Cases reported "Multiple Myeloma"

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1/14. IgA-kappa type multiple myeloma affecting proximal and distal renal tubules.

    A 45-year-old male was admitted because of chest pain, lumbago, and bilateral ankle pain. Examination disclosed hypophosphatemic osteomalacia, acquired fanconi syndrome, and abnormalities in distal nephron such as distal renal tubular acidosis and renal diabetes insipidus. Further exploration revealed IgA kappa multiple myeloma excreting urinary bence jones protein (kappa-light chain). Renal biopsy revealed thick basement membranes and elec-tron-dense crystals in proximal tubular epithelial cells. Immunofluorescent studies revealed deposition of kappa-light chain in renal tubular epithelial cells that caused the renal tubular damage. Although the osteomalacia was relieved by medical treatment, the urinary bence jones protein and the renal tubular defects were not improved by the chemotherapy for the myeloma. The patient died of exacerbation of multiple myeloma at 50 years of age.
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2/14. Crystal nephropathy: a variant form of myeloma kidney--a case report and review of the literature.

    Cast nephropathy is the most common form of myeloma kidney, and also one of the major determinants of a patient's prognosis. We experienced a case of multiple myeloma with acute renal failure that was associated with massive crystal deposition in kidney and bone marrow. Large crystal formation in myeloma kidney is a rare pathological finding in renal biopsies. In the literature, most of the cases with multiple myeloma with extracellular crystal deposition often have a rapidly progressive course with a poor prognosis. Ball et al. [1993] suggested that this type of extracellular crystal deposition be designated as "crystalglobulinemia syndrome". The characteristics of the clinical and histopathological features, rapid progression and poor outcome, and crystal formation in the tubules found in the present case and in those previously reported, support the proposal of a new concept of "crystal nephropathy".
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3/14. lung osteoma--a new benign lung lesion.

    Extraskeletal osteomas have not been described in the lung. Tumors with osseous elements can be found, such as hamartoma and amyloid tumor, and reactive lesions such as osseous metaplasia. A 39-year-old male patient was treated for multiple myeloma and got a bone marrow transplantation 2 years and a few months before he presented with a solitary well-circumscribed tumor in the right middle lobe. The patient underwent surgical resection. The tumor presented with a fibrous capsule and consisted of mature bone trabecules. Within the tumor, fatty tissue was seen. There were small bone spicules interpreted as areas of new bone formation and appositional growth. No amyloid deposition, no immature epithelial tubules as in hamartomas, and no normal lung structure as in osseous metaplasia were seen. Within the osseous elements, a positive reaction was seen with antibodies for osteonectin, whereas the reaction for calcitonin was negative. To the best of our knowledge, this is the first case of an osteoma being reported in the lung looking like any other extraskeletal osteoma. This tumor might have been induced by circulating stem cells; however, due to autologous bona marrow transplantation, this cannot be proven.
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4/14. Cytoplasmic crystals in multiple myeloma-associated Fanconi's syndrome. A morphological study including immunoelectron microscopy.

    We report a case of Fanconi's syndrome associated with multiple myeloma, which displayed some unusual features. Although serum immunoelectrophoresis showed no spike, urine electrophoresis revealed monoclonal kappa light chain. The myeloma cells in multiple organs including bone marrow, lymph nodes, spleen, and kidneys were distended with characteristic intracytoplasmic crystals. In the kidneys, identical intracytoplasmic crystals were found in some proximal tubules, distal tubules, collecting ducts, glomerular cells (mostly parietal epithelial and endocapillary cells), and renal interstitial cells. Only monoclonal kappa light-chain protein was demonstrated in these crystals by immunofluorescence and immunoperoxidase techniques, a finding confirmed for the first time (to our knowledge) by immunoelectron microscopic study of the renal biopsy specimens.
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5/14. Crystalline glomerular inclusions in multiple myeloma.

    Prominent crystalline inclusions were noted in the glomeruli of a 57-year-old man with a 6-month history of swelling and pain in the hands, slight proteinuria, and elevated serum creatinine and BUN. The crystalline inclusions were most prominent in the visceral epithelium but were also noted in endothelial and mesangial cells, in the parietal epithelium, and in the epithelial cells of the proximal tubules. On electron microscopy the crystalline structures were of various geometric shapes but had no definite substructure. Immunofluorescence was negative. The patient was considered to have a hitherto undescribed metabolic disease. Several months later the patient underwent an operation for carpal tunnel syndrome and amyloid deposits, crystalline inclusions similar to those noted in the kidney were observed in the synovial tissue. Shortly after this the patient was found to have multiple myeloma of IgG, kappa type with Bence Jones proteinuria. Lymphoplasmacytic cells in the bone marrow contained the same crystalline inclusions noted in the kidney and synovium. This case therefore seems to represent a new and very rare form of glomerular involvement in multiple myeloma.
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6/14. Systemic kappa light chain deposition and amyloidosis in multiple myeloma: novel morphological observations.

