Letterer-Siwe disease

(redirected from nonlipid histiocytosis)
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Letterer-Siwe disease

 [let´er-er si´we]
a hereditary langerhans cell histiocytosis of early childhood, characterized by cutaneous lesions resembling seborrheic dermatitis, hemorrhagic tendency, hepatosplenomegaly with lymph node enlargement, and progressive anemia. If untreated it is rapidly fatal. Called also acute disseminated Langerhans cell histiocytosis.

Let·te·rer-Si·we dis·ease

(let'ĕr-ĕr sē'vĕ),
the acute disseminated form of Langerhans cell histiocytosis.

Letterer-Siwe disease

(lĕt′ər-ər-sī′wē, -sē′və)
n.
An often fatal form of Langerhans cell histiocytosis usually occurring in infants and characterized by maculopapular rash, lymphadenopathy, hepatosplenomegaly, gingival inflammation, and anemia.

Letterer-Siwe disease

A form of Langerhans' cell histiocytosis or histiocytosis X, with an onset in infancy of fever, eczematoid rash, lymphadenopathy, hepatosplenomegaly, anemia, death. See Histiocytosis, Langerhans' cell histiocytosis.

Let·te·rer-Si·we dis·ease

(let-ĕr'er sē'vē di-zēz')
The acute disseminated form of Langerhans cell histiocytosis.
Synonym(s): nonlipid histiocytosis.

Letterer-Siwe disease

A fatal disease of infants in which there is fever, generalized lymph node, liver and spleen enlargement, an eczematous skin rash and anaemia. The condition is brought about by an enormous increase in the number of antigen-presenting cells of the immune system known as Langerhans cells. These produce grossly excessive CYTOKINES resulting in great proliferation. The mortality varies but may be as high as 50 per cent. Experts argue as to whether or not the condition is a form of cancer. The preferred name for the condition is now Langerhans cell histiocytosis. (Erich Letterer, German pathologist, b. 1895, and Sture August Siwe, Swedish paediatrician, b. 1897).

Letterer,

Erich, German pathologist, 1895-1932.
Abt-Letterer-Siwe syndrome - Synonym(s): Letterer-Siwe disease
Letterer reticulosis - Synonym(s): Letterer-Siwe disease
Letterer-Siwe disease - the acute disseminated form of Langerhans cell histiocytosis. Synonym(s): Abt-Letterer-Siwe syndrome; Letterer reticulosis; Letterer-Siwe syndrome; nonlipid histiocytosis
Letterer-Siwe syndrome - Synonym(s): Letterer-Siwe disease

Siwe,

Sture August, Swedish pediatrician, 1897-1966.
Abt-Letterer-Siwe syndrome - Synonym(s): Letterer-Siwe disease
Letterer-Siwe disease - see under Letterer
Letterer-Siwe syndrome - Synonym(s): Letterer-Siwe disease

Let·te·rer-Si·we dis·ease

(let'er-er-sē'vē di-zēz')
The acute disseminated form of Langerhans cell histiocytosis.
Synonym(s): nonlipid histiocytosis.