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...

NCBI: db=pubmed; Term=rosai dorfman disease

Membranoproliferative glomerulonephritis associated with Rosai-Dorfman disease.

Clin Nephrol Case Stud. 2017;5:54-59

Authors: Sugimoto K, Ueda S, Okada M, Takemura T

Abstract
Rosai-Dorfman disease is also known as sinus histiocytosis with massive lymphadenopathy. Extranodal Rosai-Dorfman disease has been reported in ~ 43% of cases; the most frequent extranodal sites - skin, soft tissue, bone, respiratory tract, and eye - are usually involved in association with lymphadenopathy. Lack of lymph node involvement is rare, especially when patients manifest renal disease. Here, we describe a patient who developed membranoproliferative glomerulonephritis when lymphadenopathy was absent. During follow-up for sinus histiocytosis, a 7-year-old Japanese boy developed proteinuria and hematuria. No renal abnormality was present in ultrasound imaging. Histologic examination of a renal biopsy specimen disclosed moderate mesangial proliferation, focal thickening of glomerular capillary walls, and mesangial interposition. Mononuclear cells infiltrated the interstitium. Immunofluorescence showed intense IgG, C3, and C4 reactivity in portions of the mesangium and glomerular capillary walls. Electron microscopy depicted nodular deposits in mesangial, endocapillary, and subepithelial areas. Immunohistochemistry for S-100 protein, CD68, and lysozyme was positive within the interstitium. CD1a staining was absent. These findings were diagnostic for membranoproliferative glomerulonephritis. Multidrug therapy, including methylprednisolone and mizoribine, improved urinary findings and induced complete remission of both diseases. To the best of our knowledge, this is the first report of Rosai-Dorfman disease complicated by renal disease in the absence of concurrent nodal involvement. Clinicians should be alert to this diagnostic possibility.

PMID: 29043148 [PubMed]

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