Showing papers on "Primary systemic amyloidosis published in 1991"

Cranial neuropathy associated with primary amyloidosis

Abstract: We report 6 patients for whom cranial neuropathy was a major manifestation of primary amyloidosis. In 3 of the 6, multiple cranial nerves were involved. All had tissue biopsy documentation of amyloidosis. In 2, nerve biopsy also confirmed amyloidosis. All had renal involvement manifested by proteinuria. Primary systemic amyloidosis must be considered in the differential diagnosis of cranial neuropathy, especially when proteinuria is present.

Computed tomography for diagnosis of hepatic rupture in primary systemic amyloidosis.

Abstract: Hepatic rupture in primary systemic amyloidosis is extremely ominous; death occurs in hours to days. We present two cases of hepatic rupture diagnosed ante mortem with computed tomography (CT). Surgical repair was possible in one instance and successfully controlled the bleeding. In the setting of primary amyloidosis, the early use of CT scanning for undiagnosed ascites, hypotension, or rapid organ enlargement may lead to early diagnosis and potential surgical correction.

Nail changes as the first sign of systemic amyloidosis.

Abstract: A 68-year-old man had had for 3 years a severe onychodystrophy involving all fingers and toes which clinically mimicked nail lichen planus. The nail biopsy showed amyloid deposits in the superficial dermis of the nail matrix. Physical, pathological and laboratory examinations confirmed the diagnosis of primary systemic amyloidosis. At the time of the diagnosis the patient did not present any other cutaneous sign of systemic amyloidosis.

Primary systemic amyloidosis causing diffuse alopecia by telogen arrest

Abstract: To the Editor.— The primary and myeloma-associated forms of systemic amyloidosis are frequently associated with cutaneous manifestations, ie, purpuric lesions due to amyloid in vessel walls, or smooth waxy papules, plaques, and nodules due to dermal amyloid deposits. 1,2 Alopecia is less common but may occur as part of the clinical spectrum of established disease or, rarely, as a sign of occult amyloidosis. 3,4 We report a patient whose diffuse alopecia led to the diagnosis of primary systemic amyloidosis. The histopathologic changes in serial biopsy specimens demonstrate that amyloid inhibits anagen restoration of hair follicles, perhaps by vascular impairment. Report of a Case.— A 59-year-old, asymptomatic, white woman presented with a 4-month history of diffuse scalp alopecia (Fig 1), generalized thinning of body hair, and easy bruisability. Physical examination revealed a greater than 50% reduction in scalp hair density, positive hair pulls, increased hair part widths, and microscopically normal hair

Speech disorders in systemic amyloidosis.

Abstract: The purpose of this study was to define more completely the speech disorders that can occur in systemic amyloidosis. Fifty-nine patients with histologically established primary systemic amyloidosis were studied. Of this group, 81 % had abnormal voice quality, 43% had abnormal pitch, 7% had abnormal resonation and 15% had abnormal articulation. Twenty-two patients also had abnormal findings at laryngeal examination, consisting of bowing, oedema, thickening, dry irritation and polypoid changes of the vocal folds. Macroglossia was found in 17% of the patients and submandibular swelling in 10%. These results support previous studies which showed that abnormal phonation is a sign of systemic amyloidosis and that abnormal voice is by far the most common abnormal speech sign. The relationship between amyloid deposit and dysphonia was not clear. In 23 patients who had laryngeal examinations, no evidence of actual amyloid deposits was documented.Le but de cette etude etait de definir plus completement les troubles...

Incidence and Epidemiology of Primary Systemic Amyloidosis (AL) in Olmsted County, Minnesota: 1950 Through 1989

Abstract: We are unaware of any reported studies of the incidence or prevalence of AL in the US or other countries. The absence of epidemiologic data is probably indicative of the rarity of AL and the diagnostic difficulties in distinguishing AL from secondary amyloidosis (AA), familial amyloidosis (AF), and senile systemic amyloidosis (ASC1). It is difficult to assess the incidence rates over time for AL because of changing criteria of diagnosis, changes in clinical practice, variations in methods of diagnostic indexing of medical records, and differing autopsy rates. Olmsted County, Minnesota provides a rare opportunity to minimize these limitations because medical care for the population of Rochester, Minnesota and the surrounding Olmsted County has been provided primarily by the Mayo Clinic. Virtually all diagnoses made for a significant illness among Olmsted County residents have been compiled in a centralized records-linkage system. The relative stability of the local population, the unusual centralization of high-quality medical care, the high rate of autopsy, and the diagnostic indexing and records-linkage system used for many decades at Mayo Clinic provides an exceptional source for studies of incidence rates and long-term trends in this population.