    light and transmission electron microscopic studies as well as immunohistochemical investigations were performed on a case of multiple myeloma in a 40-year-old female with systemic kappa light chain deposition. The latter took the form of extensive deposits in the liver, spleen and bone marrow. Amyloid was not found in these deposits, although it was present in the wall of branches of the portal vein. Conversely, amyloid but not light chain deposition was found in the tongue, synovial membrane from the knee and myocardium. The kidney was the only organ to show both amyloid and light chain deposits intimately associated with each other in and around tubules and collecting ducts. Ultrastructurally the light chain deposits were seen to be biphasic, having two granular components of different electron density. In the spleen and bone marrow, but not in the liver, the kappa light chain deposits were surrounded by multinucleated giant cells. This is the first report of light chain deposition disease associated with a foreign body type of giant cell reaction. The morphological variability of organ involvement suggests that the molecular structure and conformation of the deposited light chains are heterogeneous and may reflect a broad spectrum of metabolism of these deposits in various organs.
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7/14. adult fanconi syndrome in kappa light chain myeloma.

    Distinctive morphological features in both the marrow infiltrate and the kidney were seen in a 52-year-old woman with kappa light chain-producing plasma cell myeloma, diagnosed on the basis of multiple osteolytic lesions, the presence of atypical plasma cells in the bone marrow, and monoclonal immunoglobulin production as demonstrated by immunoperoxidase staining on marrow sections. Large focal collections of histiocytes in the bone marrow and the renal proximal tubular epithelium had abundant glassy cytoplasm. Characteristic crystalline inclusions were seen ultrastructurally in both types of cells. It is believed that these crystalline deposits are lysosomal inclusions composed of altered kappa light chains taken up by these cells. The renal changes were entirely different from those of myeloma kidneys and were associated with proximal tubular dysfunction of adult fanconi syndrome without distal tubule abnormality.
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8/14. Myeloma-like cast nephropathy associated with acinar cell carcinoma of the pancreas.

    Obstruction of renal collecting tubules by protein cats inciting a giant cell reaction is usually characteristic of myeloma cast nephropathy. Rarely other causes of proteinuria may result in a similar morphology. We report a rare case of 'myeloma-like' tubular casts in the kidney of a patient who was subsequently found at autopsy to have acinar cell carcinoma of the pancreas with peritoneal carcinomatosis. Only two similar reports could be found in the English literature.
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9/14. The macrophagic origin of multinucleated giant cells in myeloma kidney: an immunohistologic study.

    The cast-engulfing giant cells in a case of kappa light chain nephropathy/Bence Jones cast nephropathy were characterized with monoclonal antibodies Uro-5 (renal epithelium: Henle's loop, distal tubule, collecting duct) and Leu-M5 (macrophage/monocyte/dendritic cell specific). The giant cells were Leu-M5 positive and Uro-5 negative. There was an increased concentration of Leu-M5-positive cells surrounding cast-containing tubules, and occasional Leu-M5-positive cells were found within the tubular epithelial layer. Although there were no Uro-5-positive giant cells, an occasional Uro-5-positive cell was found within casts. The majority of casts were found within Uro-5-positive tubules. These results confirm earlier electron microscopic reports that the cell of origin of the cast-engulfing multinucleated giant cell is the macrophage (Leu-M5 positive, Uro-5 negative). These giant cells may originate from macrophages that have migrated from the interstitium through the tubular epithelium.
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10/14. Widespread crystallisation of paraprotein in myelomatosis.

    Two patients with myelomatosis leading to acute renal failure were found at autopsy to have extensive crystalline deposits of paraprotein within the capsular space of the glomeruli, renal tubules and renal blood vessels. In one case, crystalline deposits in the renal arteries had caused infarction of both kidneys. Both patients had extensive paraprotein deposits in the heart and in other tissues, leading to myocardial infarction in one case. This patient also had extensive pulmonary deposits of paraprotein without a local plasma cell infiltrate, an appearance which has not been described before. Crystalline deposition does not appear to be specifically associated with a single class of paraprotein. This type of disease, affecting many organs, may be more common than has generally been appreciated and should be considered as a cause of otherwise unexplained organ failure in patients with myelomatosis.
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