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The differential diagnosis of lymphadenopathy is a classic problem in internal medicine, and different patients will have different answers. “Castleman’s disease” is a rare disease that causes lymphadenopathy. Although it is often included in our differential diagnosis framework, young doctors rarely encounter such patients during rotation, which is a challenge for doctors and patients. What is the medical treatment process for patients with rare diseases? Let’s hear her story.
This story comes from Casterman’s House – CD Gramophone Series Stories
Ordinary people’s accidents often come from diseases, Linlin living in Dongguan That’s it.
In August 2018, on the 50th day after giving birth to her son, the 29-year-old was frightened by the doctor when she went for a routine postpartum checkup: “You have a very large abdomen. Tumor, such a large retroperitoneal tumor is very likely to be malignant, so hurry to a higher-level hospital for a detailed examination.”
Linlin immediately thought of tumor and cancer. You know, she has been healthy since she was a child and has never been seriously ill. After giving birth, Linlin was entering a new phase of her life full of hope when the huge lump in her abdomen hit her on the head. She called her husband to rush to the hospital, but she was so panicked that her legs were weak and she could only squat in the corner.
Never heard of it, is it a terminal illness?
The next day, Linlin and her husband did not dare to delay, and went to one of the best local cancer hospitals for an examination.
Enhanced CT showed that there was a soft tissue mass in the abdomen, the size of a grapefruit, and it was close to the right kidney. Doctors said that no matter whether it is benign or malignant, it must be removed.
However, the surgical procedure was not as smooth as expected. The chief surgeon opened the abdominal cavity and found that the tumor and its surrounding blood vessels are very rich. It is extremely difficult to peel off the blood vessels and cut off the tumor. A little carelessness will cause heavy bleeding. The doctor immediately decided to change the originally planned complete resection to a biopsy, only taking out a small part of the mass for pathological examination, and the first operation was over.
“The pathological examination was done for more than a week, and more than 20,000 yuan was spent on various tests, but the diagnosis could not be confirmed.” The doctor finally brought the pathological section to the weekly clinic. During the general consultation for difficult diseases, she was diagnosed with Castleman disease (CD).
Castleman’s disease is also known as giant lymphadenopathy or angiofollicular lymphadenopathy. Pathological types can be divided into hyaline vascular type (HV-CD, Figure A-B), plasma cell type (PC-CD, Figure C-D) and mixed type (Mixed)[1]
“When I saw this string of English letters, I felt like my brain was struck by lightning. What kind of disease is this, I’ve never heard of it, is it a terminal illness?” She was afraid, and even thought: If I have something Unexpectedly, what to do with my son who was only a few months old.
In fact, compared to the patients that Linlin met later, her diagnosis process has been relatively smooth. This rare disease has been named for nearly 70 years [2], and many doctors still do not know much about it. Patients may be misdiagnosed, fall into treatment errors, blindly treat for several years, and travel to more than a dozen hospitals before they are finally diagnosed.
Castleman’s disease has a complex clinical classification. According to the distribution of lymph nodes and the site of organ involvement, the disease can be divided into unicentric CD (UCD)< /span> and clinical multicentric CD (MCD). UCD only involves a single lymph node area, and the systemic symptoms are mild. A small number of patients have lymph node compression symptoms and systemic symptoms. The prognosis is good and the possibility of cure is high. MCD involves multiple lymph node regions, and most patients have systemic symptoms, organ involvement, and high inflammatory state. The prognosis is poor, and it may recur, which affects long-term survival.
Clinical classification of Castleman disease[1]
< Lesion resection with the support of multiple disciplines, a 10-hour gamble
During this period of illness, Linlin found a group of patients with Castleman disease, including more than 200 patients. , We often exchange treatment experience.
“Meeting this group of brothers and sisters who are sympathetic to each other was my first turning point.”
Through the introduction of the patient group, Linlin went to the Department of Hematology of Peking Union Medical College Hospital to see a doctor. The doctor thinks that Linlin’s diagnosis of CD is relatively clear, and the possibility of single-center type is high. In the treatment of UCD, surgical resection is the main method, but Linlin’s disease is relatively large, surrounding the right kidney and blood vessels, and it is difficult to surgically remove it. You can try Systemic chemotherapy.
