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 POEMS syndrome (coined to refer to polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) remains poorly understood. Ambiguity exists over the features necessary to establish the diagnosis, treatment efficacy, and prognosis. We identified 99 patients with POEMS syndrome. Minimal criteria were a sensorimotor peripheral neuropathy and evidence of a monoclonal plasmaproliferative disorder. To distinguish POEMS from neuropathy associated with monoclonal gammopathy of undetermined significance, additional criteria were included: a bone lesion, Castleman disease, organomegaly (or lymphadenopathy), endocrinopathy, edema (peripheral edema, ascites, or effusions), and skin changes. The median age at presentation was 51 years; 63% were men. Median survival was 165 months. With the exception of fingernail clubbing (P =.03) and extravascular volume overload (P =.04), no presenting feature, including the number of presenting features, was predictive of survival. Response to therapy (P <.001) was predictive of survival. Pulmonary hypertension, renal failure, thrombotic events, and congestive heart failure were observed and appear to be part of the syndrome. In 18 patients (18%), new disease manifestations developed over time. More than 50% of patients had a response to radiation, and 22% to 50% had responses to prednisone and a combination of melphalan and prednisone, respectively. We conclude that the median survival of patients with POEMS syndrome is 165 months, independent of the number of syndrome features, bone lesions, or plasma cells at diagnosis. Additional features of the syndrome often develop, but the complications of classic multiple myeloma rarely develop.
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PMID:POEMS syndrome: definitions and long-term outcome. 1245

Multiple myeloma, which primarily affects the elderly, is rare in the head and neck. We report the case of a 71-year-old man who came to us with hoarseness, dysphagia, intermittent aspiration, and cervical lymphadenopathy. Our work-up included laboratory tests, radiographic examinations, analysis of bone marrow aspiration, and histopathologic evaluations. Cervical lymph node biopsy confirmed a diagnosis of multiple myeloma. Despite treatment with chemotherapy and radiation, the patient died of his disease 6 months later.
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PMID:Multiple myeloma in a patient with hoarseness, dysphagia, aspiration, and cervical lymphadenopathy. 1514

B cell neoplasms present heterogeneous patterns of lymphoid organ involvement, which may be a result of the differential expression of chemokine receptors. We found that chemokine receptor (CCR)7, CXC chemokine receptor (CXCR)4, or CXCR5, the main chemokine receptors that mediate B cell entry into secondary lymphoid tissues and their homing to T cell and B cell zones therein, were highly expressed in B malignancies with widespread involvement of lymph nodes. Conversely, those pathologies with little or no nodular dissemination showed no expression to very low levels of CCR7 and CXCR5 and low to moderate levels of CXCR4. These findings provide evidence for the role of CCR7, CXCR4, and CXCR5 in determining the pattern of lymphoid organ involvement of B tumors. Functional studies were performed on B malignancies expressing different levels of CCR7, CXCR5, and CXCR4. Multiple myeloma (MM) cells did not express CCR7 nor CXCR5 and did not migrate in response to their ligands; a moderate expression of CXCR4 on MM cells was accompanied by a migratory response to its ligand, CXCL12. By contrast, cells from B cell chronic lymphocytic leukemia (B-CLL) expressed the highest levels of these chemokine receptors and efficiently migrated in response to all ligands of CCR7, CXCR4, and CXCR5. In addition, the migration index of B-CLL cells in response to both of the CCR7 ligands correlated with the presence of clinical lymphadenopathy, thus indicating that the high expression of functional chemokine receptors justifies the widespread character of B-CLL, representing a clinical target for the control of tumor cell dissemination.
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PMID:Chemokine receptors that mediate B cell homing to secondary lymphoid tissues are highly expressed in B cell chronic lymphocytic leukemia and non-Hodgkin lymphomas with widespread nodular dissemination. 1515 73

A 63-year-old man was admitted because of general malaise, fever, headache, generalized lymphadenopathy and hepatomegaly in July 2002. He was diagnosed as having multiple myeloma (MM) (IgG-kappa type) with atypical plasma cells in the bone marrow, lymph nodes and cerebrospinal fluid. Systemic and intrathecal chemotherapy were effective. Because of an increase of polyclonal IgE, electrophoretic patterns revealed an M-peak which was not as sharp as that in IgG myeloma. IgE production is not impaired by the pathologic process in MM patients.
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PMID:[Multiple myeloma (IgG-kappa) infiltrating central nervous system, lymph nodes, liver, and kidneys, and with elevation of IgE]. 1519 53

The coexistence of autoimmune diseases and malignancies including lymphoproliferative diseases is often reported in the literature. Here we report an unusual case with two autoimmune diseases--myasthenia gravis (MG) and systemic lupus erythematosus (SLE) associated with unique palmoplantar keratoderma (PK) which preceded the development of multiple myeloma (MM) for twenty and seven years respectively. MG associated with non-malignant thymoma developed in 1981 and was successfully treated with thymectomy and physostigmine. Thirteen years later SLE was diagnosed and until now it is also accompanied by skin lesions corresponding to non-familial, diffuse palmoplantar keratoderma which is resistant to treatment. In 2001 the patient revealed inguinal and abdominal lymphadenopathy first diagnosed as extramedullary plasmacytoma and then as multiple myeloma on the basis of bone marrow infiltration and monoclonal gammopathy. Therapy with VAD regimen achieved complete remission of the MM and significant improvement of the skin changes lasting for six months. We failed to collect sufficient numbers of CD 34+ cells for peripheral blood stem cell transplantation. Now the malignancy is in partial remission after CHOP therapy and the skin lesions have returned to their initial status. To our knowledge, this is the first case to be reported with coexistence of these four diseases.
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PMID:Multiple myeloma in a patient with systemic lupus erythematosus, myasthenia gravis and non-familial diffuse palmoplantar keratoderma. 1522 54

