Rosai-Dorfman Disease
Sinus Histiocytosis with Massive Lymphadenopathy (SHML)

Rosai-Dorfman disease (RD), also known as sinus Histiocytosis with massive lymphadenopathy (SHML), is a rare histiocytic disorder which involves the over-production of a type of white blood cell called non-Langerhans-cell sinus histiocytes. These cells then accumulate, most often in the lymph nodes but sometimes in other areas of the body and can lead to organ damage. The reason for over production of these cells is not known, although many possibilities have been considered, including viral, bacterial, infection, environmental, and genetic causes.

In 1969, two pathologists, Juan Rosai and Ronald Dorfman, reported a distinct histiocytic disorder in several patients with massive enlargement of the lymph nodes, as well as other symptoms. They named this condition sinus histiocytosis with massive lymphadenopathy, and the disease has since come to be known as Rosai-Dorfman disease.

The true number of RD cases is not known, although it does occur worldwide and seems to affect equal numbers of males and females. It is most commonly seen in the first 10 years of life, but it also occurs in adult patients.

Because this disease is so rare, no large studies have been performed, and there is no established, widely-accepted treatment. However, RD is usually not life-threatening, and many patients do not require treatment.

Please be advised that all the information you read here is not a replacement for the advice you will get from your consultant and their team.

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Rosai-Dorfman Disease
The questions below specifically relate to Rosai-Dorfman Disease.

1. What causes Rosai-Dorfman?
Rosai-Dorfman involves over-production of a type of white blood cell called a non-Langerhans-cell sinus histiocyte. The cause of this over-production is not yet known.

2. Is there a cure for Rosai-Dorfman?
While many patients go into remission and live normal lives with or without treatment, we usually do not use the term “cure.” There is no established period of inactive disease before RDD is considered cured.

3. What are the different therapies/treatments commonly used to treat Rosai-Dorfman?
Many Rosai-Dorfman patients do not require treatment. Some patients with severe or persistent disease may need treatment with surgery, steroids, and/or chemotherapy. Rarely, radiation therapy is used.

4. Can an infant be tested at birth for Rosai-Dorfman?
A biopsy of the affected tissue, rather than a blood test, is required for diagnosis and would therefore not be appropriate as a routine test unless this disease is suspected.

5. What causes chronic pain in adults with Rosai-Dorfman?
Some pain and cramping can be a side effect of treatment, such as vinblastine and steroids. Pain may also be directly related to active disease. In cases of more chronic pain, some researchers suspect that cytokines, which are a type of messenger, stimulate white blood cells to release inflammatory molecules that produce pain.

Please be advised that all the information you read here is not a replacement for the advice you will get from your consultant and their team.

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What are the side effects of vinblastine?

Side effects include:
a. Low blood counts (with higher risk of infection)
b. Mild nausea/vomiting/constipation
c. Easily sunburned
d. Skin irritation at site of injection
e. Thin or brittle hair
f. Fatigue
g. Bone pain
h. Hoarseness
i. Seizures
j. Shortness of breath
k. Nerve damage (especially in adults) with tingling, numbness and/or pain of the hands and feet

What are the side effects of prednisone?

Side effects include:
a. increase in blood sugar
b. Increase in appetite
c. Heartburn
d. Bloating/fluid retention/weight gain
e. Difficulty sleeping
f. Mood/behavior/personality changes
g. Higher risk of infection
h. Slow wound healing
i. Muscle weakness
j. Loss of bone calcium
k. Increased hair growth

More unusual side effects may include:
a. Problems with vision/eye pain
b. Seizures
c. Confusion
d. Muscle twitching
What are the side effects of methotrexate?

Side effects include:
a. Mouth sores/swollen, tender gums
b. Nausea/vomiting/diarrhea/decreased appetite
c. Low blood counts
d. Dizziness/drowsiness
e. Headache

More unusual side effects may include:
a. Blurred vision or loss of vision
b. Seizures
c. Confusion
d. Weakness/difficulty moving one or both sides of the body
e. Loss of consciousness
f. Lung damage
g. Allergic reactions

What are the possible side effects of 6-MP (mercaptopurine)?

