Mediastinal and Neck Kaposiform Hemangioendothelioma: Report of Three Cases

Abstract:   Kaposiform hemangioendothelioma is an aggressive vascular tumor, named for its striking histologic resemblance to Kaposi sarcoma and locally invasive growth. Mortality is high, and ranges from 10% to 24% for all kaposiform hemangioendothelioma lesions, with a significantly higher mortality for deep soft-tissue or visceral lesions occurring in infants less than 6 months. Mediastinal and neck kaposiform hemangioendothelioma in particular merit special discussion, as involvement of these critical anatomic locations results in significant site-specific therapeutic challenges due to invasion of vital structures, inherent delays in establishing histopathologic confirmation, and difficulties in monitoring disease status. We report our experience with three cases of mediastinal and neck kaposiform hemangioendothelioma, emphasizing the unique diagnostic and management challenges, variable response to treatment and outcome of this anatomic variant of kaposiform hemangioendothelioma.     

The regulation of allergy and asthma

Summary: Allergic diseases and asthma are caused by exaggerated T‐helper 2 (Th2)‐biased immune responses in genetically susceptible individuals. Tolerance to allergens is a mechanism that normally prevents such responses, but the specific immunological events that mediate tolerance in this setting are poorly understood. A number of recent studies indicate that regulatory T cells (Tregs) play an important role in controlling such Th2‐biased responses. Tregs involved in regulating allergy and asthma consist of a family of related types of T cells, including natural CD25⁺ Tregs as well as inducible forms of antigen‐specific adaptive Tregs. Impaired expansion of natural and/or adaptive Tregs is hypothesized to lead to the development of allergy and asthma, and treatment to induce allergen‐specific Tregs could provide curative therapies for these problems.     

New design and development of a manual wheelchair for India

Purpose. The most common methods of delivering assistive technology in developing countries are charitable donation and workshops. This describes a new approach to solving the problem, a collaboration undertaken by a US-based lab and a manufacturer in India to produce quality wheelchairs. One goal is to publicize the design free of charge to manufacturers and interested parties world-wide. The process, a demonstration of a new technology transfer method, and the product, an adult manual wheelchair, are described.

Method. An iterative process occurred over four years to design and produce the wheelchair. This consisted of prototypes, small production runs, ANSI/RESNA testing, hardness and tensile testing and informal user testing.

Results. The design is a manual folding cross-brace design with several points of adjustability. Final pre-production prototypes experienced fastener failures during durability testing. Higher grade bolts were specified. Trial-run production has begun. An ANSI/RESNA wheelchair test lab was constructed in India. Subsequent projects include power and pediatric tilt-in-space wheelchairs.

Conclusions. The approach seems promising as a method to improve the quality of assistive technology available in India and begin to meet the vast need in India. Pitfalls encountered throughout the collaboration are described in this paper along with solutions to remedy these problems for future projects.     

In vivo demyelination by antimyelin antibodies

Summary Central nervous system (CNS) myelinspecific antiserum was capable of initiating primary demyelination within 24 h following injection into the dorsal column of guinea pig spinal cord. Control serum injected in the same manner did not produce demyelination. The demyelinating lesions occurred as local linear plaques of completely denuded intact axons surrounded by partially demyelinated and myelinated normal axons. Antiserum-mediated demyelination was followed by mononuclear cell infiltration 7–10 days later. Ultrastructural examination revealed vesiculation of myelin followed by cleavage of myelin lamellae at the intraperiod line. Remyelination began between 7 and 10 days following injection and correlated well with clinical evidence of recovery. The results of this study point to the importance of circulating autimyelin antibodies in the pathogenesis of demyelinating encephalitis. The model represents an in vivo approach to the study of the pathogenesis of immune-mediated myelinolysis in demyelinating disorders like multiple sclerosis (MS), subacute sclerosing panencephalitis (SSPE), and canine distemper encephalitis (CDE).Supported in part by the State of Ohio Canine Research Funds and by Grant 2R01-A1-09022, Allergy and Infectious Disease     

The human leucocyte-common (LC) molecule: dissection of leukaemias using monoclonal antibodies directed against framework and restricted antigenic determinants

Monoclonal antibodies have previously been raised against two separate antigenic determinants on the human LC molecule. One, F10.89.4, recognizes a 'framework' epitope on all LC molecules; these are found on the majority of leucocytes. The other, F8.11.13, recognizes only a 'restricted' epitope present on a subset of these molecules; this subset is found on B lymphocytes and a subpopulation of T lymphocytes. LC molecules on myeloid cells do not carry the 'restricted' antigenic determinant. We have investigated the differential expression of these LC epitopes on human leukaemias, using immunofluorescence on fresh leukaemic blasts and established cell lines. Our study shows that, as on normal haemopoietic cells, LC molecules on B leukaemias bear both 'framework' and 'restricted' epitopes, while the majority of T leukaemias bear only the 'framework' determinant. The small proportion of T cells that are F8.11.13+ ('restricted' epitope) are relatively mature, being of either OKT4+ or OKT8+ phenotype, and may be in an activated state (HLA-DR+). However, in contrast to normal haemopoietic cells, some myeloid leukaemias carry both 'framework' and 'restricted' epitopes (30% AML and AMML samples are F10.89.4+, F8.11.13+), and it is within this group that all TdT+ AML and AMML cases lie. Thus, these monoclonal antibodies should be useful for studying haemopoiesis in man and for analyzing human haemopoietic malignancies.