According to abbreviationfinder, histiocytic necrotizing lymphadenitis is an inflammation of the lymph nodes in the neck area, which can be accompanied by general symptoms such as fever or vomiting. Those affected are mostly Asian women in early adulthood, whose blood titer against Yersinia enterocolitica is elevated. The disease has hardly been researched, so that only therapeutics for symptomatic treatment are available.
What is histiocytic necrotizing lymphadenitis?
The lymphatic fluid plays a crucial role in the human immune system and is transported through the tissues on lymphatic vessels. In the case of lymphadenitis, the lymph nodes are inflamed, usually as part of an acute or chronic infection. Lymphadenitis is a variant of lymphadenopathy and can occur in different forms.
Histiocytic necrotizing lymphadenitis is a variant of lymphadenitis characterized by necrosis without granulocyte inclusions. The disease is also known as Kikuchi syndrome and occurs mainly in Asia. Cases are also known in Europe, but Europeans are affected much less frequently than Asians.
The disease mainly affects women. Figures show that the female population is even three times more likely to suffer from histiocytic necrotizing lymphadenitis than men. In most cases, the disease occurs in young adults and lasts an average of two months.
Little is known about the exact cause of histiocytic necrotizing lymphadenitis. Many scientists refer to an alleged connection with an increased blood titer against Yersinia enterocolitica, as it could be observed in the majority of previous patients. If such a causal connection exists, one can speak of an infectious genesis.
Previous cases of illness often occurred as a result of infections. This connection may also play a role in the pathogenesis of the disease. Some researchers reject the purely infectious genesis and consider the histiocytic necrotizing lymphadenitis to be an autoimmune reaction of the body. Whether the disease could be an infection – independent immune disease has not yet been clarified. The extent to which the Asian background plays a role in the pathogenesis is also unclear.
Symptoms, Ailments & Signs
In most cases, histiocytic necrotizing lymphadenitis affects the lymph nodes in the neck region. Some patients suffer from pathological changes only on one side. Others suffer from bilateral inflammation. In almost no case has the inflammation spread to other lymph nodes.
The inflamed tissues swell up to three centimeters, but usually do not cause any pain. As a rule, the inflammation is accompanied by general complaints. Those affected often feel similar to an infection. The most common general complaints are fever, exhaustion, tiredness, headache and dizziness.
In individual cases, these symptoms can be accompanied by other cold-like symptoms. Vomiting and sore throat are conceivable. However, absolute freedom from symptoms is also conceivable. For this reason, some patients hardly notice the disease. In the case of subjectively felt symptoms, it can happen, for example, that the swelling of the lymph nodes is only noticed during a routine examination.
Diagnosis & disease progression
The diagnosis of histiocytic necrotizing lymphadenitis is examined by means of anamnesis, palpation, sonography and, if necessary, histology. Macroscopically, there is hardening of the lymph nodes in the neck area, while all other lymph nodes are unremarkable. Histology shows a widened T-zone or paracortical zone with smaller necrotizing foci.
Characteristically, plasmacytoid monocytes are found in the zone. The foci are in turn mostly populated by histiocytes. Granulocytes are completely absent. To confirm the diagnosis, the doctor must resort to a tissue sample in the sense of removing a lymph node. All other methods do not allow a reliable diagnosis and can only indicate that pathological changes are present.
The prognosis for patients with histiocytic necrotizing lymphadenitis is rather favorable despite the below-average research situation. Most patients are cured without remission. Only a few of those affected develop sequelae such as SLE.
As a rule, this disease mainly causes problems in the neck and lymph nodes. However, the symptoms usually only spread to one side of the body. This leads to relatively severe swelling and pain in the affected areas. If the pain occurs in the form of rest pain, it can also lead to sleep problems in the patient.
There is often a general feeling of illness and exhaustion. The resilience of those affected decreases and it is not uncommon for fever and fatigue to occur. Furthermore, most of those affected suffer from nausea and vomiting and complain of severe headaches.
The quality of life is significantly restricted and reduced by this disease. In many cases, the patients no longer take an active part in life and withdraw. Loss of consciousness can also occur, which can be associated with a fall. A causal treatment of this disease is not possible, which is why only the symptoms can be limited.
Drugs are used and there are usually no complications. In most cases, the symptoms disappear after about a month and do not reappear afterwards. Life expectancy is usually not reduced.
When should you go to the doctor?
Histiocytic necrotizing lymphadenitis primarily affects Asian women in young adulthood. People in this risk group should see a doctor as soon as they experience vomiting or fever. If the symptoms persist for several days or weeks, it is advisable to have a check-up carried out. A doctor should be consulted if the lymph nodes are swollen on one side, sore throat, difficulty swallowing or pain when touching the throat.
