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In 153 patients with biopsy-proven primary systemic amyloidosis (AL), serum creatinine level correlated with median survival (creatinine less than or equal to 115 mumol/L [1.3 mg/dL], 2.56 months; creatinine greater than 115 mumol/L [1.3 mg/dL], 14.9 months; P = 0.007). The 24-hour urine total protein excretion was not correlated with survival. Patients who excreted monoclonal lambda light chains in the urine had an inferior survival (12 months) compared with those with kappa (30 months) or no monoclonal protein (35 months) in the urine (P = 0.01). The presence of lambda monoclonal protein was not associated with a higher median creatinine level. A monoclonal protein was detected in the urine in 76% of patients; when patients with more than 1 g of protein in the urine were studied, however, a monoclonal protein was detectable in 86%. The overall kappa-lambda ratio was 1:2.56, but as the urinary protein loss increased, the kappa-lambda ratio shifted to 1:4.7 for those with more than 3 g of protein in the urine.

A 5-year-old boy known to have tuberous sclerosis was admitted with an abdominal aortic aneurysm. The aneurysm was resected and replaced by a bifurcation prosthesis. Follow-up, which amounts up to 4 years, has been uneventful. Prompt recognition and surgical treatment are essential in the management of this rare but potentially lethal condition.

Thymic biopsies from two patients with combined immunodeficiency and defective expression of HLA class I and class II antigens on blood mononuclear cells (“bare lymphocyte” syndrome) were investigated. This made possible an evaluation of the significance of HLA antigen expression in a detailed (immuno)histologic study. Both thymuses showed a normal lobular architecture with distinct cortex-medulla areas, well-differentiated epithelium, including ultrastructurally defined subtypes, and Hassall's corpuscles. Normal numbers of lymphoid cells were present and normal T-cell phenotype was found. Using anti-HLA-A,B,C antisera, confluent staining of the medulla (stroma and lymphocytes) was observed. One of the thymuses was found to be negative for HLA class II antigen expression: the other revealed only HLA-DR positivity of nonlymphoid cells in the medulla. These cells were not of epithelial nature as judged from double staining with anti-keratin antibody. There was no expression of HLA-DC/DS. These observations differ from findings in the normal thymus, wherein epithelial cells in the cortex carry HLA class I and class II antigens, and epithelial cells in the medulla express HLA class I, and for a minor part class II antigens. The results indicate a normal sequential acquisition of T-cell differentiation antigens in the thymus of both cases. It is suggested that the expression of HLA class I and class II antigens on epithelial cells in the normal thymus cortex does not play a significant role in the sequential acquisition of differentiation antigens on T lymphocytes.