Primary Systemic Amyloidosis (AL) in 1990.

Abstract: Amyloidosis was described over 300 years ago, but it was not until 1856 that Wilks described a 51-year-old man with “lardaceous viscera” in whom the changes were unrelated to syphilis, osteomyelitis, other osseous disease, or tuberculosis. The patient had dropsy and albumin in his urine. At autopsy the heart was enlarged and the spleen was hard and lardaceous. Similar changes were found in the kidney. This patient probably had primary amyloidosis (AL).

Primary Systemic Amyloidosis (AL): A Randomized Trial of Colchicine vs. Melphalan And Prednisone vs. Melphalan, Prednisone, and Colchicine.

Abstract: Primary amyloidosis is an uncommon disease characterized by deposition of a fibrillar protein often in the heart and kidney resulting in organ dysfunction and death. Treatment for AL is unsatisfactory. The amyloid fibrils consist of the variable portion of monoclonal immunoglobulin light chains and are synthesized by plasma cells. Increased numbers of plasma cells are commonly found in the bone marrow of patients with AL. It is reasonable to attempt treatment with alkylating agents which are known to be effective against plasma cell proliferative processes such as multiple myeloma. We are engaged in a prospective randomized study in which patients have been assigned to melphalan and prednisone or to colchicine or to melphalan, prednisone, and colchicine. This interim analysis suggests the two melphalan-prednisone containing regimens are superior to colchicine.

A case of primary systemic amyloidosis with AA protein.

Abstract: We present here a case of young adult-onset primary systemic amyloidosis with AA protein. A 29-year-old male presenting with macrohematuria was transferred to our hospital because of aggravation of renal function. Amyloid protein was detected in the bladder, stomach and thyroid. The amyloid protein proved to be AA type by potassium permanganate histochemical analysis. Since secondary amyloidosis was excluded by the laboratory data and there was no family history of amyloidosis, a diagnosis of primary systemic amyloidosis with AA protein was reached. Although the patient was prescribed dimethyl sulphoxide, his renal function worsened with gastrocolic symptoms and a bleeding tendency. Hemodialysis (HD) was then initiated. After starting the HD, the patient's general condition recovered and subsequently the patient on treatment with maintenance HD was discharged.

Case Definition: Issues Related to Monoclonal Gammopathies and Multiple Myeloma

Abstract: During 1988, 808 patients were found with a serum monoclonal protein (M-protein) at the Mayo Clinic. The most frequent clinical diagnosis was monoclonal gammopathy of undetermined significance (MGUS) (benign monoclonal gammopathy), 64%. Other diagnoses were multiple myeloma (MM), 14%; primary systemic amyloidosis (AL), 8%; lymphoproliterative disease including lymphoma and macroglobulinemia, 8%; chronic lymphocytic leukemia, 2%; smoldering multiple myeloma (SMM), 2%; and solitary or extramedullary plasmacytoma, 2%.

Urinary Protein Patterns Predict Survival in Primary Systemic Amyloidosis (AL)

Abstract: Serum creatinine level was measured in 153 patients with primary systemic amyloidosis (AL). The median survival of patients with a serum creatinine ≤1.3 mg/dL and >1.3 mg/dL was 25.6 and 14.9 months, respectively (P = 0.007). Patients with a monoclonal lambda light chain in the urine had an inferior survival compared to those with kappa light chain or no monoclonal protein in the urine. The survivals were 12 months, 30 months, and 35 months, respectively (P = 0.01). The total protein excretion had no bearing on survival. Patients with lambda monoclonal protein in the urine excreted 4.56 g of protein per day. Patients with no monoclonal protein and a urinary kappa monoclonal protein excreted 0.52 g/day and 1.09 g/day, respectively. The loss of monoclonal lambda protein in the urine did not result in a higher serum creatinine level. A monoclonal protein was detected in the urine of three-fourths of the patients. The urinary kappa-to-lambda ratio was 1:2.5, but for those patients with nephrotic range proteinuria, the ratio was 1:4.7, suggesting that patients with lambda monoclonal protein in the urine are more likely to have amyloid nephrotic syndrome than patients with no monoclonal protein or kappa monoclonal protein in the urine.