There are diseases that have symptoms of immune diseases and cancer. It is a rare disease called multicentric castellar disease (MCD). The cumulative number of patients with multiple Castleman disease in Korea is about 700 in 2019, and the number of new patients is only 100 to 150 per year. It is a difficult and rare disease to diagnose, with only about 1,650 cases reported each year in the United States.
A large number of patients with multiple Castleman’s disease experience diagnostic wandering before a definitive diagnosisㅣSource: Getty Image Bank
Multiple Castleman’s disease is a pre-stage disease of lymphoma, a type of blood cancer. Interleukin-6, an immune protein that regulates the body’s immune and inflammatory responses, is overexpressed, causing the immune system to characterized by attacks on major organs. It can occur anywhere there is a lymph node and there are symptoms such as severe chronic fatigue △ weight loss △ fever △ night sweats △ enlarged organs such as the liver and spleen △ skin changes △ neuropathy, which makes everyday life impossible . swollen lymph nodes. . Sometimes △anemia △thrombocytopenia △proteinuria △ nephrotic syndrome appear together.
Although multiple Castleman’s disease is not cancer, it is known as a disease with a poor prognosis like cancer. According to various studies, approximately 27% of patients are diagnosed with cancer within 2 to 5 years of diagnosis, and approximately 35% of patients with idiopathic Multicentric Castleman disease (iMCD) die within 5 years of diagnosis . For information, the mortality rate for colorectal cancer patients is 33%.
The problem is that the median survival time (the median value when the target patients’ survival times are listed) is only 14 to 30 months, a disease with a poor prognosis, but it takes too long to w diagnosed because it is a rare disease, that is. It takes an average of 27.5 months and about 3.8 years to be diagnosed, but it is difficult to differentiate because the symptoms are popular and common.
For this reason, multiple Castleman’s disease is also a representative disease that causes ‘diagnostic wandering’, where patients move around various departments and medical institutions to find the cause. According to statistics from the Korea Centers for Disease Control and Prevention, about 16.4% of patients attended four or more hospitals from the onset of symptoms to the final diagnosis. Experts said, “Multiple Castleman disease is a difficult disease to diagnose, but unlike other rare diseases, it is a disease that can be treated quickly and accurately with a standard treatment guideline.” The diagnosis of multiple Castleman’s disease requires close collaboration between the pathology department and the hemato-oncology department.
The first-line standard treatment guideline currently used for multiple Castleman disease was established by the Castleman Disease Collaborative Network (CDCN) in 2018, and the standard treatment, Silvant (siltuximab), has also been covered by health insurance since February 2018. It is recognised, and the burden felt by the patient is less. If the treatment is given for more than 6 months, the symptoms improve quickly to the extent that it is possible to return to everyday life.
