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)

A variety of tumors and nontumorous lesions were used to illustrate some of the biologic, clinical, and pathologic aspects of inappropriate or ectopic endocrine and metabolic syndromes that have musculoskeletal repercussions. It is clear, both from the discussion and case material, that many mechanisms of ectopic endocrine syndromes have yet to be clarified. Elaborate techniques are available for hormonal estimations, but their routine use is prohibitively expensive and relatively unrewarding. Cells of a given lesion may be functionally heterogeneous or may fail to elaborate active products in substantial amounts. Different cells may produce similar peptides, while the same cells can produce more than one. Despite these difficulties, recognition of ectopic endocrine syndromes remains crucial to diagnosis and patient management, and thus corroboration or correlation must often rest on a cruder basis. In fact, the clinical significance of basic laboratory data, e.g., PTH elevation, may vary, as PTH may be immunoreactive but biologically inactive. As another example, hypercalcemia associated with myeloma may be variously related to coexistent hyperparathyroidism, renal disease, dehydration, or humoral osteolysis. Therefore, roentgenographic evidence of bone destruction or skeletal stigmata of hyperparathyroidism imbues laboratory data with greater significance. Paraneoplastic syndromes are of particular concern to the radiologist, as multiple systemic manifestations, occurring either synchronously or metachronously, may suggest the presence of an underlying or unifying lesion or even of a specific type of neoplasm. They may precede detection of neoplasms by months to years and can develop at any time during their course. Paraneoplastic syndromes may, furthermore, parallel the course of a lesion and be used as indicators of remission or recurrence. Conversely, those unassociated with overt symptoms possess endocrine markers that can reinforce sometimes silent skeletal stigmata. Familiarity with representative secretory products influencing the musculoskeletal system per se may often clarify seemingly innocuous and sometimes asymptomatic skeletal findings. Alert imagers may, therefore, detect, infer, or suspect particular syndromes when they present in a specific sequence or mosaic. Conversely, when apprised of their existence, imagers should know where their related effects may be sought or anticipated. Such relationships, sometimes serendipitously discovered, may be valuable assets in clinical diagnosis and patient management in both suspected and unsuspected cases.
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PMID:Skeletal manifestations of ectopic or inappropriate endocrine and metabolic syndromes. 198 23

A patient with peripheral T-cell Lymphoma and acquired, systemic osteosclerosis is described. Bone histology showed a spectacular activation of osteoblasts accompanyed by massive new bone formation. Alkaline phosphatase in serum was elevated and increased to greater than 2000 U/l when the lymphoma became refractory to chemotherapy. In the patient's serum an osteoblast-activating factor could be demonstrated using a rat osteogenic osteosarcoma cell line (ROS 17/2.8). The factor was absent during remission of the tumor. We conclude that osteosclerosis was a paraneoplastic syndrome in this patient due to the secretion of an osteoblast-stimulating factor by the T-cell lymphoma. This situation is similar to the secretion of osteoclast-activating factors described in B-cell lymphomas, particularly multiple myeloma. The characterization of such a factor could be of therapeutic relevance.
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PMID:Evidence for an osteoblast-activating factor in a patient with peripheral T-cell lymphoma and osteosclerosis. 278 45

A 64-year-old woman complained of reddish plaques that had suddenly appeared on her upper arms and trunk. Histological investigation revealed perivascular distribution of a neutrophilic infiltrate in the upper and mid-dermis; direct immunofluorescence showed deposits of C3 and fibrin in and around the vessels. Laboratory values in the blood, X-ray of the head and fine-needle biopsy of the hip showed the typical pattern of multiple myeloma. After chemotherapy the dermatological lesions improved rapidly, and the plasmocytoma reached remission stage. The diagnosis of Sweet's syndrome was established on the basis of the clinical and histological changes and the improvement observed after steroid therapy. The coincidence with plasmocytoma confirms the interpretation of Sweet disease as a paraneoplastic syndrome.
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PMID:[Sweet syndrome. Acute neutrophilic dermatosis in multiple myeloma]. 848 3

