Cases reported "Multiple Myeloma"

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1/27. sjogren's syndrome associated with multiple myeloma.

    sjogren's syndrome (SS) is a chronic autoimmune disease of unknown etiology characterized by lymphocytic infiltration of the exocrine glands and a polyclonal B-cell activation; it is demonstrated by the presence of multiple autoantibodies against organ- and non-organ-specific autoantigens. SS is associated with malignant lymphomas, Waldenstrom's macroglobulinemia and benign monoclonal gammopathy, while its relationship with multiple myeloma is extremely rare. The association between multiple myeloma and rheumatoid arthritis and other autoimmune diseases has been established, but it is not clear why a B-cell proliferation like myeloma occurs more rarely than other B-cell disorders in patients with SS. We describe a patient who presented with multiple myeloma and SS that might have existed for at least 2 years prior to the appearance of myeloma.
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2/27. multiple myeloma involving the lacrimal gland.

    The third case of myeloma presenting as a lacrimal gland tumor is reported. The lesion was originally diagnosed as a solitary extramedullary myeloma, but on follow-up, the patient was seen to develop other myelomas. This case illustrates the necessity for careful follow-up of patients with solitary myelomas, even in the absence of bone marrow pathology, for the solitary extramedullary myeloma may represent an early stage in the development of multiple myeloma.
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3/27. multiple myeloma with myeloma nephropathy in a patient with Hashimoto's thyroiditis.

    Hashimoto's thyroiditis is associated with myeloproliferative and lymphoproliferative neoplasms. The risk of carcinoma of the thyroid gland is increased in these patients. Furthermore, multiple myeloma can present together with some autoimmune diseases. We report the case of a 57-year-old woman with Hashimoto's thyroiditis who developed multiple myeloma with myeloma nephropathy. Her renal function deteriorated to end stage and she required maintenance hemodialysis. Although autoimmune disorder might play an important role in lymphomagenesis in patients with Hashimoto's thyroiditis, it is not known whether the chronic inflammation that takes place in Hashimoto's thyroiditis stimulates the development of multiple myeloma. The pathogenetic mechanisms responsible for the development of multiple myeloma in patients with Hashimoto's thyroiditis remain unclear.
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4/27. Thyroid involvement in multiple myeloma.

    Involvement of the thyroid gland by plasma cell neoplasms is a rare occurrence. Two modes of presentation are described; firstly as part of disseminated myeloma and secondly, as an isolated plasma cell neoplasm of the thyroid as the only evidence of disease. We wish to describe two cases of thyroid involvement as part of disseminated disease.
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5/27. Extramedullary presentation of multiple myeloma in the parotid gland as first evidence of the disease -- a review with case report.

    Myeloma is a neoplasm of plasma cells that causes painful, bone-destructive lesions. It may present as a solitary lesion (plasmacytoma), or involve multiple sites (multiple myeloma). Myeloma may occur in an extraosseous location as a solitary lesion, or less commonly, in combination with multiple myeloma. oral manifestations may be the first sign or symptom of the disease. Soft tissue plasmacytomas of the head and neck tend to involve the nasal cavity or nasopharynx, rather than the oral cavity. Its occurrence in the parotid gland is rare. We present an unusual case of multiple myeloma with extramedullary involvement involving the parotid gland as first evidence of the disease. multiple myeloma should be considered in the differential diagnosis of salivary gland swellings.
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6/27. Rare skin disorder complicating doxorubicin therapy: miliaria crystallina.

    miliaria is a self-resolving disorder of the eccrine sweat glands caused by blockage of the sweat ducts resulting in the leakage of eccrine sweat into epidermis or dermis. In addition to known predisposing factors such as heat, humidity, and fever, a few drugs (eg, isotretinoin and bethanechol) can also precipitate miliaria. We present here a case of miliaria crystallina that developed in a 65-year-old patient on the fifth day after administration of doxorubicin for multiple myeloma in the absence of known predisposing factors. miliaria crystallina resolved in 3 days without any complications. To our knowledge, this is the first reported case of doxorubicin-induced miliaria crystallina.
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7/27. "Occult" mastocytosis with activating c-kit point mutation evolving into systemic mastocytosis associated with plasma cell myeloma and secondary amyloidosis.

