Swollen lymph nodes, swollen hands and feet, tiredness, and inflammation all over the body. The 35-year-old hairdresser went to the doctor repeatedly and could not find the cause. At first, she thought it was chronic anemia, or a lymphatic-related disease. It was also speculated that it might be an immune disease. After various examinations, the final biopsy was found to be Castleman’s disease. Zhang Mingzhi, a senior attending physician in the Department of Hematology and Oncology of Mackay Hospital who treated the case, recalled that because the disease was so rare, it took six months to diagnose it. Fortunately, it was almost cured after using IL-6 targeted therapy drugs. Medicines must be taken for life to maintain health.
Castleman’s disease is rare, hard to diagnose, 30% fatal within five years
Castleman’s disease is a disease of abnormal lymph node hyperplasia. According to the prevalence rate, there should be 100 people diagnosed every year in Taiwan, but in fact, only 20 to 30 people are diagnosed a year. It is quite rare, mainly because of many Physicians are not very familiar with this disease, even if there are symptoms, it is difficult to judge immediately.
It is divided into three subtypes according to the location and extent of the proliferation, and a small part is related to HIV virus and herpes virus. One of the subtypes is “idiopathic multicentric Castleman disease (idiopathic multicentric Castleman disease, iMCD) )” is still unknown and difficult to diagnose.
Dr. Zhang Mingzhi pointed out that Castleman’s disease (iMCD) is basically between benign and malignant, but it may develop into lymphoma; 80% of patients will not progress too much, but because of repeated inflammation in the body and exhaustion of energy , can also cause various symptoms, which greatly affects life, and 30% of patients will still die within five years.
Excessive cytokine IL-6 causes immune storm
“It’s a bit like the cellular immune storm we mentioned during the COVID-19. This disease is also caused by the ups and downs and excessive secretion of the cytokine interleukin 6 (IL-6), which leads to the immune storm and many symptoms.” Dr. Zhang Mingzhi explained, introducing Excessive concentration of white matter 6, a cytokine, can cause lymphadenopathy, fever, night sweats, fatigue, and weight loss. It can also cause ascites, pleural effusion, or enlarged spleen and liver due to mucous membrane reactions.
It usually occurs between the ages of 30 and 50. Dr. Zhang Mingzhi pointed out that relatively older people are less prone to immune storms, and younger people’s immune systems are more likely to respond. Since only 3 to 4 people in a million people get sick, it is difficult been diagnosed.
It takes an average of six months for multiple Castleman’s disease (iMCD) to be diagnosed These indicators should pay attention
“Multiple symptoms are like a girl with thousands of faces. It usually takes six months to diagnose.” Dr. Zhang Mingzhi said frankly, usually the first thing to suspect is whether it is cancer? Because fever can also be suspected as infection, blood drawing may be judged as anemia, and since there are no special markers to identify, lymphatic biopsies will also be diagnosed as benign inflammation, and the key role of diagnosis falls on pathologists.
Multiple Castleman’s disease (iMCD) does not progress quickly, but there are several indicators to pay special attention to:
1. Immunoglobulin will rise inexplicably
2. Decreased albumin but no proteinuria
4. Decreased red blood cells
5. C-reactive protein (CRP) is too high
6. Blood sedimentation rate is extremely high
7. Since the liver is also affected, alkaline phosphatase also rises
Although there is no immediate threat of death in multiple Castleman’s disease, mild cases can survive for a long time without incident, but Dr. Zhang Mingzhi emphasized that there is a 1/4 chance that it may be severe. If it is not treated, it may develop into lymphoma, and even death.
IL-6 targeted therapy inhibits cytokines and is expected to achieve complete remission
The threat of this disease is that it is difficult to diagnose, and it is impossible to prescribe the right medicine, and the wrong medicine may also cause adverse effects. Dr. Zhang Mingzhi said that in the past, when there were no drugs, it could only be treated as follicular lymphoma, but the improvement was limited. At present, there are drugs targeting interleukin-6 receptors in Japan, and Taiwan also has IL-6 targeted therapy that directly inhibits interleukin-6.
In 2014, Dr. Zhang Mingzhi published a clinical trial on IL-6 targeted therapy. Among the four testers, three responded well. The aforementioned hairdresser also achieved complete remission after 2 to 3 years of treatment. He mentioned that the use of drugs About 80% of patients will improve, and there will be significant improvement in about 4 to 6 months, and they need to be taken for life.
Physicians reminded that if severe multiple Castleman’s disease is not treated, renal function deterioration, hepatosplenomegaly, pulmonary edema, and even life-threatening, remind the public and the medical community to be vigilant, and also call for the use of drugs Pay through health insurance as soon as possible to reduce the burden on patients.