More common signs/symptoms include:
a. Low blood counts (red cells, white cells, and clotting cells)
b. Nausea/vomiting/decreased appetite
c. Headache
d. Weakness/fatigue/achiness
e. Rash/darkening of the skin

Please be advised that all the information you read here is not a replacement for the advice you will get from your consultant and their team.

Help ensure that we can continue to bring you this vital informational material, make a donation today

Rosai-Dorfman disease Newsfeed

pubmed: rosai dorfman diseas...

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Related Articles

Rosai-Dorfman-Destombes disease with renal involvement and secondary glomerulopathy: Report of an exceptional case.

Urol Case Rep. 2019 Jan;22:17-18

Authors: Bassa C, Tagle R, Claro E, Mendez G, Dominguez J

PMID: 30306052 [PubMed]

Related Articles

Rosai-Dorfman Disease: Self-Resolving Unilateral Lymphadenopathy and a Brief Review of Literature.

Case Rep Oncol Med. 2018;2018:4869680

Authors: Feriante J, Lee RT

Abstract
Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a disease of histiocytic proliferation with no known pathogenesis. This disease is defined by histological and cytological characteristics, including emperipolesis and positive S100 and CD68 markers. Although the disease typically presents clinically with massive bilateral lymphadenopathy due to sinus expansion from excessive histiocytosis, only unilateral lymphadenopathy was observed in this patient. The case involves a 40-year-old Caucasian male from the Netherlands presenting with unilateral lymphadenopathy suspicious for malignancy. Subsequent histological and laboratory testing led to the rare diagnosis of RDD. The lymphadenopathy resolved spontaneously over the course of several weeks following the initial presentation. Rosai-Dorfman disease reportedly has a benign prognosis with approximately 20% of patients experiencing spontaneous disease resolution (as was the case for this patient) with 70% experiencing chronic symptoms that may last years but not require intervention. We therefore advocate observation as a mainstay of treatment for most cases of this rare disease with intervention only being pursued in symptomatic cases. A review of recent literature regarding pathogenesis, epidemiology, diagnostic factors, prognosis, and treatment is provided and discussed.

PMID: 30305972 [PubMed]

Related Articles

Simultaneous Occurrence of Rosai-Dorfman Disease and Nodal Marginal Zone Lymphoma in a Patient with Sjögren's Syndrome.

Case Rep Hematol. 2018;2018:7930823

Authors: Gorodetskiy VR, Klapper W, Probatova NA, Vasilyev VI, Rozhnova EV

Abstract
We present an exceptionally rare case of co-occurrence of Rosai-Dorfman disease (RDD) and nodal marginal zone lymphoma (NMZL) in a 60-year-old Caucasian female with a 20-year course of Sjögren's syndrome (SS). In response to treatment for lymphoma, the patient presented a short positive response, followed by a rapid progression of the disease accompanied by the development of the peripheral facial nerve palsy. We failed to detect Epstein-Barr virus (EBV) in the NMZL/RDD sample by EBV-encoded RNA (EBER) in situ hybridization but identified genomic DNA of EBV by polymerase chain reaction. A second biopsy revealed EBV-positive diffuse large B-cell lymphoma (DLBCL), not otherwise specified. The identical clonal immunoglobulin heavy chain gene rearrangements in the NMZL and DLBCL pointed to their clonal relationship. Though the role of EBV in the pathogenesis of some lymphomas is well-known, there have been only few cases of EBV-induced transformation of low-grade B-cell lymphoma into high-grade lymphoma and no cases of a patient with an NMZL background. To our knowledge, this is the first report of a concomitant occurrence of RDD and NMZL in a SS patient.

PMID: 30305966 [PubMed]

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