heavy chain disease


Also found in: Dictionary, Thesaurus, Legal, Encyclopedia.
Related to heavy chain disease: macroglobulinemia, light chain disease, mu heavy chain disease

heavy chain disease

 
a rare monoclonal gammopathy characterized by neoplastic proliferation of plasma cells and precursors that secrete immunoglobulin heavy chains. It is often accompanied by hepatosplenomegaly, lymphadenopathy, and soft tissue tumors. There are three varieties, each characterized by a specific heavy chain: alpha chain disease, gamma chain disease, and mu chain disease.

heav·y chain dis·ease

a term used for a group of diseases, the paraproteinemias, characterized by production of homogeneous immunoglobulins or fragments, and associated with malignant disorders of the plasmacytic and lymphoid cell series. Three types have been recognized: γ-heavy-chain disease, α-heavy-chain disease, and μ-heavy-chain disease; each is diagnosed by the finding of the appropriate heavy-chain fragment in the serum, urine, or both.

heavy chain disease

Etymology: AS, heafig + L, catena, chain; dis, opposite of; Fr, aise, ease
a plasma cell disorder characterized by a proliferation of immunoglobulin heavy chains. Excessive levels of alpha, gamma, delta, and mu chains are produced, and effects tend to vary according to the predominant type of heavy chain. Alpha heavy chain disease mainly affects children living in the Middle East, causing diffuse abdominal lymphoma and malabsorption disorders. Most gamma heavy chain disease patients are elderly men who have symptoms resembling those of malignant lymphoma: enlarged liver and spleen, fever, anemia, and increased susceptibility to infections. Delta heavy chain disease is rare and marked by symptoms similar to those of multiple myeloma. Mu heavy chain disease presents symptoms of chronic lymphocytic leukemia, and treatment is symptomatic.
Any of a family of monoclonal gammaglobinopathies (paraproteinaemias) which are characterised by excess production of an immunoglobulin Fc fragment, detectable in the serum and/or urine and accompanied by lymphoproliferative disease.

heavy chain disease

Any of a family of monoclonal gammopathies or paraproteinemias characterized by excess production of an immunoglobulin Fc fragment, detectable in the serum and/or urine and accompanied by lymphoproliferative disease. See Hyperimmunoglobulin D. E, M syndromes.
Heavy chain disease
alpha heavy chain disease Seligmann's disease The most common heavy chain disease or paraproteinemia, in which there is an excess production of an incomplete IgA1 molecule–partial heavy chain and no light chain, which affects Sephardic Jews, Arabs and Mediterranean rim inhabitants Clinical Onset in childhood or adolescence as either a lymphoproliferative disorder confined to the respiratory tract or an enteric form–see IPSID with severe diarrhea, malabsorption, steatorrhea, weight loss, hepatic dysfunction, hypocalcemia, lymphadenopathy, marked mononuclear infiltration which may eventuate into lymphoma–see Mediterranean lymphoma; AHCD may remit spontaneously, respond to antibiotic therapy, or if clearly monoclonal, may require combination chemotherapy, potentially causing death by ages 20-30 Laboratory ↑ Alkaline phosphatase, ↓ Ca2+ Treatment Antibiotics, or if monoclonal, chemotherapy.
gamma heavy chain disease A disorder of older ♂, ranging from fulminant, ie death in weeks to prolonged, lasting 20 years, most die in the first year, often due to infection Clinical GHCD presents as a lymphoproliferation with fever, fatigue, anemia, angioimmunoblastic lymphadenopathy, hepatosplenomegaly, uvular and palatal edema, eosinophilic infiltrates, leukopenia, associated with autoimmune disease, tuberculosis and lymphoma Laboratory ↑ IgG1; most cases excrete less than 1 g/day of paraprotein, rarely up to 20 g/day Treatment Cyclophosphamide, vincristine, prednisone
mu chain disease A rare paraproteinemia that affects the middle-aged to elderly, most of whom have or slowly progress to CLL Clinical Lymphadenopathy, hepatosplenomegaly and BM infiltration by vacuolated plasma cells, often accompanied by ↑ kappa chain production Treatment As with CLL

heav·y chain dis·ease

(hev'ē chān di-zēz')
A term used for a group of diseases, the paraproteinemias, characterized by production of homogeneous immunoglobulins or fragments, and associated with malignant disorders of the plasmacytic and lymphoid cell series.

disease

traditionally defined as a finite abnormality of structure or function with an identifiable pathological or clinicopathological basis, and with a recognizable syndrome or constellation of clinical signs.
This definition has long since been widened to embrace subclinical diseases in which there is no tangible clinical syndrome but which are identifiable by chemical, hematological, biophysical, microbiological or immunological means. The definition is used even more widely to include failure to produce at expected levels in the presence of normal levels of nutritional supply and environmental quality. It is to be expected that the detection of residues of disqualifying chemicals in foods of animal origin will also come to be included within the scope of disease.
For specific diseases see under the specific name, e.g. Aujeszsky's disease, Bang's disease, foot-and-mouth disease.

