An unusual form of primary systemic amyloidosis: amyloid elastosis: report of a case treated by haematopoietic cell transplantation.

Amyloid elastosis is a rare variant of primary systemic amyloidosis characterized by amyloid deposited around elastic fibres. Only two cases, with pseudoxanthoma elasticum-like features and fatal outcome, have been reported. A 56-year-old woman presented with polyneuropathy and a diffuse plane xanthoma-like eruption. Light and electron microscopy studies revealed deposits of amyloid L encasing either normal-looking or short, fragmented elastic fibres in the dermis in a pattern characteristic of amyloid elastosis. The patient had medullary plasmocytosis with lambda light chain restricted expression and underwent autologous stem cell transplantation, which resulted in progressive regression of mucocutaneous signs and stabilization of the polyneuropathy. Our case extends the spectrum of clinical and histopathological presentations of amyloid elastosis. Haematopoietic cell transplantation might improve outcome in patients with multisystem disease.

Vulvodynia after CO2 laser treatment of the female genital mucosa.

We have observed 3 cases of vulvodynia after CO2 laser (pulse or scan) treatment of condylomata acuminata (n = 1) or bowenoid papulosis (n = 2) of the female genital mucosa. Laser treatment was associated with a considerable delay in healing (3-4 months) and chronic pain. The histology of the treated areas showed a scar tissue and severe mucosal atrophy. The occurrence of painful scars following CO2 laser treatment could be related to an inadequate laser technique considering the morphology of the vagina.

Mast cells induce autoantibody-mediated vasculitis syndrome through tumor necrosis factor production upon triggering Fcgamma receptors.

The generation of autoantibodies and deposition of immune complexes (ICs) in tissue play a primary role in autoimmune diseases. However, the IC-triggered response consists of complex mechanisms that make it difficult to identify the pathogenesis and develop specific therapy. We clarified here a sequential mechanism for the induction of hypersensitivity angiitis by analyzing the responsible Fc receptor (FcR), effector cells, and mediators in an animal model using FcR-deficient mice. In this model, rheumatoid factor-mediated skin vasculitis was induced in wild-type mice, whereas FcRgamma-deficient mice did not develop the vasculitis. Adoptive transfer of various FcR(+) cells into FcRgamma-deficient mice showed that mast cells but not macrophages derived from wild-type mice triggered skin vasculitis. Mast cells derived from either FcgammaRIII-deficient or tumor necrosis factor (TNF)-deficient mice did not possess the inducibility of skin vasculitis. These results indicate that triggering of vascular inflammation was induced by mast cells through IC binding on FcgammaRIII. TNF produced by such activated mast cells was mainly responsible for the pathogenesis of autoantibody-mediated vasculitis. These findings illustrate the clinical significance of mast cells, Fcgamma receptors, and TNF in IC-induced vasculitis syndrome.

Rhabdomyolysis after correction of hyponatremia due to psychogenic polydipsia possibly complicated by clozapine.

OBJECTIVE: To report a case of rhabdomyolysis related to rapid correction of hyponatremia attributable to compulsive drinking of water, possible complicated by clozapine use. CASE SUMMARY: A 42-year-old white man treated with clozapine for schizophrenia was admitted for a generalized seizure. Marked hyponatremia due to psychogenic polydipsia was present. He developed a marked elevation of creatine kinase concentrations after correction of hyponatremia with hyperosmolar sodium solution, without clinical signs of rhabdomyolysis. DISCUSSION: Rhabdomyolysis associated with hyponatremia due to water intoxication has been reported in 17 patients to date. A possible explanation may lie within the framework of the calcium-sodium exchange across the skeletal muscle cell membrane. By increasing muscle cell permeability, clozapine treatment may possibly enhance the destruction of muscle cells. CONCLUSIONS: Hyponatremia due to water intoxication and concurrent use of clozapine should be considered in the differential diagnosis of rhabdomyolysis, especially in the severely psychiatrically disabled population.

Treatment of metastatic colorectal cancer by means of specific monoclonal antibodies conjugated with iodine-131: a phase II study.

Eighteen consecutive patients with advanced and/or metastatic colorectal carcinoma have been treated with intraperitoneal administration of radiolabelled (iodine-131) monoclonal antibodies raised against different antigens associated to these kinds of tumours: anti-CEA FO23C5, anti-CEA BW494/32, anti-TAG B72.3, AUA1. The doses of isotope ranged between 21 and 150 mCi (777-5550 MBq) which delivered a radiation dose to the target tumour from 768 to 4628 cGy. Thirteen patients were previously treated with conventional regimens which consisted of chemotherapy (5-fluoracil with or without other anti-neoplastic drugs) both in adjuvant or palliative setting. Three patients are considered non-evaluable owing to concomitant chemotherapy in 2 and lack of objective parameters in 1. Out of 15 evaluable patients 2 achieved complete remission and 2 partial remission with a response rate of 26.6%. Three stable and 8 with progressive disease have also been registered. The toxicity was negligible consisting of hematologic WHO grade 1 in 7 patients, grade 2 in 1 patient and grade 3 in 1 patient, as well as hepatic WHO grade 1 in 8 and grade 2 in 2 patients. The authors conclude that this innovative way of treatment for advanced colorectal carcinoma seems to offer promising therapy; from these data, therefore, a new trial is justified employing radiolabelled MoAbs in well selected patients with metastatic or locally advanced colorectal carcinoma.

Locoregional immunotherapy of human ovarian cancer: preliminary results.

Ten patients with advanced ovarian carcinoma (5 in FIGO stage III and 5 in stage IV), following the failure of repeated chemotherapy courses, were submitted to i.p. radioimmunotherapy (RIT). Several antibodies (HMFG1, HMFG2, H17E2, B72.3) were employed. The antibody choice was made on the basis both of immunohistochemistry and immunoscintigraphy results. The mean dose administered in each single course was 17 mg, 3 patients underwent two treatments and 2 patients were injected three times. The isotope employed was 131I (mean dose 92 mCi). The early or late untoward effects were negligible. All patients developed anti-mouse antibodies (HAMA) which reduced, during the further RIT, the MoAbs' effective half-life. The estimated radiation dose delivered to the tumour ranged from 2910 to 11,000 cGy. Both wholebody and liver radiation burden resulted in tolerable limits while the thyroid irradiation was high. In 3 patients the RIT result was ineffective; in 2 cases a temporary ascitis staunch was obtained. In 2 cases the tumour growth was slower than expected and in 3 patients instrumental evidence of partial tumour reduction, still lasting after 4-11 months, was observed.

Ultrastructural study of gonads in the complete and incomplete feminization syndrome.

The gonads of two individuals with complete and one with incomplete testicular feminization were examined by light and electron microscopy. In the seminiferous tubules of the complete forms, clear, intermediate, and dark-type Sertoli cells could be distinguished. These are immature elements which had assumed, under the influence of hormones, different morphology. Leydig cells were also immature, at varying stages of differentiation. In the complete form Sertoli and Leydig cells had an almost normal fine structure; the lamina basalis of the tubules, however, was considerably thickened.