Treating Autoimmune lymphoproliferative syndrome (ALPS)




Treating Autoimmune lymphoproliferative syndrome (ALPS)


Understanding Autoimmune diseases.

Autoimmune diseases are rare diseases that affect the human immune system. Our Immune system guards us against external aggression, say bacteria or any other foreign cells. Autoimmune diseases occur when the immune system fails to distinguish between healthy tissues and harmful antigens thus destroying body tissues by mistake. It releases autoantibodies that end up attacking healthy body cells. There are more than 80 types of autoimmune disorders and ALPS is one among them.


What is ALPS?

ALPS or Autoimmune lymphoproliferative syndrome is a rare genetic disorder that creates an excessive number of lymphocytes (lymphoproliferation), causing enlargement of lymph nodes and spleen. This excessive number of lymphocytes may spread to other parts of the body other than lymph nodes or spleen, particularly blood cells which may lead to possible cancer.



Signs and symptoms

ALPS can affect all age groups and predominantly women. The main symptoms are enlarged lymph nodes and spleen.  Enlargement may become severe in children. Swollen lymph nodes in the neck, armpit, and groin are the most noticeable symptoms.

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Patients with ALPS will show tiredness and pallor, easy bruising, and bleeding as their red blood cells and platelets are attacked by the antibodies. They may also get affected by Lymphoma or cancer of the blood cells. The symptoms are fever, fatigue, loss of appetite, and sudden weight loss. Lymph nodes will get enlarged.


Diagnosis

The diagnosis of ALPS may become a long process as the symptoms associated with it are more like other medical conditions. In other words, many medical conditions have similar symptoms and hence it requires a careful evaluation. Consulting a general physician is enough in the initial stages with routine blood tests, looking for different types of lymphocytes. If a strong chance of ALPS is suspected, the need to get evaluated with genetic tests, scans, and advanced blood tests by specialised physicians. To rule out the chance of lymphoma, the physician may suggest a small biopsy.


Treatment

In most cases, doctors do not prescribe any medicines but require monitoring of the blood cells and the size of their glands. Regular blood checks are all it requires. Some patients may have to take medication for the overgrowth of lymphocytes. Immunosuppressive treatment is available according to the patient’s immunity and needs. In severe scenarios, the replacement of abnormal lymphocytes by hematopoietic stem cell transplantation might be prescribed.


ALPS may not respond well to medicines or immunosuppressants as the symptoms will recur. If Blood transfusion is prescribed,it is recommended to take ABO & D blood test. This blood check will help to determine blood type. Study shows that antimalarial drugs pyrimethamine and sulphadoxine can help reduce lymphadenopathy.





Prognosis

The unfortunate part of this disease is that there is no long-term follow-up or clinical treatment available. This disease has been identified only recently. But researchers are confident of finding one with the help of research on the medical history of patients and their family history. Death due to ALPS or related diseases usually arises when there is a development of haematological malignancy or the complication of severe autoimmune disease or post-splenectomy sepsis


Only 50% of patients with ALPS require immunosuppression as a treatment. The important goal of the treatment is to resolve life-threatening cytopenia and avoid splenectomy. Short-duration treatment with a high dose of corticosteroids proved useful. 


Both steroid and steroid-sparing treatment should be supplemented with corticosteroids.


Managing Lymphoproliferation

Patients suffering from ALPS show a considerable amount of anxiety and the children can be socially ostracised.  It is essential to help the patients not get depressed due to this. A better way of controlling ALPS is to treat resultant health issues the patient is facing. Continued surveillance is the key. Regular check-up of lymph nodes, spleen to check splenomegaly and checking for lymphadenopathy is beneficial. 


Please remember to document regular check-ups as it is required to devise a treatment plan. 

Unlike other diseases, the symptoms and impact of ALPS may vary from person to person and hence we cannot make a general way of managing the disease. So the doctor plans a treatment according to the patient’s detailed test history. Should require a long-time support of a Rheumatologist or Gastroenterologist to see the progression.


Conclusion

Because of a limited understanding of the disease, there is no effective cure till today. So the key is early detection, and not allowing it to become fatal. The second important requirement is to have continued, regular check-ups.  For some patients, the medication prescribed as part of treatment, particularly antibiotics may create some side effects.  In such cases, discuss it with the healthcare provider so that medicine can be changed.  Last and important thing of this treatment is to encourage the patient into confidence, be positive, follow healthy lifestyles, avoid junk foods, and regular exercise. This will reduce the patient’s fear and depression with  improved immunity and confidence to face the challenge.


Written for Kent Winter

Magnizent Digital Solutions


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