Histiocytosis

Classification according to ICD-10
C96.0	Multifocal and multisystemic (disseminated) Langerhans cell histiocytosis [Abt-Letterer-Siwe disease] - histiocytosis X, multisystemic
C96.5	Multifocal and unisystemic Langerhans cell histiocytosis - Hand-Schüller-Christian disease - histiocytosis X, multifocal
C96.6	Unifocal Langerhans cell histiocytosis - Eosinophilic granuloma - Histiocytosis X, unifocal - Histiocytosis X onA - Langerhans cell histiocytosis onA
D76.3	Other histiocytosis syndromes
ICD-10 online (WHO version 2019)

Photomicrograph of Langerhans cell histiocytosis, a histiocytosis. HE staining .

Microphoto of a juvenile xanthogranuloma , a non-Langerhans cell histiocytosis. HE staining.

The histiocytoses are a heterogeneous group of rare disorders with tumor-like lesions caused by an abnormally high number ( proliferation ) of histiocytic cells are characterized.

Occurrence and frequency

The frequency is given as 1 in 200,000 newborns . While the disease can only occur in adults, most are diagnosed in childhood. According to the German Childhood Cancer Register , the frequency is 1–2 in 1 million newborns. The male gender is affected slightly more often.

Classification

The World Health Organization distinguishes three forms:

I Langerhans cell histiocytosis (LCH) Certain forms have been referred to as
- Eosinophilic granuloma , synonym: chronic and localized Langerhans cell histiocytosis
- Hand-Schüller-Christian syndrome , synonyms: Langerhans cell histiocytosis, chronic and multifocal; Granuloma, eosinophilic multifocal
- "Letterer-Siwe disease", synonym: Langerhans cell histiocytosis, acute and disseminated

II

III
- Acute monocytic leukemia
- Malignant histiocytosis

Other forms are:

Rosai-Dorfman disease , synonym: Sinus histiocytosis with massive lymphadenopathy
Hashimoto-Pritzker syndrome , synonym: Langerhans cell histiocytosis, congenital
Pulmonary Langerhans cell histiocytosis in adults , synonym: pulmonary histiocytosis X

Group of non-Langerhans cell histiocytoses
- Histiocytosis of the indeterminate cell
- Benign cephalic histiocytosis
- Generalized eruptive histiocytosis
- Hereditary mucinous progressive histiocytosis
- Nodular progressive histiocytosis
- Primary cutaneous anaplastic large cell lymphoma , synonym: regressive atypical histiocytosis
- Multicenter reticulohistiocytosis

Veterinary medicine

When dog playing malignant histiocytosis a role.

Individual evidence

↑ ^a ^b Emedicine, Medscape
^ Histiocytosis Association of America
^ NL Harris, ES Jaffe, J. Diebold, G. Flandrin, HK Muller-Hermelink, J. Vardiman, TA Lister, CD Bloomfield: The World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues. Report of the Clinical Advisory Committee meeting, Airlie House, Virginia, November, 1997. In: Annals of Oncology . Volume 10, Number 12, December 1999, pp. 1419-1432, PMID 10643532 .
↑ Langerhans cell histiocytosis. In: Orphanet (Rare Disease Database).
↑ granuloma, eosinophilic. In: Orphanet (Rare Disease Database).
^ Letterer-Siwe disease. In: Orphanet (Rare Disease Database).
↑ Lymphohistiocytosis, hemophagocytic, familial form. In: Orphanet (Rare Disease Database).
↑ Histiocytosis, sea blue. In: Orphanet (Rare Disease Database).
↑ Rosai-Dorfman disease. In: Orphanet (Rare Disease Database).
^ Hashimoto-Pritzker syndrome. In: Orphanet (Rare Disease Database).
↑ Langerhans cell histiocytosis, pulmonary, in adults. In: Orphanet (Rare Disease Database).
↑ Histiocytosis of the indeterminate cell. In: Orphanet (Rare Disease Database).
↑ Histiocytosis, benign cephalic. In: Orphanet (Rare Disease Database).
↑ histiocytosis, eruptive generalized. In: Orphanet (Rare Disease Database).
↑ Histiocytosis, progressive, mucinous, hereditary. In: Orphanet (Rare Disease Database).
↑ Histiocytosis, progressive, nodular. In: Orphanet (Rare Disease Database).
↑ Primary cutaneous anaplastic large cell lymphoma. In: Orphanet (Rare Disease Database).
↑ Reticulohistiocytosis, multicenter. In: Orphanet (Rare Disease Database).

Web links

www.histio.net The official website of the European Reference and Expert Centers for Histiocytosis.
Guideline histicytosis

[emed-1] Emedicine, Medscape

[2] Histiocytosis Association of America

[3] NL Harris, ES Jaffe, J. Diebold, G. Flandrin, HK Muller-Hermelink, J. Vardiman, TA Lister, CD Bloomfield: The World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues. Report of the Clinical Advisory Committee meeting, Airlie House, Virginia, November, 1997. In: Annals of Oncology . Volume 10, Number 12, December 1999, pp. 1419-1432, PMID 10643532 .

[4] Langerhans cell histiocytosis. In: Orphanet (Rare Disease Database).

[5] granuloma, eosinophilic. In: Orphanet (Rare Disease Database).

[6] Letterer-Siwe disease. In: Orphanet (Rare Disease Database).

[7] Lymphohistiocytosis, hemophagocytic, familial form. In: Orphanet (Rare Disease Database).

[8] Histiocytosis, sea blue. In: Orphanet (Rare Disease Database).

[9] Rosai-Dorfman disease. In: Orphanet (Rare Disease Database).

[10] Hashimoto-Pritzker syndrome. In: Orphanet (Rare Disease Database).

[11] Langerhans cell histiocytosis, pulmonary, in adults. In: Orphanet (Rare Disease Database).

[12] Histiocytosis of the indeterminate cell. In: Orphanet (Rare Disease Database).

[13] Histiocytosis, benign cephalic. In: Orphanet (Rare Disease Database).

[14] histiocytosis, eruptive generalized. In: Orphanet (Rare Disease Database).

[15] Histiocytosis, progressive, mucinous, hereditary. In: Orphanet (Rare Disease Database).

[16] Histiocytosis, progressive, nodular. In: Orphanet (Rare Disease Database).

[17] Primary cutaneous anaplastic large cell lymphoma. In: Orphanet (Rare Disease Database).

[18] Reticulohistiocytosis, multicenter. In: Orphanet (Rare Disease Database).