Cases reported "Amyloidosis"

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1/187. Tc-99m HDP uptake in cardiac amyloidosis.

    amyloidosis is characterized by the soft-tissue deposition of amyloid protein. It may occur as a primary disorder but more often is seen as a manifestation of chronic illness. Scattered reports of the affinity of amyloid for bone scanning agents have appeared over many years. Isolated cardiac uptake of Tc-99m HDP is described in a patient with biopsy-proved cardiac amyloidosis on a background of tuberculosis, prostate cancer, and coronary artery disease.
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2/187. familial mediterranean fever. No role of mycobacterium tuberculosis in ten patients.

    BACKGROUND: Tuberculosis (TB) and familial mediterranean fever (FMF) are two common diseases in our region, turkey. Both share some properties in common: Both cause AA type amyloidosis and have association with some immunological abnormalities. Upon incidentally observing mycobacterium tuberculosis in bone marrow biopsies of three patients with FMF in a previous study, we intended to elucidate this association prospectively. MATERIAL AND methods: In this study, we examined prospectively 10 FMF patients, 5 male and 5 female, with a median duration of 31 years disease activity. All were under colchicine therapy. They had no sign of renal involvement. The bone marrow biopsies of these patients were examined for the presence of M. tuberculosis by polymerase chain reaction (PCR), BACTEC culture and pathological stains. Pathological examination was performed for the existence of granuloma and amyloid deposition by hematoxylin-eosin, Crystal Violet and congo red stains. RESULTS: The examination of all bone marrow specimens by the mentioned methods suggest that mycobacterium tuberculosis has no role in the ethiopathogenesis of FMF. Although the patients had a positive family history of 60% for tuberculosis and in 80% of them with positive tuberculin skin test. CONCLUSIONS: We concluded that although there seemed to be a kind of association between both diseases, this relationship is not via the direct existence of bacteria itself. Considering high family history and skin test positivity, one should look for the presence of autoimmune mechanisms under this suspicious relationship between tuberculosis and FMF. Also, this is the first study examined the state of amyloidosis in the bone marrow at an earlier stage of FMF without overt renal findings.
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3/187. amyloidosis: recognition, confirmation, prognosis, and therapy.

    amyloidosis should be considered in any patient older than 40 years who has nephrotic syndrome, congestive heart failure (not on an ischemic basis), idiopathic peripheral neuropathy, or unexplained hepatomegaly. When a patient has one of these problems, immunoelectrophoresis and immunofixation of the serum and urine should be done for the detection of a monoclonal light chain. If a monoclonal light chain is found, a diagnosis usually can be established by amyloid stains performed on a bone marrow biopsy specimen or a subcutaneous fat aspirate. The presence or absence of cardiac involvement with amyloid is the most important prognostic factor. Treatment can range from observation to oral chemotherapy to hematopoietic stem cell transplantation. A practical understanding of the mechanisms underlying this disease can lead to prompt diagnosis and early therapeutic intervention.
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4/187. Rapid reversal of nephrotic syndrome due to primary systemic AL amyloidosis after VAD and subsequent high-dose chemotherapy with autologous stem cell support.

    In a patient with nephrotic syndrome, renal biopsy revealed AL amyloid deposits. Monoclonal lambda light chains were identified in serum and urine. A low percentage of monoclonal plasma cells was detected in the bone marrow. The patient received four cycles of VAD and subsequent high-dose chemotherapy (HDCT) with melphalan (200 mg/m2) followed by autologous peripheral blood stem cell transplantation. proteinuria rapidly diminished during chemotherapy. Three months after HDCT, the patient has no edema, and no signs of plasma cell dyscrasia are currently detectable. Using VAD before starting HDCT may improve the condition of patients with amyloidosis and reduce transplantation-related morbidity and mortality.
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5/187. Improvement of amyloid-related symptoms after autologous stem cell transplantation in a patient with hepatomegaly, macroglossia and purpura.

    AL amyloidosis was diagnosed in a 56-year-old woman with spontaneous purpura, macroglossia and hepatomegaly, a serum IgGk monoclonal gammopathy and a 25% plasma cell bone marrow infiltration. She was started on high-dose treatment consisting of four monthly cycles of VID chemotherapy, then underwent a stem cell collection after priming with cyclophosphamide G-CSF. Myeloablative therapy was with melphalan and busulfan. Hematologic recovery was fast and uncomplicated. At follow-up 22 months from ASCT, the patient shows a complete remission of the clonal plasma cell disorder, normalization of liver size and alkaline phosphatase level and a significant improvement in the signs of vascular and soft tissue amyloid infiltration.
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6/187. Bone amyloidoma in a diabetic patient with morbid obesity.

