Nigerians were consumed with grief over the unexpected and tragic news of the death of Sound Sultan, a celebrated singer, actor, and entertainer. According to the family source who broke the news, the musician whose real name is Olanrewaju Ganiu Fasasi died on Sunday, 11 July 2021 after a battle with cancer. He died aged just 44.
It was revealed that Sound Sultan succumbed to a rare form of cancer called angioimmunoblastic T-cell lymphoma.
What is angioimmunoblastic T-cell lymphoma, and why is it so fatal?
Let us start by understanding the disease and possible causes. Angioimmunoblastic T-cell lymphoma is a nonspecific and peripheral T-cell lymphoma, causing high fever and large lymph nodes.
Lymphomas are cancers affecting the lymphatic system (white blood cells). Lymphomas can be B-Lymphomas or T-Lymphomas, depending on the cell type.
Since the lymphatic system is an important part of the immune system, any disease affecting it will predispose the body to a host of potentially life-threatening infectious diseases.
This disease has no definite symptoms, but varies from person to person. It progresses aggressively and can affect all organs. The symptoms exhibited by patients are similar to that of other diseases. High and persistent fever is an early symptom.
As the disease progresses, other symptoms such as weight loss, malaise, enlarged liver, and immune system glands occur. The lymph nodes of the armpit, neck, and groin also enlarge. Fluid accumulation in the lungs can occur.
Patients also tend to have skin rashes that can be itchy. There is also the destruction of red blood cells (autoimmune hemolytic anemia) causing fatigue, dark urine, pale skin, and shortness of breath.
People between 40 to 90 years form the major population of those with the disease. It is rare for children.
Angioimmunoblastic T-cell lymphoma is very lethal, with a survival rate of less than 3 years even with intensive treatment.
What are the Causes?
The causes of this disease are not yet known. It is, however, believed to be due to a disorder in the immune response to an antigen (antigen induces the body to produce antibodies)
There are also suggestions that they are caused by the Epstein-Barr virus (This cancer has been found in 90% of patients with the viral disease. However, Epstein Barr virus affects B cells, not T cells, unlike angioimmunoblastic T-cell lymphoma).
Other suspected causes are cytomegalovirus, hepatitis C virus, HIV, human herpesvirus 6 and 8. Research has also linked angioimmunoblastic T-cell lymphoma with some genes such as DNMT3A, TET2, and substances such as endothelial growth factor A (VEGF-A) that is responsible for tumor angiogenesis.
Can it be treated?
This disease can be managed with the hope of full remission. A team of professionals such as oncologists (cancer specialists), hematologists (blood disorder specialists), and radiation oncologists (specialists that use radiation to treat cancer) is necessary.
The treatment will depend on the size of the tumor, stages of cancer (Ann Arbor staging is mostly used, classifying it into Stages I, II, III, and IV), age of the patient. In the management, doctors used an approach called “watch and wait”. This is waiting for patients to develop certain symptoms before treating the patient.
Drugs such as corticosteroids (prednisolone is used mostly) and procedures such as chemotherapy have been used to treat it. Combination therapy of several anticancer drugs is used. A particular regimen called CHOP is mostly recommended (Cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisolone used)
The medication can be followed up with autologous stem cell transplantation, and it has shown promising results.
There is no effective treatment of this disease, as most patients experience relapse after remission. However, there is promising evidence that drugs such as cyclosporine A, interferon A, and another drug romidepsin recently approved by FDA will be more effective in the future.