A 12-year-old female, neutered German shepherd dog developed progressive hindlimb followed by forelimb ataxia leading to tetraplegia. Neurological examination suggested lower motor dysfunction. Biochemical evaluation revealed a monoclonal hypergammaglobulinaemia, hypoalbuminaemia and hypercalcaemia. Multiple lytic lesions were identified radiographically in numerous bones. A bone marrow aspirate confirmed the diagnosis of multiple myeloma, with large numbers of plasma cells seen in clusters. An electromyogram revealed positive sharp waves and fibrillation potentials in the skeletal muscles of the limbs, suggesting a polyneuropathy. The dog was treated with chemotherapy using melphalan and prednisolone. Both the hypergammaglobulinaemia and the polyneuropathy resolved and the dog had normal motor function four weeks after commencing treatment. Polyneuropathy may occur as a paraneoplastic syndrome secondary to myeloma, and in this case was reversible following treatment of the underlying disease.
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PMID:Multiple myeloma with associated polyneuropathy in a German shepherd dog. 963 62

POEMS syndrome is a rare condition with cutaneous manifestations commonly including angiomas, hypertrichosis, hyperpigmentation, and thickening of the skin. We describe a male patient with a 2-year history of cervical lymphadenopathy, erythematous thickening of the skin on the neck, and progressive walking difficulties. The patient had an occipital erythema with scarring alopecia and sparse follicular pustules at the edge of the lesion. Further investigation revealed symmetric polyneuropathy, hepatosplenomegaly, monoclonal gammopathy, subclinical thyreopathy, and an osteolytic bone lesion of the skull. Histologically, a plasmacytoma with lambda cell restriction was found. The overlying skin showed marked fibrosis, with loss of hair follicles, and a plasma cell infiltrate of polyclonal origin. The cervical lymph nodes showed histologic characteristics of multicentric Castleman's disease, and the skin of the neck showed thickening and vasoproliferation. There was no evidence of further plamacytomas. After excision of the plasmacytoma and postoperative irradiation, the symptoms gradually resolved within a few months. A cicatricial lesion remained on the occiput without further folliculitis or hair loss on the rest of the scalp. This case illustrates the reactive character of POEMS syndrome as a paraneoplastic syndrome in myeloma patients.
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PMID:POEMS syndrome: cicatricial alopecia as an unusual cutaneous manifestation associated with an underlying plasmacytoma. 1032 21

Hypercalcemia is a common paraneoplastic syndrome that may result in metastatic calcification. We report here on four autopsy cases with paraneoplastic hypercalcemia with metastatic calcification, to evaluate the clinicopathologic manifestations. All were males, aged 37-63 years old. Primary tumors included one transitional cell carcinoma of the urinary bladder, one multiple myeloma, and two squamous cell carcinomas of the esophagus. Calcium concentrations ranged from 3.3 to 5.9 mmol/L. Chronic hypercalcemia resulted in metastatic calcification. The kidney and stomach were the most vulnerable organs. Only case 1 presented with an increase in plasma calcium above 5 mmol/L (about twice the normal value); the metastatic calcification involved the capillary walls of his lungs, and he died of fulminant pulmonary edema. Our conclusion is that judicious treatment for paraneoplastic hypercalcemia is important with respect to the occurrence of pulmonary edema associated with metastatic calcification.
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PMID:Paraneoplastic hypercalcemia with metastatic calcification--clinicopathologic studies. 1656 25

A 52-year-old patient presented himself with weight loss and night sweats. Laboratory analyses revealed a high sedimentation rate, elevated immunoglobulines and anaemia with sludge phenomenon. Differential diagnoses included Multiple Myeloma and Lymphoma. Having a risk constellation for HIV infection and just having recovered from oral thrush also made this diagnosis possible. Urinary analysis and chest x-ray were normal; however, CT-scan detected renal cell cancer with pulmonary metastases. Renal cell cancer is heterogeneous in presentation, symptoms are unspecific, therefore they are often discovered late when they have already metastasized. Paraneoplastic syndromes, e.g. hypercalcaemia or hypertension are not infrequent in renal cell cancer.
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PMID:[Weight loss and night sweats with unexpected tumor localization]. 1807 82

