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)

Necrobiotic xanthogranuloma with paraproteinemia is a distinct clinicopathologic entity defined by skin lesions that are characteristic both clinically and histologically, as well as a by variety of hematologic and chemical abnormalities. It is frequently associated with multiple myeloma or chronic lymphocytic leukemia. A patient with the characteristic findings but with an unusual course is described.
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PMID:Necrobiotic xanthogranuloma with paraproteinemia. 258 52

Necrobiotic xanthogranuloma with paraproteinemia is a clinical and histopathological entity characterized by xanthelasma-like lesions in the periorbital region and elsewhere, paraproteinemia, leukopenia, and an elevated erythrocyte sedimentation rate. Multiple myeloma has been reported as an accompanying feature in several cases. We examined a patient with necrobiotic xanthogranuloma and multiple myeloma in whom an IgG kappa monoclonal protein was identified in serum, urine, bone marrow, and bilateral periorbital lesions. We speculate that increased serum immunoglobulins complexed with lipid may be deposited in the skin, leading to a foreign body giant cell reaction and the subsequent characteristic histopathologic features of necrobiotic xanthogranuloma.
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PMID:Necrobiotic xanthogranuloma with paraproteinemia. Case report and a pathogenetic theory. 310 Oct 22

Necrobiotic xanthogranuloma with paraproteinemia is characterized by multiple nodules or plaques that involve the periorbital area along with other parts of the body. A dysproteinemia due to an IgG paraprotein is associated with the condition; low serum complement, cryoglobulinemia, leukopenia, and hyperlipemia are also sometimes seen. Multiple myeloma is present in some cases. Two cases of necrobiotic xanthogranuloma with IgG monoclonal gammopathy were seen. Both initially had ocular symptoms and in the second case, the ocular manifestations have dominated the clinical picture. Histologically, these granulomas are characterized by collagen necrobiosis and by the presence of many foamy histiocytes and Touton giant cells. Because necrobiotic xanthogranuloma with monoclonal gammopathy frequently has prominent manifestations in the orbital region, may result in dysfunction of the eyelids or extraocular muscles, and is associated with potentially life-threatening systemic conditions, its recognition by the ophthalmologist is important.
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PMID:Necrobiotic xanthogranuloma of the eyelid. 684 54

Necrobiotic xanthogranuloma with paraproteinemia is a progressive and destructive process that is often confused both clinically and histologically with other granulomatous and xanthomatous entities. It was first described by Kossard and Winkelmann in 1980. Prior to this, the entity was reported under a variety of names such as atypical multicentric reticulohistiocytosis with paraproteinemia, atypical xanthoma disseminatum, and atypical necrobiosis lipoidica. A 69-year-old woman experienced slightly pruritic and painful papules, plaques, and nodules. Initial biopsy specimens showed a granulomatous process consistent with granuloma annulare. Later biopsy specimens demonstrated histologic changes indicative of necrobiosis lipoidica. The most recent histologic findings are those of necrobiotic xanthogranuloma, with results of laboratory studies revealing a coexistent IgG kappa paraproteinemia. Patients with necrobiotic xanthogranuloma who demonstrate a benign monoclonal proteinemia and are evaluated for several years show a 9 to 11 percent risk of myeloma, amyloidosis, or macroglobulinemia. Multiple treatment regimens have been attempted, none of which are curative. We propose that this entity may be part of an evolutionary process that may start as a granulomatous entity and culminate in a xanthogranulomatous process with an accompanying paraproteinemia.
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PMID:Necrobiotic xanthogranuloma with paraproteinemia: an evolving presentation. 921 93