Flashcards: Immune system disorders

Severe combined immunodeficiency, SCID, also known as alymphocytosis, Glanzmann–Riniker syndrome, severe mixed immunodeficiency syndrome, and thymic alymphoplasia, is an extremely rare genetic disorder characterized by the disturbed development of functional T cells and B cells caused by numerous genetic mutations that result in heterogeneous clinical presentations.

Transplant rejection occurs when transplanted tissue is rejected by the recipient's immune system, which destroys the transplanted tissue.

A cytokine storm, also known as cytokine cascade and hypercytokinemia, is a potentially fatal immune reaction consisting of a positive feedback loop between cytokines and white blood cells, with highly elevated levels of various cytokines.

Familial Mediterranean fever (FMF), also known as Armenian disease, is a hereditary inflammatory disorder.

Chronic fatigue syndrome (CFS) is a medical condition characterized by long-term fatigue and other symptoms that limit a person's ability to carry out ordinary daily activities.

Wiskott–Aldrich syndrome (WAS) is a rare X-linked recessive disease characterized by eczema, thrombocytopenia (low platelet count), immune deficiency, and bloody diarrhea (secondary to the thrombocytopenia).

An opportunistic infection is an infection caused by pathogens (bacteria, viruses, fungi, or protozoa) that take advantage of an opportunity not normally available, such as a host with a weakened immune system, an altered microbiota (such as a disrupted gut flora), or breached integumentary barriers.

Erdheim–Chester disease (also known as Erdheim–Chester syndrome or polyostotic sclerosing histiocytosis) is a rare disease characterized by the abnormal multiplication of a specific type of white blood cells called histiocytes, or tissue macrophages (technically, this disease is termed a non-Langerhans-cell histiocytosis).

Isolated primary immunoglobulin M deficiency (or selective IgM immunodeficiency (SIgMD)) is a poorly defined dysgammaglobulinemia characterized by decreased levels of IgM while levels of other immunoglobulins are normal.

Oral allergy syndrome (OAS) is a type of food allergy classified by a cluster of allergic reactions in the mouth in response to eating certain (usually fresh) fruits, nuts, and vegetables that typically develops in adults with hay fever.

The foreign body granuloma is a response of biological tissue to any foreign material in the tissue.

XMEN disease is a rare genetic disorder of the immune system that illustrates the role of Mg2+ in cell signaling.

An immune disorder is a dysfunction of the immune system.

Aagenaes syndrome is a syndrome characterised by congenital hypoplasia of lymph vessels, which causes lymphedema of the legs and recurrent cholestasis in infancy, and slow progress to hepatic cirrhosis and giant cell hepatitis with fibrosis of the portal tracts.

In immunology, the Arthus reaction (/ˌɑːrˈtuːs/, /ˌɑːrˈtjuːs/) is a type of local type III hypersensitivity reaction.

Idiopathic CD4+ lymphocytopenia (ICL) is a very rare medical syndrome in which the body has too few CD4+ T lymphocytes, which are a kind of white blood cell.

Cutaneous small-vessel vasculitis (also known as "Cutaneous leukocytoclastic angiitis", "Cutaneous leukocytoclastic vasculitis", "Cutaneous necrotizing venulitis" and "Hypersensitivity angiitis") is inflammation of small blood vessels (usually post-capillary venules in the dermis), characterized by palpable purpura.

Gleich's syndrome or episodic angioedema with eosinophilia is a rare disease in which the body swells up episodically (angioedema), associated with raised antibodies of the IgM type and increased numbers of eosinophil granulocytes, a type of white blood cells, in the blood (eosinophilia).

Seabather's eruption is a pruritic dermatitis caused by a hypersensitivity reaction to the immature nematocysts of larval-stage Thimble Jellyfish (Linuche unguiculata), sea anemones (Edwardsiella lineata) and other larval cnidarians.

Caspase-8 deficiency state (CEDS) is a very rare genetic disorder of the immune system.

Hyper IgM Syndrome Type 1 (HIGM-1) is the X-linked variant of the Hyper-IgM syndrome.

Castleman disease, also known as giant or angiofollicular lymph node hyperplasia, lymphoid hamartoma, angiofollicular lymph node hyperplasia, is a group of uncommon lymphoproliferative disorders that share common lymph node histological features that may be localized to a single lymph node (unicentric) or occur systemically (multicentric).

Pityriasis lichenoides et varioliformis acuta (also known as "acute guttate parapsoriasis", "acute parapsoriasis", "acute pityriasis lichenoides", "Mucha–Habermann disease", "parapsoriasis acuta", "parapsoriasis lichenoides et varioliformis acuta", and "parapsoriasis varioliformis") is a disease of the immune system.

Graft-versus-host disease (GvHD) is a medical complication following the receipt of transplanted tissue from a genetically different person.

Pityriasis lichenoides chronica (also known as "Chronic guttate parapsoriasis," "Chronic pityriasis lichenoides," "Dermatitis psoriasiformis nodularis," "Parapsoriasis chronica," and "Parapsoriasis lichenoides chronica" "PLC") is an uncommon, idiopathic, acquired dermatosis, characterized by evolving groups of erythematous, scaly papules that may persist for months.

Mast cell activation syndrome (MCAS), also commonly referred to as mast cell activation disorder (MCAD), is an immunological condition in which mast cells inappropriately and excessively release chemical mediators, resulting in a range of chronic symptoms, sometimes including anaphylaxis or near-anaphylaxis attacks.

Kimura's disease is a benign rare chronic inflammatory disorder.

Cytokine release syndrome is a common immediate complication occurring with the use of anti-T cell antibody infusions such as ATG, OKT3, and TGN1412, but also with the CD-20 antibody rituximab.

The autosomal dominant syndrome associated with monocytopenia, B and NK cell lymphopenia and mycobacterial, fungal and viral infections (abbreviated MonoMAC) is a rare genetic disorder first described by Vihn and colleagues in 2010 and is associated with myelodysplasia, cytogenetic abnormalities, pulmonary alveolar proteinosis and myeloid leukemias.

Immunoproliferative disorders,also known as immunoproliferative diseases or immunoproliferative neoplasms, are disorders of the immune system that are characterized by the abnormal proliferation of the primary cells of the immune system, which includes B cells, T cells and Natural killer (NK) cells, or by the excessive production of immunoglobulins (also known as antibodies).

Gulf War syndrome (GWS), also known as Gulf War illnesses (GWI), is a chronic multisymptom disorder affecting returning military veterans and civilian workers of the 1990–91 Gulf War.

Heavy chain disease is a form of paraproteinemia with a proliferation of cells producing immunoglobulin heavy chains.

Asplenia refers to the absence of normal spleen function and is associated with some serious infection risks.

Cryofibrinogenemia is defined by the presence of circulating cold insoluble complexes of fibrin, fibrinogen, fibronectin, and fibrin split products with albumin, immunoglobulins and plasma proteins that precipitate from the patient's plasma in the cold.

Ectopic thymus is a condition where thymus tissue is found in an abnormal location.

Ichthyosis acquisita (or "acquired ichthyosis") is a disorder clinically and histologically similar to ichthyosis vulgaris.