If you experience fatigue, headaches, feeling unwell or dizziness, you should see a doctor. The flu-like symptoms of histiocytic necrotizing lymphadenitis increase in intensity if untreated, and the condition then persists for several months. This affects the performance of daily duties and can lead to further complications. Tiredness, inner weakness and a general feeling of illness should be checked if there is no improvement within a few days.
Sleep disorders and reduced performance must be investigated. In the case of stress, psychological problems, mood swings or behavioral problems, a doctor should be consulted. People who do not belong to the risk group but still suffer from the symptoms described should also consult a doctor.
Treatment & Therapy
The causes of HNL have not yet been conclusively determined. Therefore, no causal treatment option is currently available for patients with the disease. For this reason, purely symptomatic treatment is indicated, which is primarily intended to improve the subjectively felt symptoms.
In most cases, symptomatic treatment consists of administration of analgesics and antipyretics or nonsteroidal anti-inflammatory drugs. Other conservative drug treatment options include corticosteroids. However, these drugs are rarely required for those affected, as drugs with significantly fewer risks and side effects already lead to improvement in most patients.
Spontaneous improvement often occurs after a month with the administration of analgesics or antipyretics. However, there are also known cases in which the symptoms could only subside after four months. Whether the medication is actually responsible for the spontaneous improvement or whether the remission in all documented cases was due to endogenous processes remains controversial.
Since, according to previous documentation, SLE can develop after histiocytic necrotizing lymphadenitis, patients are advised to have regular check-ups for the next few years.
Outlook & Forecast
Histiocytic necrotizing lymphadenitis usually has a good prognosis. In most cases, the course is self-limiting. So the disease will heal on its own. Not much is known about the cause of the disease. The occurrence of lymph node swelling after infections is typical. Since the disease occurs mainly in Asia, it must be an infection that is more common there. The bacterium Yersinia enterocolitica is suspected to trigger an autoimmune process leading to histiocytic necrotizing lymphadenitis. Young women are mostly affected. The disease is observed much less frequently in men.
Histiocytic necrotizing lymphadenitis is often not diagnosed because it is very rare and its symptoms resemble many other, often more serious diseases such as adenocarcinoma, malignant lymphoma or systemic lupus erythematosus (SLE).
However, only the right diagnosis enables the right therapy. In the case of histiocytic necrotic lymphadenitis (Kikuchi-Fujimoto lymphadenitis), this is limited to symptomatic measures. A causal therapy is not yet possible due to the low level of knowledge. However, it is not necessary because the disease usually heals on its own. Spontaneous improvement occurs after one to four months. However, monitoring the patient for several years is recommended because the development of systemic lupus erythematosus cannot be ruled out.
The cause of histiocytic necrotizing lymphadenitis is not yet known. Until science agrees on a cause, no promising preventive measures for the condition will be available. If an infectious cause can actually be assumed, general infection prophylaxis may be a possible preventive measure.
The therapy of the histiocytic necrotizing lymphadenitis goes directly into the follow-up care. The patients can actively participate in the measures and thus promote healing. This is related to the symptomatic approach to disease treatment. It is important that those affected undergo regular check-ups and keep a precise overview of their complaints.
In this way, physical weaknesses can be recognized and combated in good time. The quality of life improves through medical care and support with individually tailored medication. This not only helps against the symptoms of the disease, but also promotes social contacts. Aftercare also includes the right diet, which strengthens the immune system with lots of vitamins and nutrients.
As a result, those affected feel more vital and deal with their illness in a more relaxed manner. In addition to the reduced stress, it is very important that patients become aware of their bodies. In this way, any complications caused by the medication can be detected at an early stage. If side effects appear, a short-term appointment with a specialist is required. This helps the patient to check the symptoms and adjust the medication if necessary. Close contact between patient and doctor is therefore an advantage for aftercare.
You can do that yourself
In the therapy of histiocytic necrotic lymphadenitis, the active participation of the patient in cooperation with the treating physicians is of great importance. Because a causal therapy of the disease is not yet possible, so that only symptomatic treatment approaches are practicable. The patients provide crucial information about the respective complaints, so that appropriate drugs are used.
Many of those affected suffer from general symptoms of the disease such as fever and fatigue. In severe cases, the physical exhaustion leads to the affected person withdrawing socially and having difficulties in carrying out their work. Medical care is particularly important here in order to prescribe the patient appropriate medication or to positively influence the immune system and the subjective body feeling through a changed diet. A diet rich in vitamins and nutrients supports the immune system of people with histiocytic necrotizing lymphadenitis. In this way, the patients at best get the feeling of increased vitality and experience the time during the treatment as less stressful.
Since there are sometimes multiple drugs to be taken for different symptoms, patients are wary of potential complications and side effects of the drugs. If the symptoms are unclear, the patient should contact a specialist or an emergency doctor.