Considering that Linlin’s condition is not serious, she did not blindly accept chemotherapy and radiotherapy to cause physical damage. The doctor recommended a combination of cyclophosphamide, dexamethasone, and thalidomide, and re-examined after three months.
Thalidomide-based chemotherapy[3]
< >Linlin went home to take medicine and relax, and went out to travel. During a visit to Hong Kong a few months later, she noticed that her stomach suddenly swelled, as if there was something inside, so she immediately went home to the local hospital for examination. It turned out to be ascites at work.
“Our local doctor said that he had treated patients with Castleman’s disease and said that the chemotherapy effect of the other party was very good, and he wanted to arrange chemotherapy for me. But I already know a little about this disease and know that chemotherapy is not the best The choice, I just want to check the composition of the ascites, and the treatment still has to go to Beijing.”
After being hospitalized in a local hospital for a period of time, Linlin went to Beijing alone, planning to follow the doctor’s instructions. suggested to try surgery.
It was difficult to wait for beds in Union Medical College Hospital. Linlin waited for two months in Beijing before being admitted to the hospital, and the life was quite difficult. Her serum albumin level was low, her body was weak, her body was thin, and there was a lot of ascites in her stomach, just like in the fourth or fifth month of pregnancy. …
Finally, Linlin waited for the bed. The ward’s attending doctor heard that she was a patient with Castleman’s disease, and was interested in talking with her for a long time. He also believed that the excision surgery might give Linlin a better prognosis, but there was no data to support this statement at the time.
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After a For a series of preoperative evaluations, the husband left his job and rushed to Beijing to sign the consent form for the operation. This consent form is no different from a life-and-death gambling contract, because the operation is extremely dangerous. Before signing the consent form for surgery, experts from the departments of interventional medicine, hematology, basic surgery, vascular surgery, urology, blood transfusion and other departments conducted multi-disciplinary consultation to discuss the operation plan .
The doctors told Linlin very bluntly: “This is a very difficult operation, because the tumor is very large, close to the kidney, In addition, the surrounding area is covered with blood vessels, and the right kidney may be removed or artificial blood vessels may be used during the operation, which means that it may be possible to take medicine for life after the operation. In addition, because of the severe abdominal adhesions that have been done once, the operation will be more difficult. Difficulty, there may be heavy bleeding during the operation, and it may even be impossible to continue.”
“I was very afraid of removing a kidney at the time, but I was determined to perform the operation. They are also very firm, so nothing they say can get into my ears, I only know that surgery must be done.” Linlin understands that this step has to be taken, and only after this step can she have a future life.
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Because of the abundant vascular distribution of the tumor, Linlin went to the interventional department for a vascular embolization before undergoing resection to reduce the blood flow of the tumor. supply to reduce the possibility of major bleeding. Despite this, the operation continued from 11:00 am to 9:00 pm, and there was still heavy bleeding, and even the 2000cc of blood that had been prepared in advance was used up. Fortunately, the operation was successful and the mass was completely removed.
Out of the operating room, Linlin was immediately sent to the ICU. “After I woke up, I had a tube in my mouth and couldn’t speak. With difficulty, I tapped the hospital bed with my hands to call the nurse, and asked vaguely: ‘Is my kidney still there?’ The nurse looked through the operation records and gave me a positive answer. , I finally let go of my hanging heart.” The kidneys are still there, but a small section of the colon has been cut off, so I can’t eat or drink for 7 days. This feeling is like being thrown into the desert.
“This surgery was my second turning point.”
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After the mass was completely excised, the ascites gradually decreased until it disappeared, Linlin’s physical strength gradually recovered, and no recurrence was found in the re-examination CT.
“We have achieved a phased victory and may be able to achieve a complete cure”.
The road ahead is long, there is hope when we walk together
on the MyCgChasing website Disease “warrior”
After being diagnosed with Castleman disease, Linlin and her husband help doctors to translate international medical information and research literature from time to time. Her role has gradually changed, no longer just a patient, but began to help some patients within her capacity. The second time she went to Beijing for reexamination after the operation, the doctor knew that she had joined a patient group and invited her to serve as a researcher to complete a patient survey.