    Bone localisations of amyloidosis are rare, usually diffuse and associated with myeloma. We report the case of a patient with massive obesity complicated by diabetes, hypertension, sleep apnea and liver steatosis, who complained of rapidly worsening bilateral polyradiculalgia of the lower limbs. After sufficient weight loss made nuclear magnetic resonance imaging feasible, a spinal tumour was visualised on the 5th lumbar vertebra, extending to soft tissues. Total excision was performed, and pathological studies revealed an amyloid bone tumour with no evidence of myeloma.
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7/187. Acute leukemia following prolonged cytotoxic agent therapy.

    1. Nine patients in whom acute non-lymphoblastic leukemia (ANLL) developed following prolonged alkylating agent therapy are described. Five of the patients received no radiotherapy. The conditions treated were: Hodgkin's disease (four patients), primary amyloidosis, primary macroglobulinemia, malignant lymphoma, multiple myeloma, and carcinoma of the tonsil. 2. Prior to the advent of chemotherapy, this complication was not observed in large series of patients with lymphoproliferative disorders and multiple myeloma. However, the medical literature now contains at least 125 other detailed reports of ANLL developing after prolonged cytotoxic agent therapy. 3. multiple myeloma and Hodgkin's disease, both of which commonly have good responses to chemotherapy, predominate as the underlying diseases. However, 35% of the case reports involve patients with other illnesses, including 12 patients who did not have neoplasms. 4. More than half of the patients developing ANLL have received chemotherapy alone without radiotherapy. 5. At least half of the patients developing ANLL experienced long periods of significant cytopenia during therapy, often with documentation of bone marrow dysplasia. 6. The wide variety of drugs associated with this complication suggests that any cytotoxic agent may be leukemogenic. However, alkylating agents overwhelmingly predominate as the class of compounds which are most often associated with terminal ANLL. 7. The vast majority of patients reported in the literature with ANLL complicating underlying malignancies have received cytotoxic drugs for prolonged periods (median 3 1/2 years) and leukemia developed most commonly 3 to 5 years after the diagnosis of the underlying disease. Most of these patients benefited from therapy and survived longer (median 5 years) than historical control of untreated patients. 8. The leukemogenic potential in man of prolonged cytotoxic agents therapy, especially with alkylating agents, seems to be well established. This evidence admonishes against the prolonged use of these drugs in non-fatal disorders. 9. More accurate assessment of risk: benefit ratios awaits the results of prospective controlled studies. The results of these studies could also lead to significant modifications in recommendations for long-term maintenance therapy with cytotoxic agents.
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8/187. A case of hereditary amyloidosis transthyretin variant Met 30 with amyloid cardiomyopathy, less polyneuropathy, and the presence of giant cells.

    Transthyretin-Met 30 (TTR-Met 30) is a variant of transthyretin and is usually associated with familial amyloid polyneuropathy. It is rare that patients with TTR-Met 30 will primarily develop amyloid cardiomyopathy. This report presents a patient with late-onset TTR-Met 30 who primarily developed amyloid cardiomyopathy, with less amyloid polyneuropathy in the peripheral nervous system than is usually seen. An autopsy was performed, and histological examination revealed many foreign-body giant cells and macrophages in the area of amyloid deposition that was found in nearly all of the organs.
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9/187. Systemic AL amyloidosis in gaucher disease. A case report and review of the literature.

    Chronic gaucher disease [GD] in association with systemic AL amyloidosis is extremely rare. We describe a 46-year-old Greek male with chronic GD confirmed by low glucocerebroside activity in fibroblasts and N370S/L444P mutations at the cerebrosidase gene, who also had systemic AL amyloidosis diagnosed by bone marrow diffuse plasmacytosis, serum monoclonal IgA-lambda, severe total proteinuria with monoclonal IgA-lambda, Bence-Jones-lambda and amyloid deposits in bone marrow, liver, spleen and kidney biopsy specimens. Treatment with melphalan and prednizolon has dramatically decreased both levels of serum M-IgA and proteinuria and also improved the clinical symptoms of amyloidosis. He died from restrictive cardiac disease 30 months after the diagnosis of amyloidosis. Previously reported cases of GD in association with AL amyloidosis are reviewed.
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10/187. Myeloma-associated systemic amyloidosis presenting as chronic paronychia and palmodigital erythematous swelling and induration of the hands.

    Mucocutaneous involvement occurs predominantly in primary systemic amyloidosis as well as in myeloma-associated systemic amyloidosis. It is rarely observed in other types of amyloidoses. Signs of such involvement may aid in the early diagnosis of the disease process. Herein, we describe a 64-year-old white male patient with myeloma-associated systemic amyloidosis in whom the disease presented with unique cutaneous lesions consisting of chronic paronychia and palmodigital erythematous swelling and induration of the hands. Following weekly regimens with prednisone (20 mg/day) and melphalan (2 mg/day) administered every 16 weeks, almost complete resolution of the cutaneous lesions was observed after 1 year of therapy. Also, in response to chemotherapy, modest regression of the myelomatous bone lesions and complete resolution of the underlying gammopathy occurred.
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