A report of two cases of Werner's syndrome and review of the literature.

Two cases of Werner's syndrome are reported. The first case is that of a man with grey hair since his 20s, and alopecia since aged about 50 years. At the age of 53 years, Werner's syndrome was diagnosed, along with a malignant soft tissue tumour of the hand. The patient underwent ray amputation for the tumour. The subsequent histopathological diagnosis was synovial cell sarcoma, and the patient died of lung metastasis at 15 weeks postsurgery. The second case is that of a woman diagnosed with diabetes mellitus when aged 34 years. At 39 years, a bilateral cataract was diagnosed and at 40 years, diabetic gangrene of the left calcaneal region and calcaneal osteomyelitis necessitated left below-knee amputation. The incidence of Werner's syndrome in Japan is extremely high (1000 of the around 1300 cases reported worldwide) compared to other countries. Most patients develop malignant tumour or arteriosclerosis, the most important complications of this syndrome. The average life expectancy for patients with Werner's syndrome is 46 years. The incidence of epithelial cancer and mesenchymal sarcoma is 10 times that of the general population. The onset of symptoms of Werner's syndrome generally precedes any later symptoms of associated conditions, such as malignant tumour. Therefore, early recognition of Werner's syndrome is important to assist identification of malignant tumours at an early stage in this patient group.

ABA-specific helper T cells in A/J mice bear the major cross-reactive idiotype.

Attempts were made to induce azobenzenearsonate (ABA)-specific helper cell responses in A/J mice. These were measured by an increase in TNP plaque-forming cells following administration of the double hapten conjugate ABA-bovine serum albumin-TNP. Immunization with ABA coupled homologous immunoglobulin or spleen cells produced ABA-specific help only when the same carrier was used to boost. Hapten-specific help was achieved by two injections of ABA-N-acetyltyrosine in complete Freund's adjuvant 5 weeks apart. This help was passively transferable by T cells as shown by its elimination with anti-Thy 1.2 serum and complement treatment. The presence of the major ABA cross-reactive idiotype (CRI) on these T helper cells could be similarly shown by the elimination of help when the cells were treated with rabbit anti-CRI antibody and complement prior to passive transfer. The same treatment did not effect ABA-specific helper activity of CBA/J mice.

Hapten-specific T cell response to azobenzene-arsonate-N-acetyl-L-tyrosine (ABA-tyr) in Lewis rats. II. Induction and suppression of ABA-specific helper cell activity for antibody production.

Immunization of Lewis rats with azobenzenearsonate-N-acetyl-L-tyrosine (ABA-tyr) in complete Freund's adjuvant (CFA), produces a hapten-specific helper T cell response measured by an increase in plaque forming cells (PFC) against a different hapten. The response seen is primarily direct (IgM) PFC unless B cells are primed by injection of trinitrophenylated keyhole limpet hemocyanin (TNP-KLH) prior to immunization with ABA-tyr. The response requires both ABA and TNP to be on the same carrier molecule which can be as diverse as bovine serum albumin (BSA), poly L-glutamine-lysine-tyrosine (L-GLT); however, a D-amino acid polypeptide does not work. The in vitro demonstration of such help was successful only with peritoneal exudate lymphocytes, not spleen or lymph node cells. Repeated pretreatment of rats by intraperitoneal injection of ABA-tyr in incomplete Freund's adjuvant (IFA) induced an unresponsiveness for helper activity to subsequent immunization with the same antigen in CFA. Passive transfer of lymphoid cells from spleens and lymph nodes from rats pretreated with ABA-tyr in IFA followed by boosting with ABA-tyr in CFA induced unresponsiveness to subsequent induction of hapten-specific help.