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Sézary syndrome 650 Shwartzman (or Shwartzman–Sanarelli) reaction<br />

Sézary syndrome<br />

A disease that occurs in middle age, affecting males more<br />

commonly than females. It is a neoplasm (malignant lymphoma<br />

of CD4 + T helper lymphocytes with prominent skin<br />

involvement). Generalized erythroderma, hyperpigmentation,<br />

and exfoliation are observed. Fissuring and scaling<br />

of the skin on the palms of the hands and soles of the feet<br />

may occur. The peripheral blood and lymph nodes contain<br />

the typical cerebriform cells that have nuclei that resemble<br />

the brain. Infiltration of the skin by leukocytes is extensive,<br />

with prominent clustering in the epidermis forming<br />

Pautrier’s abscesses. Late in the disease, T immunoblasts<br />

may appear. The so-called Sézary cells are T lymphocytes.<br />

This syndrome is an advanced type of mycosis fungoides.<br />

SH2 domain<br />

Refer to Src family tyrosine kinases.<br />

shared haplotype<br />

Phenotypic characteristic shared by two siblings based on<br />

closely linked genes on one chromosome inherited from each<br />

parent. There are four different possibilities of reassortment<br />

among offspring, which leads to a particular sibling pair<br />

sharing two, one or no haplotypes. Selected haplotypes are<br />

in strong linkage disequilibrium between alleles of different<br />

loci. According to Mendelian genetics, 25% of siblings will<br />

share both haplotypes. Siblings are much more likely to share<br />

one or two haplotypes than are unrelated individuals.<br />

sheep red blood cell agglutination test<br />

An assay in which sheep erythrocytes are agglutinated<br />

by antibody or are used as carrier particles for an antigen<br />

adsorbed to their surfaces, in which case they are passively<br />

agglutinated by antibodies specific for the adsorbed antigen.<br />

shift assay<br />

A useful method for identifying protein–DNA interactions<br />

that may mediate gene expression, DNA repair, or<br />

DNA packaging. The assay can also be used to determine<br />

the affinity abundance, binding constants, and binding<br />

specificity of DNA-binding proteins. The gel shift assay is<br />

performed by annealing two labeled oligonucleotides that<br />

contain the test binding sequence, then incubating the duplex<br />

with the binding protein. The mixture is then separated on<br />

a nondenaturing polyacrylamide gel. Duplexes bound by<br />

protein migrate more slowly than unbound duplexes and<br />

appear as bands that are shifted relative to the bands from<br />

the unbound duplexes. Also called gel mobility shift assay,<br />

gel shift assay, gel retardation, or band shift assay.<br />

Shigella immunity<br />

The host protective immune response to Shigella infection<br />

is poorly understood. Because M cells of the gut take up<br />

the microorganisms, secretory immunoglobulin A (IgA)<br />

immune responsiveness has been postulated to be protective<br />

in shigellosis, but this has been difficult to establish.<br />

The immunity induced is type-specific, with reinfection<br />

occurring only within different Shigella species or<br />

serotypes. This immunity is believed to be associated with<br />

an immune response to lipopolysaccharide determinants.<br />

Shigella may destroy antigen-presenting cells in a host following<br />

systemic exposure to Shigella antigens and toxins<br />

before an immune response can be established. Serum<br />

IgG has no protective effect. Oral vaccines with attenuated<br />

Shigella induce type-specific protection. Previous<br />

Shigella infection leads to specific IgA secretion in breast<br />

milk. Antibodies develop early against somatic Shigella<br />

antigens. Shiga toxin is a multimeric protein comprised of<br />

a single enzymatically active A subunit and five B subunits<br />

needed for toxin binding. It is synthesized only by Shigella<br />

dysenteriae type I strains and is an important virulence<br />

factor in the pathogenesis of hemolytic–uremic syndrome<br />

that may be a complication of infection. Shiga toxin induces<br />

IgM antibody responses, but the IgG response is lacking;<br />

however, IgG can be raised against Shiga toxin in animal<br />

models. Protection against Shigella has been associated<br />

with the humoral response to lipopolysaccharide (LPS) or<br />

plasmid-encoded protein antigens. T cells also become activated<br />

in vivo during Shigella infection. There may be some<br />

correlation between T cell activation and disease severity.<br />

Local cytokine synthesis is also significant in shigellosis.<br />

Increased levels of interleukin-1 (IL1), IL6, tumor necrosis<br />

factor (TNF), and interferon-γ (IFN-γ) have been found<br />

in stool specimens and plasma of infected patients. Both<br />

Th1 and Th2 cytokines are present in shigellosis. Humoral<br />

and cytotoxic defense mechanisms may be present during<br />

infection. High serum antitoxin titers failed to protect<br />

monkeys against intestinal disease following challenge with<br />

live Shigella dysentariae. Heat-killed whole cell Shigella<br />

vaccines failed to protect. Post-infection reactive arthritis<br />

may occur. Although mucosal secretory IgA is thought to<br />

prevent bacterial attachment to the mucosa and neutralize<br />

toxins, the significance of these mechanisms in shigellosis<br />

has not been proven.<br />

shingles (herpes zoster)<br />

A virus infection that occurs in a band-like pattern according<br />

to distribution in the skin of involved nerves. It is usually<br />

a reactivation of the virus that causes chickenpox.<br />

SHIV<br />

Simian–human immunodeficiency virus. A SIV mac and<br />

HIV-1 chimera capable of infecting macaques, it possesses<br />

the HIV genes ENV, Tat, Rev, and Vpu, with its remaining<br />

genes derived from SIV mac.<br />

shocking dose<br />

The amount of antigen required to elicit a particular clinical<br />

response or syndrome.<br />

shock organ<br />

An organ involved in a specific reaction such as an anaphylactic<br />

reaction.<br />

short-lived lymphocytes<br />

Lymphocytes with lifespans of 4 to 5 days, in contrast to<br />

long-lived lymphocytes that may exist in blood circulation<br />

from months to years.<br />

Shulman, Sidney<br />

Professor of microbiology and immunology at the<br />

University of Buffalo who performed important work on<br />

immune aspects of human infertility.<br />

Shwartzman, Gregory (1896–1965)<br />

Russian–American microbiologist who described systemic<br />

and local reactions that follow injection of bacterial endotoxins.<br />

The systemic Shwartzman reaction, a nonimmunologic<br />

phenomenon, is related to disseminated intravascular<br />

coagulation. The local Shwartzman reaction in skin resembles<br />

the immunologically based Arthus reaction in appearance.<br />

(Refer to Phenomenon of Local Tissue Reactivity and<br />

Its Immunological and Clinical Significance, 1937.)<br />

Shwartzman (or Shwartzman–Sanarelli) reaction<br />

A nonimmunologic phenomenon in which endotoxin<br />

(lipopolysaccharide) induces local and systemic reactions.

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