Readily available elements were used to build an automatic apparatus dedicated to the preparation of erythrocyte ghosts. The apparatus is designed around a low-cost re-usable hollow-fiber filtration cartridge (marketed for therapeutic plasmapheresis). The apparatus is controlled by a simple programmer (based on a diode matrix and low cost timers and liquid level sensors): once the apparatus is loaded with whole red blood cells, washing of cells, as well as hemolysis and washing of ghosts, is performed by the machine in about 4.5 h without any operator intervention. Automatic filter cleaning takes a further 110 min.

Automation↗

PubMedDate not supplied

Late-onset rod myopathy associated with monoclonal gammopathy.

A 31-yr-old woman presented with a severe and rapidly progressive myopathy affecting proximal limbs, neck flexors and respiratory muscles. Muscle biopsy revealed numerous atrophic fibres with marked structural alterations, without inflammatory infiltrate. By electron microscopy, atrophic fibres displayed many rods. A benign monoclonal gammopathy (IgG, lambda chain) was evident in serum. A sarcolemmal deposit of IgG, lambda chain was found by immunostaining. Plasmapheresis and immunosuppressive therapies produced a decrease in paraproteinemia and a partial clinical improvement. This observation is the third to associate monoclonal gammopathy with "late-onset rod myopathy". The pathogenetic role of paraproteinemia remains unclear.

Adult↗

PubMedDate not supplied

Potential danger of thrombosis after plasma exchange in the treatment of patients with immune disease.

Coagulation studies were serially performed before and after repeated plasma exchange in seven patients with myashtenia gravis. The fall in platelets and coagulation factor activities after plasmapheresis using a replacement material devoid of clotting factors was not so low that bleeding episodes were to be feared and no hemorrhages were observed. In contrast, thrombotic phenomena were suspected in two patients. Antithrombin III fell to undetectable levels in some cases. This finding associated with elevated values of factor VIII related antigen, factor VIII coagulant activity and von Willebrand factor suggested a potential danger of thrombosis in such treated patients.

Adolescent↗

PubMedDate not supplied

[Resistant acquired bullous epidermolysis with severe ocular involvement: the success of extracorporeal photochemotherapy].

BACKGROUND: Epidermolysis bullosa aquisista can leave several functional sequelae. The lesions sometimes resist treatment. CASE REPORT: We report a case of a 25-year-old young man presenting with a severe epidermolysis bullosa acquisita confirmed by the electronic immunomicroscopy. He had a major ocular involvement with symblepharon and cicatricial synechial lesions. He was almost blind because of corneal scars. All immunosuppressive treatments had failed: systemic corticoids, cyclosporin, azathioprine. The introduction of extracorporeal photochemotherapy resulted in the healing of the lesions, after a total of 32 procedures. All treatment are now stopped, and the lesions are purely cicatricial, without any relapse of the disease since 9 months. Corneal grafts are now under process, to try to recover a part of the lost visual acuity. DISCUSSION: This case demonstrates the efficacy of extracorporeal chemotherapy to be tried in case other treatments failed.

Adult↗

PubMedDate not supplied

[Plasma exchange in the treatment of lepromatous leprosy].

Explore the source record for details and available documents.

Adult↗

PubMedDate not supplied

[Cytogenetic studies in four cases of Fanconi's anemia (author's transl)].

In two sibships, four patients with Fanconi's anaemia were studied cytogenetically. Interfamilial variations of the frequency of chromosome breakage and rearrangements were found, suggesting the heterogeneity of the disease. Variations of chromosomal abnormalities have also been found during the course of the disease. An abnormal 47 chromosomes clone has been observed in the bone marrow cells from one patient. A mechanism of somatic segregation is probably involved in the constitution of that clone.

Abnormalities, Multiple↗