patients were prospectively studied at diagnosis and during treatment for a mean time of 33 months (range: 12-65+). Tc99m-sestaMIBI (MIBI) scintigraphy was compared to conventional imaging (CI: X ray with CAT or NMR details) at diagnosis and during follow up. At diagnosis, CI and MIBI were concordant in three patients; CI showed more focal lesions than MIBI in four patients, while MIBI revealed more focal lesions than CI in two patients. During the follow up, MIBI uptake was normal in the four patients who achieved remission. Five patients did not achieve remission: CI and MIBI were concordant in three, while MIBI was falsely negative in two patients. In conclusion, Tc99m-sestaMIBI scintigraphy has high sensitivity (no false positive cases) and 78% specificity (2/9 false negative cases) in tracing active nsMM lesions; it should be considered complementary to CI for monitoring this rare disease.
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PMID:Tc99m-sestaMIBI uptake in nonsecretory multiple myeloma. 1608 47

Solitary plasmocytoma (SP) represent only about 5% of plasma cell neoplasia. Most patients have generalized disease, that is, multiple myeloma (MM). Solitary bone plasmocytoma (SBP) is a localized plasma cell tumor and is a very rare disease in young patients. We reported here, a case of SPB in a 14-year-old girl with a 10-year disease-free survival after an aggressive treatment. The relationship of SBP to MM continues to be controversial. Recommendations on the diagnosis and management of SBP in adults, based on a literature search and consensus of expert opinion, were recently published on behalf of the Guidelines Working Group of the United Kingdom Myeloma Forum 1. MRI of the spine is necessary to assess local disease. Radiotherapy with doses of 45-50 Gy is the recommended treatment and gives a high rate of local control (83-96%). Chemotherapy remains controversial in contrast to MM, in which intensive chemotherapy with autologous bone marrow transplantation (ABMT) is widely accepted. At the present time, considering the good prognosis of patients with a normal MRI at diagnosis and a complete disappearance of the M protein after radiotherapy, we believe that ABMT should be reserved for relapse or primary therapeutic failure.
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PMID:Solitary bone plasmocytoma of the spine in an adolescent. 1608 17

Pancreatic lymphoma is an extremely rare disease. The role of radiation therapy as an adjunct to chemotherapy for the treatment of this disease is poorly defined. We present a case of primary pancreatic lymphoma treated with radiation therapy as an adjunct to chemotherapy.
Clin Lymphoma Myeloma 2005 Sep
PMID:Role of radiation therapy in a patient with primary pancreatic lymphoma. 1623 54

Plasma cell leukemia (PCL) is a rare disease and is the least common variant of multiple myeloma accounting for 2-3% of all plasma cell dyscrasias. We report a patient who presented with history of high grade fever, weakness, palpitations, loss of appetite, bone pains and mental confusion for twenty days. Initial evaluation revealed plasmacytosis with blood plasma cell count of 5184/cumm. His hemoglobin (Hb) was 11.3 gm/dl, platelets were 75000/cumm and total leucocyte count (TLC) was 21600/cumm (24% plasma cells). Bone marrow examination revealed >60% plasmablasts. Serum LDH was high at 3117 U/L and serum calcium was also elevated at 13.9 mg/dl. A diagnosis of PCL was made and the patient was started on treatment for hypercalcaemia with Melphalan/Prednisolone regime along with supportive care. Patient deteriorated very rapidly despite treatment and died on the eighth day. A detailed report of this case and a review of PCL is presented here.
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PMID:Plasma cell leukemia: case report of a rare and aggressive variant of multiple myeloma. 1630 56

Primary pulmonary lymphoma (PPL) is a rare disease with a favorable prognosis compared with lung cancer. Although a number of histologic variables of B- and T-cell lymphoma were reported as PPL, marginal zone lymphoma of the mucosa-associated lymphoid tissue type is by far the most frequent diagnosis. This review summarizes the present knowledge of histopathology, molecular biology, diagnosis, prognosis, and treatment of this heterogeneous entity.
Clin Lymphoma Myeloma 2005 Nov
PMID:Primary pulmonary lymphoma: current status. 1635 27

