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Sinus Histiocytosis With Lymphadenopathy - Causes, Symptoms, Diagnosis, and Treatment

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Sinus histiocytosis with lymphadenopathy is a condition characterized by an excess of histiocytes. Read below to know more about it.

Written by

Dr. Anjali

Medically reviewed by

Dr. Kaushal Bhavsar

Published At January 12, 2023
Reviewed AtJanuary 11, 2024

Introduction

Sinus histiocytosis, also known as Rosai-Dorfman disease, is a rare condition with an unknown etiology that is characterized by an abundance of histiocytes in the lymph nodes and other body parts. The Rosai-Dorfman disease (RDD) is a condition that manifests as an excess of histiocytes in the lymph nodes and is first identified in 1969. Although other lymph nodes and other areas of the body may also be impacted, the cervical nodes in the neck are where these histiocytes most frequently collect. The skin, sinuses, and upper respiratory system may also develop histiocyte accumulation. Rosai-Dorfman disease rarely poses a threat to life. The patient's symptoms frequently get better on their own.

What Is Rosai-Dorfman Disease?

Histiocytes, which are white blood cells, overgrow in Rosai-Dorfman disease (RDD), a rare benign disorder. The extra histiocytes typically result in lymphadenopathy or swollen lymph nodes in the neck, but they can also affect other lymph nodes. These cells can also gather in locations other than lymph nodes (extranodal sites). Histiocytes are a crucial type of immune system cell. They combat pathogens that could make you ill. However, having an excessive number of histiocytes, as seen in Rosai-Dorfman's illness, might have unpleasant side effects or severely affect a biological system. The term sinus histiocytosis with extensive lymphadenopathy refers to Rosai-Dorfman's illness. An uncommon non-Langerhans cell histiocytosis exists in this case.

Who Is Affected by Rosai-Dorfman Disease?

Most people with Rosai-Dorfman disease are children, teenagers, and young adults. The average age of diagnosis for RDD is around 20, but incidences of RDD involving adults in their 70s have been documented. People of African origin who are born male most typically get RDD that affects the lymph nodes. People of Asian heritage assigned to the female gender at birth are most commonly diagnosed with cutaneous RDD (CRDD). Most CRDD sufferers are in their 20s, 30s, and 40s.

What Are the Symptoms of Rosai-Dorfman Disease?

Depending upon the location of extra histiocytes gathered will affect the symptoms. If the histiocytes impact the neck's lymph nodes, a person will suffer minor symptoms or none at all. If the histiocytes interfere with an organ's proper operation, a person can experience more severe symptoms.

  • Histiocytes typically develop in the lymph nodes of the neck. The most typical symptom is a lump on each side of the neck that is enlarged but not painful. The neck and groin are two locations where histiocyte expansion may induce lymph node swelling.

  • Skin-related Rosai-Dorfman disease can manifest itself anywhere on the body. Growths created by the histiocytes usually expand slowly. Skin growths that may occur are one of the signs and symptoms of flat or elevated, solid or pus-filled, brown, purple, yellow, or red, across the skin or confined to a single location.

  • Other symptoms include fever, fatigue, night sweats, a stuffy nose, and unaccounted-for weight loss.

  • RDD that affects the eyes results in double vision. RDD, which affects the central nervous system, results in seizures, and RDD, which affects the lungs, results in a chronic cough.

What Are the Causes of Rosai-Dorfman Disease?

The Rosai-Dorfman disease root cause is unknown. RDD may have several reasons because each person is affected by it differently. For instance, current research indicates that CRDD is likely to have a distinct etiology from classic RDD. Other than CRDD, researchers have recently found gene alterations that occasionally manifest in classic and extranodal RDD kinds. A cell's growth may become uncontrolled due to gene mutations. Many individuals with RDD also suffer from other illnesses. These illnesses and RDD might be related in some way. To be certain, more study is required.

  • Infections caused by viruses are linked to RDD (herpes, Epstein-Barr, cytomegalovirus, human immunodeficiency virus).

  • Cancer (Hodgkin and non-Hodgkin lymphoma, cutaneous clear-cell sarcoma).

  • Autoimmune diseases (lupus, juvenile idiopathic arthritis, autoimmune hemolytic anemia).

How to Diagnose Rosai-Dorfman Disease?

A doctor will conduct a physical examination and inquire about the symptoms. They will search for RDD symptoms, including enlarged lymph nodes, skin growths, or lumps. To determine whether the patient has or has ever had any of the illnesses linked to RDD, they will also inquire about medical history.

Any of the following may also be done by the healthcare provider:

  • Imaging: Depending on the tissue type, doctors suspect extra histiocytes, then they may perform an ultrasound, MRI (magnetic resonance imaging), CT (computed tomography) scan, bone scan, or X-ray.

  • Blood Testing: A healthcare provider may carry out blood tests, such as a comprehensive metabolic panel and a complete blood panel (CBC).

  • Biopsy: A healthcare professional may take a sample of the damaged tissue and examine the cells for indications of RDD. The healthcare provider can use a biopsy to rule out other disorders causing abnormal cell development.

What Is the Treatment for Rosai-Dorfman Disease?

RDD spontaneously remit remission. In a few months or a few years, it might disappear. Other times, the illness does not get better on its own, or after remission, the cells start to grow back. If RDD is not treated, it can get worse.