We describe a 79-yr-old man with a history of androgen-independent metastatic prostate cancer treated with exogenous estrogens presenting with bilateral breast masses associated with bilateral axillary lymphadenopathy. Although the findings on physical examination with the concomitant history of estrogen therapy for metastatic prostate cancer raised the clinical suspicion of breast cancer, fine-needle aspiration (FNA) cytology identified the lesions as multiple myeloma.
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PMID:Fine-needle aspiration diagnosis of plasmacytoma presenting as breast masses in a patient on estrogen therapy for prostate cancer. 1554 Jan 81

Chronic idiopathic myelofibrosis (CIMF) is a chronic myeloproliferative disorder (CMPD) with progressive fibrosis and extramedullary hematopoiesis. Similar to other CMPDs, the stem cell in CIMF has the potential to differentiate into myeloid or lymphoid lineages, and thus CIMF can culminate in acute leukemia of myeloid or, rarely, lymphoid lineage. We describe an unusual case of CIMF terminating in extramedullary anaplastic plasmacytoma. The patient was a 61-year-old male with an 11-year history of CIMF. His course was complicated by rapidly growing abdominal and inguinal lymphadenopathy. Lymph node biopsy revealed a diffuse undifferentiated infiltrate in the background of extramedullary hematopoiesis. Flow cytometric and immunohistochemical analysis demonstrated plasma cell-related antigens (CD138, CD38, cytoplasmic kappa light chain), epithelial membrane antigen and CD43 in the tumor cells. The myeloid, B-cell or T-cell markers were negative. A clonal immunoglobulin heavy chain gene rearrangement was identified by polymerase chain reaction. The plasma cell origin was further confirmed by electron microscopic examination, which revealed stacks of rough endoplasmic reticulum. Monoclonal gammopathy may occur in CIMF, and rare cases of simultaneous plasma cell myeloma and CIMF have been reported in the literature. However, to the best of our knowledge, this is the first report of CIMF terminating in extramedullary anaplastic plasmacytoma.
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PMID:Chronic idiopathic myelofibrosis terminating in extramedullary anaplastic plasmacytoma. 1632 64

Plasma cell leukaemia is a rare variant of multiple myeloma (2-3%), with an aggressive disease with short survival. It is defined as circulating peripheral blood plasma cells exceeding 2.000/ul and 20% of peripheral blood white cells. We present the case of 32-years-old man with left chest-wall pain, hepatomegaly, righ cervical adenopathy, 8800 leukocytes/ul with 33% of lymphocytes and 40% of plasma cell, 10.8 g/dl of proteins and a monoclonal hypergammaglobulinemia. Bone marrow biopsy showed 40-50 % of intramedullary plasma cell. There was a Ig G-kappa paraproteinemia.
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PMID:[Plasma cell leukaemia: a rare variant of multiple myeloma. A case report]. 1645 87

A 73-years old patient came to our outpatient clinic because of a blue discoloration of his hands. Furthermore there was a systemic inflammatory reaction, burning pain and numbness of his feet due to a polyneuropathy, and an indolent enlargement of the cervical lymph nodes. In 1996 and 2001 cervical lymph node resections were done because of localized angiofollicular lymphnode hyperplasia (Castleman's disease). The laboratory values confirmed a systemic inflammatory reaction, a hypothyreosis and a monoclonal gammopathy. A CT-scan showed enlarged cervical, intrathoracic and abdominal lymphnodes and a splenomegaly. So all the criterias for a POEMS syndrome (special form of multiple myeloma) were met with polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes. The generalized lymphadenopathy corresponded histologically to the prior mentioned Castleman's disease. The patient responded well to systemic glucocorticoid treatment and today he is asymptomatic.
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PMID:[Blue discoloration of hands, numbness of feet, indolent cervical lymph node swelling in a 73-year-old man]. 1708 Jul 61

We report on bone marrow plasmacytosis in four cases of idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia (IPL). Pathologically, the plasma cells increased in number and accounted for 20-40% of nucleated cells of bone marrow. These plasma cells diffusely infiltrated or formed numerous clusters with 50-200 cells on histological sections. Some binuclear plasma cells and Russell bodies were seen, but all plasma cells showed mature cytomorphology. One case contained two lymphoid follicles with normal germinal centers. Immunoperoxidase studies of light chain determinants for plasma cells and their precursors demonstrated a polyclonal pattern. The immunohistochemical study revealed that there were no human herpes virus-8-positive cells. Bone marrow plasmacytosis of striking proportions may occur in a number of inflammatory conditions, chronic infections, autoimmune diseases, and hypersensitivity states. These reactive plasmacytoses, although sometimes striking, are generally composed of scattered, non-aggregated plasma cells. The four cases described here contained numerous tumor-like aggregations on mature plasma cells. Our four cases should be differentiated from plasma cell myeloma composed of mature plasma cells. However, electrophoresis generally demonstrated a broad-based polyclonal hypergammmaglobulinemia. Moreover, the immunohistochemical study revealed a polytypic nature of the plasma cells. To avoid overdiagnosis and overtreatment, it is important to be aware of the bone marrow findings of IPL.
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PMID:Bone marrow plasmacytosis in idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia: a report of four cases. 1790


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