Rosai-Dorfman Disease of Bone

Rosai-Dorfman Disease of Bone

Article
Bone, Muscle, & Joint
Diseases & Conditions
+4
Contributed byKrish Tangella MD, MBAJul 23, 2019

What are the other Names for this Condition? (Also known as/Synonyms)

  • RDD of Bone

What is Rosai-Dorfman Disease of Bone? (Definition/Background Information)

  • Rosai-Dorfman disease (RDD) is a rare, benign condition that causes proliferation of histiocytes, a type of white blood cells, within the lymph nodes and other organs of the body. It can cause painless, lymph node enlargements at various sites in the body
  • Apart from the lymph nodes, RDD can also involve other body tissues and organs, such as the skin, bone, breast, respiratory airways, and central nervous system. In such cases, it is known as extranodal Rosai-Dorfman disease
  • Rosai-Dorfman Disease of Bone is a rare extranodal form of RDD, wherein along-with the involvement of the lymph nodes, the bone is also affected. The condition is seen in both children and adults
  • The individual can also have signs and symptoms of other organs along-with bone signs and symptom, such as bone pain and swelling. The treatment of the condition is typically symptomatic
  • The prognosis of Rosai-Dorfman Disease of Bone is usually good, since it can be a self-resolving condition, in a majority of cases. With appropriate treatment, as and when necessary, most individuals are able to make a complete recovery

Who gets Rosai-Dorfman Disease of Bone? (Age and Sex Distribution)

  • Rosai-Dorfman Disease of Bone can affect both children and adults. A wide age range of 3-56 years is seen (average age of diagnosis is 27 years)
  • Both male and female genders are affected
  • All racial and ethnic groups are at risk; although, more cases of RDD are seen in Africans and Afro-Americans than in Caucasians
  • Approximately 10% of RDD cases show bone involvement

What are the Risk Factors for Rosai-Dorfman Disease of Bone? (Predisposing Factors)

  • The risk factors for Rosai-Dorfman Disease of Bone are not well-established
  • However, some researchers believe that RDD may be influenced by certain viral and bacterial infections

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Rosai-Dorfman Disease of Bone? (Etiology)

The definitive cause of Rosai-Dorfman Disease of Bone is unknown. It may occur due to an abnormal immune system of the body, caused by a variety of triggers.

  • Infectious etiology: Some researchers believe that RDD may be caused by viral infections, such as caused by varicella zoster virus (VZV), Epstein-Barr virus (EBV), cytomegalovirus (CMV), and herpes virus; and, by bacterial infections caused by Brucella sp and Klebsiella
  • The abnormal immune response results in abnormal growth and multiplication of histiocytes within the lymph nodes. The histiocytes are a component of the white blood cells (WBCs or leukocytes) in the body
  • An associated increase in leukocyte count in peripheral blood may also be seen

What are the Signs and Symptoms of Rosai-Dorfman Disease of Bone?

The signs and symptoms of Rosai-Dorfman Disease of Bone can vary from one individual to another. Some are known to have mild symptoms, while others may have severe symptoms. Also, in some individuals, the symptoms are known to ‘appear and disappear’ over time.

The signs and symptoms of RDD of Bone may include:

  • Fever, night sweats, and headache
  • Since the bone is involved, it can result in bone pain and swelling of the affected bone
  • The affected bone can become weak and be more prone to fractures
  • The bones commonly involved include the tibia, femur, collar bone (clavicle), skull bone, bones of hand/feet
  • Joint pain
  • Weight loss, fatigue
  • Anemia, resulting in pale skin, tiredness, and shortness of breath
  • Lymphadenopathy or swollen lymph nodes; the enlargement is usually painless
    • The most commonly involved lymph nodes are the cervical (neck) lymph nodes, when it is termed cervical lymphadenopathy
    • Other lymph nodes involved include those located in the inguinal (groin), axillary (armpits), mediastinal (chest), and para-aortic region
    • Lymph nodes at other regions of the body may also be affected

How is Rosai-Dorfman Disease of Bone Diagnosed?

