Home > Maligne hematologie > Myeloïde ziekten > Histiocytaire aandoeningen > Rosai-Dorfman



Histiocytaire aandoeningen zijn zeer zeldzaam (incidentie bij volwassenen minder dan 5 per miljoen) en hematologen worden er maar incidenteel mee geconfronteerd. Hieronder volgen enkele globale adviezen voor diagnostiek en therapie, waarbij de tekst beperkt is in de zorg voor volwassenen.

Rosai-Dorfman (sinushistiocytose met massieve lymfkliervergroting)

Goedaardige woekering van histiocyten, uitgaande van de lymfklieren. Geeft aanleiding tot zeer sterke lymfkliervergroting, en gaat veelvuldig gepaard met koorts, leukocytose en verhoogde bezinking. De oorzaak van het ziektebeeld is niet bekend. Er is zeer weinig te vinden over therapie. De literatuur van de laatste 20 jaar omvat grotendeels case-reports.

In het literatuuroverzicht hieronder een enkele selectie met tevens het abstract van elke referentie toegevoegd.


Filter trials en protocollen voor dit ziektebeeld
Bij asymptomatische patiënten is afwachten gerechtvaardigd. Bij symptomen is het gebruikelijk om symptomatisch te behandelen in de vorm van corticosteroiden. Een enkele keer is chirurgie zinvol wanneer de lymfadenopathie tot compressieverschijnselen aanleiding geeft. Radiotherapie is ook toegepast, evenals cytostatica, waarbij met name cladribine succesvol leek te zijn.

Onlangs is een interessante combinatie beschreven van Rosai-Dorfman met een hyper-IgG4 syndroom, waarbij sprake is van een sterke toename aan polyclonale IgG4-secernerende plasmacellen. Een en ander gaat gepaard met toegenomen systemische sclerosering, waaronder pancreatitis en nierinsufficiëntie. Ook dit beeld schijnt goed te reageren op corticosteroïden.


  1. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman Disease) – Review of the entity. Seminars in Diagnostic Pathology 1990; 7:19-73.
  2. Komp DM. The treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Semin Diagn Pathol 1990; 7:83-86.
    Abstract: The available information on treatment of sinus histiocytosis with massive lymphadenopathy (SHML) was reviewed. Treatment is not necessary in most instances, but some patients may require surgery, radiation therapy, and/or chemotherapy because of severe disease manifestations. An ideal treatment has yet to be identified. Of the various chemotherapeutic regimens that have been tried so far, a combination of vinca alkaloid, alkylating agent, and corticosteroid appears to be the most effective.
  3. Pulsoni A, Anghel G, Falcucci P et al. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review. Am J Hematol 2002; 69:67-71.
    Abstract: Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare disorder of unknown etiology, usually associated with lymph node enlargement in various superficial or deep sites. It usually shows a prolonged clinical course with occasional exacerbation and remission phases. We describe the long-term follow-up of a case of SHML that showed typical clinical features and in which various therapeutic strategies were attempted. Chemotherapy and alpha-interferon (IFN) were ineffective; surgery was ultimately required with satisfactory results. From an extensive literature review we found different treatment strategies in SHML in the 80 cases published between 1969 and 2000. Spontaneous resolution of adenopathies is frequently observed: 32 out of 40 cases which did not receive chemotherapy, radiotherapy, or surgery were healthy at the time of publication. Radiotherapy alone showed conflicting results: 3 complete remissions (CR) were obtained in the 9 patients treated. Surgical debulking when required was effective–8/9 CR–while chemotherapy showed generally negative results. IFN has been previously employed in only one case. In conclusion, clinical observation without treatment is advisable when possible. In the presence of vital organ compression and/or extranodal localization with important clinical signs, surgical debulking may be necessary. Radiotherapy has shown limited efficacy, while chemotherapy is in general ineffective. More experience is needed to evaluate the role of IFN.
  4. Konca C, Ozkurt ZN, Deger M, Aki Z, Yagci M. Extranodal multifocal Rosai-Dorfman disease: response to 2-chlorodeoxyadenosine treatment. Int J Hematol 2009; 89:58-62.
    Abstract: Rosai-Dorfman disease (RDD) or “sinus histiocytosis with massive lymphadenopathy” is a rare lymphoproliferative disorder of unknown etiology. The disease usually presents with painless lymphadenopathy with occasional extranodal involvement in various organs. We report a case of a 36-year-old man with a history of non-Hodgkin lymphoma (NHL), who recently presented with inguinal lymphadenopathy. Following the diagnosis of RDD on lymph node biopsy, he developed symptoms of spinal cord compression due to a mass lesion discovered at T6-7 vertebral level. 18F-Fluorodeoxyglucose (18FDG) positron emission tomography (PET-CT) revealed extensive disease with lung, renal and bone involvement. The patient received a short course of steroid therapy for cord compression findings and 2-chlorodeoxyadenosine (2-CdA) treatment was initiated for long-term disease control. He had a dramatic sustained response to treatment with six courses of 2-CdA. These results suggest that 2-CdA can be an effective treatment of choice and positron emission tomography with 18FDG can be used for determining the extent of disease and for follow-up in RDD.
  5. Bateman AC, Deheragoda MG. IgG4-related systemic sclerosing disease – an emerging and under-diagnosed condition. Histopathology 2009; 55:373-383.
    Abstract: Autoimmune pancreatitis was first described in 1961, although it was not more widely recognized as an autoimmune condition until 1995. It has now become apparent that this form of pancreatitis is part of a clinical syndrome that is commonly multisystem in nature. One of the most common histopathological features is the presence of IgG4+ plasma cells within involved tissues. Many terms have been proposed to describe the condition, but ‘IgG4-related systemic sclerosing disease’ appears most appropriate. Commonly affected extrapancreatic tissues include the biliary tract, liver, kidneys and lung, but a wide range of other sites may be involved. Histological examination reveals features that are not entirely disease-specific, but that are often sufficiently characteristic to provide useful support to a clinicopathological diagnosis. The disease often responds well to systemic steroid therapy, unlike some of the conditions that it may simulate clinically. The emergence of this disease as a specific and treatable entity has favourably altered the clinical outlook for patients in whom steroid therapy might not previously have been considered appropriate.

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