Depending on the course of RDD, treatment will vary. It includes:

  • Observation: If symptoms are not affecting the quality of life, the doctor may decide to keep monitoring health.

  • Surgery: A doctor may do surgery to remove the growths. If the patient has CRDD or growths obstructing the airway, affecting the spinal cord, etc.

  • Radiotherapy: If the doctor cannot remove the histiocytes with surgery, radiation therapy may be given to kill the cells. An apparatus directs focused energy beams at the cells during radiation therapy, killing them.

  • Chemotherapy: In serious illness or if other treatments do not improve symptoms, the doctor prescribes chemotherapy medications. Prednisone and other corticosteroids can reduce lymph node swelling and relieve symptoms.

  • Immunotherapy: Immunotherapy treatments make it easier for the immune system to find and get rid of extra histiocytes.

What Is the Prognosis of Rosai-Dorfman Disease?

The prognosis relies on several variables, including the number of lymph nodes affected and the location of extra histiocytes in the body. A person's reaction to the medication also matters. RDD frequently resolves by itself. In some cases, it can be necessary to receive therapy, or it might result in serious problems. The prognosis is generally better when fewer lymph nodes are involved. The results are frequently better if extranodal RDD affects the skin, chest, or upper respiratory system. The prognosis is poor if the histiocytes collect in the lower respiratory tract, kidneys, or liver.

Conclusion

RDD is uncommon and affects people differently, so diagnosing it can be difficult. There is not a single recommendation for treating RDD. To fully understand diagnosis and treatment options, it is important to communicate with a healthcare professional. A combination of treatments may be necessary for proper recovery from RDD, or it may be a mild case that goes away independently. Inquire with the healthcare provider about how the condition will affect treatment strategy and probable results.

Frequently Asked Questions

1.

How Long Will Rosai-Dorfman Be Alive?

The Rosai-Dorfman illness goes away on its own in a few months or years without any therapy. Our term for this is spontaneous remission. Ongoing monitoring is the recommended method to assess whether the patient will benefit from therapy. 

2.

What Is the Medical Term for Rosai-Dorfman Disease?

Rosai-Dorfman disease, sometimes called Destombes-Rosai-Dorfman disease or sinus histiocytosis with extensive lymphadenopathy, is a rare ailment with an unclear etiology that is characterized by an abundance of histiocytes in the body's lymph nodes and other areas.

3.

What Are the Symptoms of Rosai-Dorfman?

White blood cells known as histiocytes proliferate excessively in Rosai-Dorfman disease (RDD), an uncommon but benign disorder. The overgrowths can impact the skin and other organs, but they often result in enlarged lymph nodes in the neck.

4.

Is Rosai-Dorfman Cancerous or Benign?

As opposed to malignancies or cancer, Rosai-Dorfman is benign, meaning it does not spread to other body regions. Complications and organ damage might still result from the growths associated with RDD.

5.

Is Rosai-Dorfman Illness Curable?

Rosai-Dorfman occasionally disappears on its own. However, the precise time is only sometimes known. It might disappear in a few months or years. Localized illness with symptoms can be treated with radiation therapy or with surgical excision.

6.

Rosai-Dorfman Is a Rather Common?

Rosai-Dorfman illness is not common. Rosai-Dorfman disease affects about 1 in 200,000 individuals. Every year, over 100 new instances are detected in the United States.

7.

How Uncommon Is Rosai-Dorfman Disease?

Histiocyte proliferation is the hallmark of Rosai-Dorfman disease (RDD), a rare non-malignant condition with an incidence of just 1 in 200,000 in the United States. RDD was initially identified as a unique clinicopathological entity in 1969.

8.

What Is Recurrent Rosai-Dorfman Disease?

An uncommon benign tumor called Rosai-Dorfman Disease is distinguished by an overabundance of histiocytes in the affected tissue. When soft tissue RDD does not affect lymph nodes, recurrence is frequently observed.

9.

What Is a Rosai-Dorfman Illness Differential Diagnosis?

Several conditions are frequently included in the differential diagnosis, such as:
- Hemophagocytic syndrome.
- Reactive lymphoid hyperplasia with sinus histiocytes (RLHSH).
- Granulomatous lesions.
- Infectious lesions.
- Malignant lymphoma.

10.

Is Rosai-Dorfman Syndrome Fatal?

Dexamethasone and Methylprednisolone were used to treat lymph node enlargement, acute circulatory deficit, and acute respiratory failure. The presumed respiratory infections brought on by therapy-based immunosuppressants and the first chemotherapy treatment were the cause of mortality in Rosai-Dorfman's illness.
 
 

11.

What Are the Skin Signs of Rosai-Dorfman Disease?

The many forms of cutaneous symptoms include:
- Nodules.
- Plaques.
- Maculae.
- Papules.
- Reddish-brown to yellowish papules. 

12.

Rosai-Dorfman Illness With Granulomas: What Is It?

Massive cervical lymphadenopathy is the hallmark symptom of the uncommon, benign granulomatous illness Rosai-Dorfman.  A benign proliferative condition of the histiocytes, Rosai-Dorfman illness is sometimes called Sinus histiocytosis with extensive lymphadenopathy. 
Source Article IclonSourcesSource Article Arrow
Dr. Kaushal Bhavsar
Dr. Kaushal Bhavsar

Pulmonology (Asthma Doctors)

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