Histiocytosis

Histiocytosis
Histiocytosis
Classification and external resources
ICD-10 C96.1, D76.0
ICD-9 202.3, 277.89
eMedicine ped/1997
MeSH D015614

In medicine, histiocytosis refers to an excessive number of histiocytes,[1] (tissue macrophages), and is typically used to refer to a group of rare diseases which share this as a characteristic. Occasionally and confusingly, the term "histiocytosis" is sometimes used to refer to individual diseases.

The histiocytes may attack skin, bone, muscles, and other important organs, including the liver, lung, spleen, and hematopoietic system. The disease is somewhat similar to cancer, and treatment often involves radiation and chemotherapy.

According to the Histiocytosis Association of America, 1 in 200,000 children in the United States are born with histiocytosis each year.[2] HAA also states that most of the people diagnosed with histiocytosis are children under the age of 10. The University of California, San Francisco, states that the disease usually occurs from birth to age 15.[3]

Histiocytosis (and malignant histiocytosis) are both important in veterinary as well as human pathology.

Contents

Classification, and relationships to other conditions

There are competing systems for classifying histiocytoses. According to the 1999 classification proposed by the World Health Organization, they can be divided into three categories.[4][5] However, the classifications in ICD10 and MeSH are slightly different, as shown below:

Name WHO ICD10 MeSH
Langerhans cell histiocytosis (LCH) I D76.0 Langerhans-cell histiocytosis
Juvenile xanthogranuloma (JXG) II D76.3 non-Langerhans-cell histiocytosis
Hemophagocytic lymphohistiocytosis (HLH) II D76.1 non-Langerhans-cell histiocytosis
Niemann-Pick disease II E75.2 non-Langerhans-cell histiocytosis
Sea-blue histiocyte syndrome II - non-Langerhans-cell histiocytosis
Acute monocytic leukemia III C93.0 malignant histiocytic disorders
Malignant histiocytosis III C96.1 malignant histiocytic disorders
Erdheim-Chester disease II C96.1 malignant histiocytic disorders

Types of LCH have also been known as "Eosinophilic Granuloma", "Hand-Schuller-Christian Disease", "Letterer-Siwe Disease", and "Histiocytosis X". (See Langerhans cell histiocytosis for details).

Alternatively, histiocytoses may be divided into the following groups:[6]:714-724

  • X-type histiocytoses
  • Non-X histiocytoses

Common treatments

Organizations

Patients and families can gain some support by contacting patient organisations such as the Histiocytosis Association of America, which has several stable and proven treatment protocols available only for physicians,[7] or the Histiocytosis Research Trust.

References

  1. ^ Histiocytosis at eMedicine Dictionary
  2. ^ Disease information at the Histiocytosis Association of America
  3. ^ Histiocytosis - Signs and Symptoms
  4. ^ Harris N, Jaffe E, Diebold J, Flandrin G, Muller-Hermelink H, Vardiman J, Lister T, Bloomfield C (1999). "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". Ann Oncol 10 (12): 1419–32. doi:10.1023/A:1008375931236. PMID 10643532. 
  5. ^ Histiocytosis at eMedicine
  6. ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0. 
  7. ^ Histio.Org Physicians Information Request

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Look at other dictionaries:

  • Histiocytosis — vgl. Histiozytose …   Das Wörterbuch medizinischer Fachausdrücke

  • Histiocytosis — A rare but potentially deadly disorder with similarities to cancer, in which histiocytes start to multiply and attack the person’s own tissues or organs. The result can be tissue damage, pain, development of tumor like lumps called granulomas,… …   Medical dictionary

  • Histiocytosis X — Klassifikation nach ICD 10 C96.0 Abt Letterer Siwe Krankheit Letterer Siwe Syndrom C96.1 Maligne Histiozytose D76.0 …   Deutsch Wikipedia

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  • Histiocytosis, Langerhans cell — Histiocytosis in which the active histiocytes normally occur in the skin. See histiocytosis …   Medical dictionary

  • Histiocytosis, malignant — Histiocytosis in which the histiocytes actually become cancerous. Treatment is by radiation and chemotherapy, and in some cases bone marrow transplantation. See also histiocytosis …   Medical dictionary

  • Histiocytosis, sinus — A variant of histiocytosis in which the lymph nodes are the main site of histiocyte proliferation. The sinuses become filled with and distended by masses of histiocytes. See also histiocytosis …   Medical dictionary

  • Histiocytosis, Hand-Schuller-Christian — A disease in which histiocytes start to multiply and attack the tissues or organs of the patient. The disease usually affects children age 2 to 5, less often older children and adults. The most frequent sites of bony involvement are the flat… …   Medical dictionary

  • Histiocytosis, lipid — A form of histiocytosis that affects lipid (fat) storage. Also known as Niemann Pick disease, Erdheim Chester disease …   Medical dictionary

  • histiocytosis — his·ti·o·cy·to·sis (hĭs tē ō sī tōʹsĭs) n. pl. his·ti·o·cy·to·ses ( sēz) Any of several abnormal conditions characterized by the appearance of histiocytes in the blood or tissues. * * * …   Universalium

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