air-borne disease
the causative agent is transmitted via the air without the need for intervention by other medium. See also wind-borne disease.
disease carrier
clinical disease
see clinical (3).
disease cluster
a group of animals with the same disease occurs at an unusual level of prevalence for the population as a whole. The cluster may be in space, with high concentrations in particular localities, or in time, with high concentrations in particular seasons or in particular years.
communicable disease
infectious disease in which the causative agents may pass or be carried from one animal to another directly or indirectly on inanimate objects or via vectors.
complicating disease
one that occurs in the course of some other disease as a complication.
constitutional disease
one involving a system of organs or one with widespread signs.
contagious disease
see communicable disease (above).
disease control
reducing the prevalence of a disease in a population, including eradication, by chemical, pharmaceutical, quarantine, management including culling, or other means or combinations of means.
disease control programs
organized routines specifying agents, administration, time and personnel allocations, community support, funding, participation of corporate or government agencies, animal and animal product disposal.
deficiency disease
a condition due to dietary or metabolic deficiency, including all diseases caused by an insufficient supply of essential nutrients.
degenerative joint disease
see degenerative joint disease, osteoarthritis.
demyelinating disease
any condition characterized by destruction of myelin.
disease determinant
any variable associated with a disease which, if removed or altered, results in a change in the incidence of the disease.
egg-borne disease
an infectious disease of birds in which the agent is spread via the egg.
endemic disease
see endemic.
environmental disease control
control by changing the environment, e.g. draining a swamp, ventilating a barn.
epidemic disease
etiological disease classification
diseases arranged in the order of their etiological agents, e.g. bacterial, mycoplasma.
exotic disease
a disease that does not occur in the subject country. Said of infectious diseases that may be introduced, e.g. rabies is exotic to the UK, contagious bovine pleuropneumonia is exotic to the USA.
focal disease
a localized disease.
fulminant disease
an explosive outbreak in a group or a rapidly developing, peracute development of a disease in an individual. Called also fulminating.
functional disease
any disease involving body functions but not associated with detectable organic lesion or change.
generalized disease
one involving all or many body systems; often said of infectious diseases in which there is spread via the bloodstream. See also systemic disease (below).
glycogen disease
any of a group of genetically determined disorders of glycogen metabolism, marked by abnormal storage of glycogen in the body tissues. See also glycogen storage disease.
heavy chain disease
hemolytic disease of newborn
see alloimmune hemolytic anemia of the newborn.
hemorrhagic disease of newborn
see neonatal hemorrhagic disease.
disease history
that part of a patient's history which relates only to the disease from which the patient is suffering.
holoendemic disease
most animals in the population are affected.
hyperendemic disease
the rate of infection is steady but high.
hypoendemic disease
the rate of infection is steady and only a few animals are infected.
immune complex disease
see immune complex disease.
infectious disease
one caused by small living organisms including viruses, bacteria, fungi, protozoa and metazoan parasites. It may be contagious in origin, result from nosocomial infections or be due to endogenous microflora of the nose and throat, skin or bowel. See also communicable disease (above).
manifestational disease classification
diseases arranged in the order of their clinical signs, epidemiological characteristics, necropsy lesions, e.g. sudden death diseases.
mesoendemic disease
the disease occurs at an even rate and a moderate proportion of animals are infected.
metabolic disease
see metabolic diseases.
molecular disease
any disease in which the pathogenesis can be traced to a single, precise chemical alteration, usually of a protein, which is either abnormal in structure or present in reduced amounts. The corresponding defect in the DNA coding for the protein may also be known.
multicausal disease
1. a number of causative agents are needed to combine to cause the disease.
2. the same disease can be caused by a number of different agents.
multifactorial disease
see multicausal disease (above).
new disease
disease not previously recorded. May be variants on an existing disease, e.g. infectious bovine rhinotracheitis, or escapes from other species, e.g. the Marburg virus disease of humans.
notifiable disease
a disease of which any occurrence is required by law to be notified to government authorities.
organic disease
see organic disease.
pandemic disease
a very widespread epidemic involving several countries or an entire continent.
quarantinable disease
a disease which the law requires to be restricted in its spread by putting the affected animals, farms or properties on which it occurs in quarantine.
reportable disease
see notifiable disease (above).
disease reservoir
any animal or fomite in which an infectious disease agent is preserved in a viable state or multiplies and upon which it may depend for survival.
secondary disease
1. a disease subsequent to or a consequence of another disease or condition.
2. a condition due to introduction of incompatible, immunologically competent cells into a host rendered incapable of rejecting them by heavy exposure to ionizing radiation.
self-limited disease
sex-limited disease
disease limited in its occurrence to one or other sex. See also sex-linked.
sexually transmitted disease (STD)
a disease that can be acquired by sexual intercourse.
slaughter disease control
see slaughter (2).
sporadic disease
occurring singly and haphazardly; widely scattered; not epidemic or endemic. See also sporadic bovine encephalomyelitis, sporadic leukosis, sporadic lymphangitis.
storage disease
disease syndrome
systemic disease
sufficiently widespread in the body to cause clinical signs referable to any organ or system, and in which localization of infection may occur in any organ.
disease triangle
interaction between the host, the disease agent, and the environment.
disease wastage
loss of income generated by production of milk, eggs, fiber, or loss of capital value because of diminution in the patient's value.
wasting disease
any disease marked especially by progressive emaciation and weakness.
zoonotic disease
disease capable of spread from animals to humans. See also zoonosis.