Rosai Dorfman Disease

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Classification according to ICD-10
D76.3 Other histiocytosis syndromes
ICD-10 online (WHO version 2019)
Photomicrograph of Rosai-Dorfman Disease. HE staining .

The Rosai-Dorfman Syndrome is a rare disease from the group of histiocytosis . The disease is characterized by the proliferation of histiocytes , which mainly accumulate in the lymph nodes .

Synonyms : Sinus Histiocytosis Rosai Dorfman; Sinus histiocytosis with massive lymphadenopathy; Histiocytic lymphophagocytic panniculitis; Benign pseudolymphomatosis; 'Destombes-Rosaï-Dorfman disease; Rosaï-Dorfman-Destombes disease; SHML

It was first described in 1969 by US pathologists Juan Rosai and Ronald F. Dorfman .

pathology

There is a pronounced, bilateral enlargement of the cervical lymph nodes, which is mainly due to the widening of the sinus of the lymph nodes. These sinuses are filled with activated histiocytes that envelop intact erythrocytes and lymphocytes in what is known as emperipolesis .

At the same time there is an increase in plasma cells , lymphocytes and neutrophils in the sinus . Other localizations are also possible: foci can occur in the skin, the meninges , the upper respiratory tract , salivary glands and bone marrow .

Occurrence

The frequency has not yet been clarified; it is assumed that viral infections can play a role. In Europe, Rosai-Dorfman syndrome is very rare and affects v. a. Adolescents between the ages of 15 and 20, and more black people around the world are affected.

root cause

The disease are mutations in SLC29A33 - gene based encoding nucleoside transporter for one.

The same gene is also involved in H syndrome and dysosteosclerosis .

Clinical manifestations

In addition to pronounced lymph node swelling ( lymphadenopathy ), general symptoms such as fever and weight loss can occur.

In about half of the cases, the foci of the disease are found outside the lymph nodes (skin, upper respiratory tract, salivary glands), which are then shown by specific symptoms for the localization site.

The course of the disease is predominantly self-limiting; general signs of inflammation can be found in the laboratory.

literature

  • S. Vandersee, HJ Röwert-Huber, S. Wöhner, C. Loddenkemper, M. Beyer, D. Humme: Cutaneous Rosai-Dorfman Syndrome. Successful therapy with intralesionally applied steroids. In: The dermatologist ; Journal of Dermatology, Venereology, and Allied Fields. Volume 65, No. 8, August 2014, pp. 725-727, doi: 10.1007 / s00105-014-2797-0 , PMID 24831530 .
  • Werner Böcker: Textbook Pathology . 4th edition. Urban & Fischer Verlag, 2008, ISBN 978-3-437-42382-6

Individual evidence

  1. ↑ a b Rosai-Dorfman disease. In: Orphanet (Rare Disease Database).
  2. ↑ Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
  3. ^ J. Rosai, RF Dorfman: Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. In: Archives of pathology. Volume 87, Number 1, January 1969, pp. 63-70, PMID 5782438 .
  4. ↑ WD James, TG Berger, DM Elston: Andrews' Diseases of the Skin: Clinical Dermatology . 11th edition. Saunders Elsevier, 2006, ISBN 978-1-4377-0314-6
  5. ^ A b E. Foucar, J. Rosai, R. Dorfman: Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. In: Seminars in diagnostic pathology. Volume 7, No. 1, February 1990, pp. 19-73, PMID 2180012 (review).
  6. ↑ Dysosteosclerosis.  In: Online Mendelian Inheritance in Man . (English)
  7. ↑ W. Hindermann, D. Katenkamp: Extranodal Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy). In: The Pathologist. 25, 2004, p. 222, doi: 10.1007 / s00292-003-0642-9
  8. ↑ a b Werner Böcker et al. (Ed.): Textbook Pathology 4th Edition. Urban & Fischer Verlag, 2008, ISBN 978-3-437-42382-6