Primary hepatic non-Hodgkin's lymphoma (NHL) is a rare disease that presents unique diagnostic and therapeutic challenges. Secondary liver involvement by lymphoma is common and can complicate treatment decisions. A review of the published case reports and the few larger series suggests that primary hepatic NHL represents a heterogeneous mixture of disparate diseases rather than a single entity. Presentations vary from the incidental discovery of hepatic abnormalities in an otherwise asymptomatic patient to that of fulminant hepatic failure with rapid progression of encephalopathy to coma and death. The clinical, laboratory, and radiographic characteristics are nonspecific, which means the diagnosis is often not suspected until histopathologic examination of liver tissue. There appears to be a strong association between primary hepatic NHL and the hepatitis C virus. Hepatosplenic T-cell lymphoma has attained its own status as a unique disease, whereas case reports suggest that the spectrum of hepatic lymphoma includes many histologies. Involvement of the liver by lymphoma can compound the difficulty of pursuing aggressive chemotherapy in patients who have a life-threatening illness and impaired metabolism of the most effective drugs. Therapy should be tailored to the individual clinical situation, with consideration of the underlying histology and degree of hepatic insufficiency.
Clin Lymphoma Myeloma 2006 Jan
PMID:Non-Hodgkin's lymphoma involving the liver: clinical and therapeutic considerations. 1650 4

Primary lymphoma of the upper urinary tract is an extremely rare entity without specific clinical or laboratory findings. Thus, this particular diagnosis is rarely anticipated and might well be reached only after nephroureterectomy. We describe a patient with primary follicular and diffuse follicle center lymphoma arising in the renal pelvis that was treated with surgery and postoperative immunochemotherapy. Furthermore, we review the literature regarding the treatment and outcome of this rare disease.
Clin Lymphoma Myeloma 2006 Mar
PMID:Non-Hodgkin's lymphoma of the renal pelvis. 1664 Aug 18

Amyloidosis is a rare disease in which amyloid fibrils compromise organ function and lead to death. Systemic immunoglobulin light-chain amyloidosis, usually caused by free light chains (FLCs) made by clonal plasma cells, is the most frequent type. Hereditary and senile systemic amyloidosis are less frequent types. Rarely, a patient with a tissue diagnosis of amyloidosis might have a monoclonal gammopathy and a hereditary protein. In systemic immunoglobulin light-chain amyloidosis, circulating clonal light chains can be measured with the FLC assay and provide a target for therapy aimed at eliminating the underlying plasma cell disorder while supporting the patient. Elimination of the pathologic FLC can lead to resorption of amyloid deposits and improvement in organ function. Monthly oral melphalan and dexamethasone for 1 year is effective therapy for patients not eligible for autologous stem cell transplantation (SCT) but carries a risk of myelodysplasia. For patients with limited organ involvement, SCT is an effective approach and, when followed after SCT by adjuvant thalidomide and dexamethasone for persistent plasma cell disease, achieves a high 1-year hematologic response rate. Complete hematologic responses can be durable beyond a decade and are usually associated with organ recovery. New agents, such as bortezomib and lenalidomide, have shown promising activity, and novel monoclonal antibody approaches are also under active investigation.
Clin Lymphoma Myeloma 2006 Nov
PMID:Systemic immunoglobulin light-chain amyloidosis. 1722 32

Amyloidosis is a rare disease in which a specific protein is deposited as aggregated interstitial fibrils that can compromise organ function and lead to death. Immunoglobulin (Ig) light-chain amyloidosis (AL), caused by the monoclonal gammopathy of a plasma cell dyscrasia, is the most common type. A hereditary type is also caused by mutant transthyretin and other proteins. Rarely, a patient with amyloid has both a monoclonal gammopathy and a hereditary protein. In AL, circulating monoclonal Ig light chains can be measured with the free light-chain (FLC) assay and provide a target for therapy to eliminate the underlying plasma cell dyscrasia while supporting the patient's organ function. Amyloid deposits can be resorbed and organ function restored if the amyloid-forming precursor FLC is eliminated. For patients with limited organ involvement, intravenous melphalan in doses from 100 to 200 mg/m2 with autologous stem cell support (SCT) is an effective approach and, when followed at 3 months post-SCT with adjuvant thalidomide and dexamethasone for persistent plasma cell disease, has a 1-year hematologic response rate of 77%. Monthly oral melphalan and dexamethasone for 1 year can also be effective therapy for patients too sick for SCT (67% response rate). Hematologic complete responses are usually durable and result in long-term survival and a variable degree of organ recovery. For patients with advanced cardiac involvement, the prognosis remains guarded even with treatment. Drugs effective in multiple myeloma are usually active in AL, depending on side effects. New agents such as bortezomib and lenalidomide have shown promising activity, and novel antibody-based approaches for imaging amyloid and accelerating removal of deposits are being actively investigated.
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PMID:Managing systemic light-chain amyloidosis. 1733 87


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