POEMS syndrome is a rare multisystemic disorder characterized by polyneuropathy (P), organomegaly (O), endocrinopathy (E), blood dyscrasia with M-protein band (M), and skin changes (S). The pathogenesis of POEMS syndrome is not completely understood. This paraneoplastic syndrome may be associated to bone lesions that are characteristically sclerotic or lytic with sclerotic halo and negative at bone scintigraphy. We report a case of a 37-year-old man with several manifestations of POEMS syndrome who presented bone lesions characteristic of osteosclerotic myeloma confirmed by bone biopsy.
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PMID:[POEMS syndrome: a case report]. 1940 40

POEMS syndrome is a paraneoplastic syndrome whose acronym stands for less than half of the defining features of the disease, that is, polyradiculoneuropathy, organomegaly, potentially including coexisting Castleman disease, endocrinopathy, monoclonal plasma cell neoplasm, and skin changes. The other important features include papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis, elevated VEGF, and abnormal pulmonary function. The diagnosis is based on having both the polyradiculoneuropathy and the monoclonal plasma cell disorder, and at least 1 of the other 3 major criteria (Castleman disease, sclerotic bone lesions, or elevated VEGF) and at least one minor criterion. The diagnosis is often delayed with intervening incorrect diagnoses of chronic inflammatory demyelinating polyradiculoneuropathy, myeloproliferative disorder, and monoclonal gammopathy of undetermined significance. Prompt treatment directed at the underlying plasma cell clone produces dramatic responses in the majority of patients. Although there are no randomized clinical trial data to direct best therapy, for patients with disseminated disease, high-dose chemotherapy with peripheral blood transplantation has yielded durable benefit, whereas radiation therapy is typically effective for patients with a more localized presentation. More universal recognition of and more scientific inquiry into the underpinnings of the disease will provide direction toward the best treatment strategies in the future.
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PMID:How I treat POEMS syndrome. 2254 81

Cancer-related microangiopathic hemolytic anemia (CR-MAHA) is a paraneoplastic syndrome characterized by Coombs-negative hemolytic anemia with schistocytes and thrombocytopenia. We reviewed and analyzed all cases of CR-MAHA reported since 1979 (the time of the last published review on this topic) according to predefined criteria. We found 154 cases associated with solid cancer and 14 with lymphoma. Among the solid cancers, gastric, breast, prostate, lung, and cancer of unknown primary (CUP) were most common; 91.8% of cancers were metastatic, and in 19.4% of solid cancers CR-MAHA did not occur until recurrence of cancer. Lymphoma cases included Hodgkin disease, angiotropic lymphoma, diffuse large cell lymphoma, and myeloma. Evaluation of the clinical and laboratory findings revealed that only a minority of cases presented with the features of thrombotic thrombocytopenic purpura (TTP) or atypical hemolytic uremic syndrome (aHUS), with the exception of prostate cancer, where aHUS was a common presentation. Compared to hereditary or immune TTP or aHUS, disseminated intravascular coagulation and pulmonary symptoms were more common in CR-MAHA. Plasma exchange or fresh frozen plasma was rarely effective except in prostate cancer patients with aHUS. CR-MAHA responded to antitumor therapy in many patients with gastric, breast, lung, and CUP cancers. These patients had a superior survival compared to patients without chemotherapy. Compared to the prognosis of patients with metastatic cancer without CR-MAHA, the prognosis of CR-MAHA patients was greatly inferior. There is evidence that some cases of CR-MAHA in lymphoma are immune mediated.
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PMID:Cancer-related microangiopathic hemolytic anemia: clinical and laboratory features in 168 reported cases. 2273 49


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