Rosai-Dorfman Disease of Bone is diagnosed using the following methods:

  • A thorough physical examination and assessment of one’s complete medical history
  • Radiological studies (X-ray, CT scan, MRI scan, etc.) of the affected bone; the affected bones on imaging studies may show lytic lesions (hole-like lesions)
  • Complete blood count
  • Tests to diagnose any underlying bacterial or viral infections
  • Biopsy of the affected bone

A biopsy on the affected lymph node may be performed:

  • Lymph node biopsy: A lymph node biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. The pathologist may use special techniques, such as immunohistochemistry stains, to help reveal the presence of the pathogen
  • The lymph node biopsy may be performed through any of the following procedures:
    • Fine needle aspiration biopsy (FNAB): A device called a cannula is used to extract tissue or fluid from the lymph nodes
    • Lymph node core biopsy of the enlarged lymph node
    • Lymph node open biopsy of the enlarged lymph node
  • Flow cytometry to rule-out lymphoma: The test helps identify cells as they flow through an instrument, called a flow cytometer. Flow cytometry measures the number and percentage of cells in a blood sample, and cell characteristics such as size, shape, and the presence of biomarkers on the cell surface

Note: A differential diagnosis may be undertaken to eliminate conditions presenting similar signs and symptoms, prior to arriving at a definite diagnosis of RDD of Bone.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Rosai-Dorfman Disease of Bone?

The complications of Rosai-Dorfman Disease of Bone may include the following:

  • Emotional stress: Prolonged enlargement of the lymph nodes raising a concern for malignant conditions such as lymphomas
  • Easy bone fractures due to weakening of the involved bones

How is Rosai-Dorfman Disease of Bone Treated?

No treatment may be required for many individuals with mild Rosai-Dorfman Disease of Bone. The healthcare provider may choose to undertake close observation of the affected individual, in such cases.

However, if the symptoms are significant, then the following treatment measures may be considered:

  • Steroid therapy to treat RDD
  • Surgical removal of enlarged lymph nodes
  • Rarely, chemotherapy and radiation therapy may be recommended on a case-by-case basis
  • Specific treatments may be considered based on the involved bone

How can Rosai-Dorfman Disease of Bone be Prevented?

There are currently no preventive methods available for Rosai-Dorfman Disease of Bone.

What is the Prognosis of Rosai-Dorfman Disease of Bone? (Outcomes/Resolutions)

The prognosis of Rosai-Dorfman Disease of Bone is based on the severity of the signs and symptoms. In many cases, the prognosis is good, since it is self-limited (i.e., goes away without treatment on its own).

Additional and Relevant Useful Information for Rosai-Dorfman Disease of Bone:

General information on lymph nodes:

The lymph nodes are part of one’s immune system. The lymph nodes are present throughout the body, usually in groups. In normal healthy adults, one cannot feel (see or touch) the lymph nodes readily. However, if they are enlarged, they can be felt either by the individual himself/herself or by the healthcare provider.

Enlarged lymph nodes can occur in both benign and malignant conditions. Hence, the cause of enlarged lymph nodes should be evaluated. If no obvious cause for enlargement of lymph nodes is found, then the possibility of a lymph node malignancy should be ruled out.

In a majority of individuals, a lymph node swelling is caused by a benign process such as an inflammation or infection. In many cases, swollen lymph nodes are part of other signs and symptoms. This can help a healthcare provider arrive at a list of follow-up tests and ensure an accurate diagnosis. Superficial enlarged lymph nodes can be felt by the healthcare provider through palpation. Enlarged lymph nodes deep in the body are often detected by radiological studies such as X-rays, ultrasound scan, CT and MRI scans.

Some enlarged lymph nodes can be painful, while others may be painless. Depending upon the underlying cause, enlarged lymph nodes can be localized to a particular area of the body, or they can be generalized, meaning that they are present throughout the body.

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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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