    A case of a 70-year-old man presenting with exsudative enteropathy due to light-chain-associated amyloidosis is reported. The diagnosis of systemic mastocytosis associated with IgG/lambda plasma cell myeloma and secondary generalised amyloidosis was carried out by morphological evaluation of bone marrow biopsy. The c-kit point mutation D816Y was detected by molecular analysis. Two years before, a cystadenolymphoma of the left parotid gland had been removed. A moderate increase of loosely scattered spindle-shaped mast cells, a subpopulation of them expressing CD25, an antigen that is not expressed by normal or reactive mast cells, was shown by retrospective analysis carried out on an intraparotideal lymph node. The c-kit mutation D816Y was shown by the molecular analysis of the lymph node. In summary, the notion that systemic mastocytosis may very rarely be associated with B cell neoplasms and that neoplastic mast cell infiltrates may be obscured because of only a minimal increase of atypical mast cells, which are outnumbered by other non-neoplastic cells in the same tissue, is supported by this case. This finding was preliminarily termed "occult" mastocytosis.
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8/27. Epstein-Barr virus-associated enteritis with multiple ulcers after stem cell transplantation: first histologically confirmed case.

    The present case involves unique enteritis forming multiple ulcers associated with Epstein-Barr virus (EBV). A 57-year-old man had undergone a reduced intensity allogeneic stem cell transplantation for a relapse of multiple myeloma following sequential autologous peripheral blood stem cell transplantation. The ileum, resected for massive melena, showed multiple irregular ulcers with occasional cobblestone-like appearance. There was inflammation including numerous plasma cells in the ulcer bases and surrounding areas, where many EBV-infected plasma cells were detected by double staining with EBV-encoded small rna-1 (EBER-1) in situ hybridization and CD79a, while EBV-infected epithelial cells were not noted. The number of EBER-1-positive cells in the ileum (mucosa, 1451 cells/mm(2); submucosa, 465 cells/mm(2)) was much larger than in control samples (malignant lymphoma or leukemia after allogeneic stem cell transplantation, n = 4, range 0-113 cells/mm(2); malignant lymphoma after chemotherapy, n = 14, range 0-0.89 cells/mm(2); colon cancer, n = 12, range 0-3.5 cells/mm(2)). In the mucosa near the ulcers, EBER-1-positive cells often surrounded and involved the glandular epithelium, forming lymphoepithelial-like lesions. The histological findings differ from post-transplant lymphoproliferative disorders or intestinal thrombotic microangiopathy, and this is the first case of EBV-associated enteritis with ulcers characterized by numerous plasma cells and lymphoepithelial-like lesions after stem cell transplantation.
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9/27. Crystalline light-chain deposition and amyloidosis in the thyroid gland and kidneys of a patient with myeloma.

    A 48-year-old Japanese woman died of multiple myeloma (lambda light-chain type) with chronic renal failure. Histological examination revealed deposition of a homogeneous substance and crystals in the kidneys and thyroid gland. The homogeneous substance was stained with congo red after permanganate treatment but did not stain with antibody to amyloid A protein, and it was recognized as AL-type amyloid. Crystals were not stained with congo red, but crystals were stained with antibody to the lambda light chain. Since AL-type amyloid is considered to be derived from a myeloma light chain, the present case showed two different types of deposition, both of which were derived from the same myeloma protein.
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10/27. Massive systemic amyloidosis associated with light-chain deposition disease.

    A 72-year-old woman presented with rapidly progressive renal failure and multiple myeloma. The patient died 6 months later of severe hepatic insufficiency. The light-microscopic, immunological and ultrastructural findings showed widespread kappa-light-chain deposits including the kidneys, liver, spleen, heart, lungs, tongue, ovary, pancreas and bone marrow associated with massive AL amyloid deposits in the same organs and in the thyroid gland. The concurrent presence of two different deposits is very unusual and the possible mechanisms for such an association are discussed.
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