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hypereosinophilia 346 hypersensitivity<br />

endothelial cells expressing glycolipid and glycoprotein<br />

galactose-α (1-3) galactose.<br />

hypereosinophilia<br />

Markedly elevated numbers of eosinophils in the peripheral<br />

blood.<br />

hypergammaglobulinemia<br />

Elevated serum γ globulin (immunoglobulin) levels. A<br />

polyclonal increase in immunoglobulins in the serum<br />

occurs in any condition involving continuous stimulation<br />

of the immune system, such as chronic infection, autoimmune<br />

disease, systemic lupus erythematosus (SLE), etc.<br />

Hypergammaglobulinemia may also result from a monoclonal<br />

increase in immunoglobulin production, as in multiple<br />

myeloma, Waldenström’s macroglobulinemia, and other<br />

conditions associated with the formation of monoclonal<br />

immunoglobulins. Repeated immunization may also induce<br />

hypergammaglobulinemia.<br />

hypergammaglobulinemic purpura<br />

Purpura hyperglobulinemia.<br />

hyper-IgD with periodic fever syndrome<br />

An autoinflammatory primary immunodeficiency associated<br />

with increased serum IgD. Attributable to mutations in<br />

mevalonate kinase, an enzyme that participates in cholesterol<br />

biosynthesis.<br />

hyper-IgE syndrome<br />

A primary immunodeficiency of unknown etiology and associated<br />

with remarkably elevated circulating levels of IgE.<br />

hyper-IgM syndrome (HIGM)<br />

Related primary immunodeficiencies associated with normal<br />

to markedly elevated levels of circulating IgM but very<br />

diminished or absent levels of the remaining immunoglobulin<br />

isotypes. Monoclonal antibodies against CD40 surface<br />

protein of B cells induce isotype switching in the presence<br />

of appropriate costimulatory cytokines. CD40L is a surface<br />

molecule transiently expressed on activated T cells mostly<br />

of the CD4 + subpopulation. Mutations of CD40L account<br />

for the X-linked form of the disease.<br />

hyperimmune<br />

A descriptor for an animal with a high level of immunity<br />

that is induced by repeated immunization of the animal to<br />

generate large amounts of functionally effective antibodies,<br />

in comparison to animals subjected to routine immunization<br />

protocols, perhaps with fewer boosters.<br />

hyperimmunization<br />

Successive administration of an immunogen to an animal<br />

to induce the synthesis of antibody in relatively large<br />

amounts. This procedure is followed in the preparation of<br />

therapeutic antisera by repeatedly immunizing animals to<br />

render them hyperimmune.<br />

hyperimmunized individual<br />

A person who has formed alloantibodies against an antigen<br />

to which he or she was previously exposed, such as a prior<br />

allograft, blood transfusion, or pregnancy. May sometimes<br />

be attributable to natural antibodies specific for antigenic<br />

determinants of pathogens that cross react with allogeneic<br />

donor antigens of a graft.<br />

hyperimmunoglobulin E syndrome (HIE)<br />

A condition characterized by markedly elevated immunoglobulin<br />

E (IgE) levels (above 5000 IU/mL). The patients<br />

have early eczema and repeated abscesses of the skin,<br />

sinuses, lungs, eyes, and ears. Staphylococcus aureus,<br />

Candida albicans, Haemophilus influenzae, Streptococcus<br />

pneumoniae, and group A hemolytic streptococci are<br />

among the more common infectious agents. The principal<br />

infection produced by S. aureus and C. albicans is a “cold<br />

abscess” of the skin. The failure of IgE to fix complement<br />

and therefore cause inflammation at the infection site is<br />

characteristic. IgG antibodies against IgE form complexes<br />

that bind to mononuclear phagocytes, resulting in monokine<br />

release that induces calcium resorption from bone. As calcium<br />

is lost from the bone, osteoporosis results, leading to<br />

bone fractures. Patients with HIE have diminished antibody<br />

responses to vaccines and to major histocompatibility antigens.<br />

They may be anergic, and in vitro challenge of their<br />

lymphocytes with mitogens or antigens leads to diminished<br />

responsiveness. The CD8 + T lymphocyte population in the<br />

peripheral blood also decreases. The disease becomes manifest<br />

in young infants, shows no predilection for males versus<br />

females, and is not hereditary. Also called Job’s syndrome.<br />

hyperimmunoglobulin M syndrome<br />

An immunodeficiency disorder in which the serum<br />

immunoglobulin M (IgM) level is normal or elevated. By<br />

contrast, the serum IgG and IgA levels are strikingly diminished<br />

or absent. Patients have repeated infections and may<br />

develop neoplasms in childhood. This syndrome may be<br />

transmitted in an X-linked or autosomal-dominant fashion.<br />

It may also be related to congenital rubella. The condition<br />

is produced by failure of T lymphocytes to signal IgMsynthesizing<br />

B cells to switch to IgG- and IgA-producing<br />

cells. In this X-linked disease in boys who are unable to<br />

synthesize immunoglobulin isotypes other than IgM, the<br />

gene encoding the CD40 ligand is defective. The T H cells<br />

fail to express CD40L. These patients do not develop<br />

germinal centers or displaced somatic hypermutation. They<br />

do not form memory B cells and are subject to pyogenic<br />

bacterial and protozoal infections.<br />

hyperplasia<br />

An increase in the cell number that leads to an increase in<br />

organ size. It is often linked to a physiological reaction to a<br />

stimulus and is reversible.<br />

hypersensitive response (HR)<br />

An antipathogen response in plants produced by avr-R<br />

system activation that leads to alterations in Ca + flux,<br />

MAPK activation, and NO and ROI formation. There is<br />

rapid necrosis of plant cells in contact with the pathogen.<br />

This process prevents spread of the pathogen and releases<br />

hydrolytic enzymes that facilitate injury to the pathogen’s<br />

structural integrity.<br />

hypersensitivity<br />

Increased reactivity or increased sensitivity by an animal<br />

body to an antigen to which it has been previously<br />

exposed. The term is often used as a synonym for allergy,<br />

which describes a state of altered reactivity to an antigen.<br />

Hypersensitivity has been divided into categories based<br />

upon whether it can be passively transferred by antibodies<br />

or by specifically immune lymphoid cells. The most widely<br />

adopted current classification is that of Coombs and Gell,<br />

which designates immunoglobulin-mediated hypersensitivity<br />

reactions as types I, II, and III and lymphoid-cellmediated<br />

(delayed-type) hypersensitivity/cell-mediated<br />

immunity as a type IV reaction. Hypersensitivity generally<br />

represents the “dark side,” signifying the undesirable<br />

aspects of an immune reaction, whereas immunity implies a<br />

desirable effect.

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