Later, this survey was frequently used in many disease-related studies. The results showed that the number of CD patients was equal in men and women, and 33.49% of the patients needed at least one year from onset to diagnosis. 54.25% of the patients were misdiagnosed, and 75% of the patients were diagnosed in large tertiary hospitals in first-tier cities. After receiving the treatment, 93.1% of the patients had adverse reactions of varying degrees; 56.41% of the patients had a decrease in the treatment effect within 12 months after the first treatment. More than half of the patients expect that the only MCD-targeted drug stuximab, the only MCD-targeted drug approved abroad, will be launched in China as soon as possible.
On April 25, 2021, my country’s first public service platform for Castleman disease patients “Castleman’s Home”
span> established. Linlin was invited to be the principal, responsible for daily operations, including WeChat public account, patient community, etc., daily pushes the popular science knowledge and cutting-edge research information of Castleman disease, and regularly invites experts to hold online live broadcasts and offline free clinics. Answering questions for patients and extending a helping hand to some patients trapped in the quagmire.
Lin Lin, the principal, shared at the inaugural meeting of “Castman House”
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In 2021, more than 40 experts from all over the country jointly established the “China Castleman Disease Collaborative Group”, and in September The “Chinese Expert Consensus on the Diagnosis and Treatment of Castleman’s Disease (2021 Edition)” was formulated. This consensus elaborates the pathological features, clinical classification, diagnostic process, prognosis and risk stratification, treatment plan, and efficacy evaluation of Castleman disease in detail. [4]
The diagnosis and treatment of rare diseases such as Castleman disease still have obvious uneven distribution of resources. . It is believed that the establishment of “Castleman’s Home” and “China Castleman’s Disease Collaborative Group” can improve this situation to a certain extent.
Expert Comments
Linlin’s determination and determination in the face of the disease The behavior of actively helping others after good control is very admirable. The Castleman disease she unfortunately suffered from is a rare disease of the blood system included in the “First Batch of Rare Diseases List” of the National Health Commission. The clinical type of the patient in this article is single-center CD, but she also has many similar multi-center diseases. Hyperinflammatory manifestations and systemic symptoms of type CD.
For patients with monocentric Castleman disease, regardless of whether the patient has high inflammatory manifestations similar to multicentric Castleman disease, it is recommended to create conditions for complete resection of the lesion to achieve cure. For patients with symptomatic multicentric Castleman disease, since the internationally widely used targeted therapy (stuximab) has not been officially launched in China, relatively low-intensity continuous therapy is currently recommended. Thalidomide-based regimen is a promising chemotherapy regimen [5]. Stuximab is the only targeted drug approved by the US FDA for the treatment of multicentric Castleman disease so far. It is currently widely used in dozens of countries around the world, and will be launched in China in the near future. Provides an important treatment option.
Deputy Chief Physician Zhang Lu, Department of Hematology, Peking Union Medical College Hospital
Dedicated to the clinical, scientific research and popularization of Castleman’s disease for many years
Rare is many. Rare is strong. Rare is proud.
Rare disease is not alone , rare is powerful, proud of being rare.
References
1. Dispenzieri A, Fajgenbaum DC. Overview of Castleman disease. Blood. 2020;135(16):1353-1364.
2. CASTLEMAN B, TOWNE VW. Case records of the Massachusetts General Hospital: Case No. 40231. N Engl J Med. 1954;250(23):1001-1005.
3. van Rhee F, Stone K. Storming the Castle with TCP. Blood. 2019 Apr 18;133(16):1697-1698. doi: 10.1182/blood-2019-02-900803. PMID: 31000513.
4. Chinese Society of Hematology Lymphocyte Disease Group, Hematology and Tumor Professional Committee of China Anti-Cancer Association, China CASTLEMAN Disease Collaborative Group. Chinese Expert Consensus on the Diagnosis and Treatment of Castleman Disease (2021 Edition) [J]. Chinese Journal of Hematology, 2021,42(7):529 -534.
5. Zhang L, Zhao AL, Duan MH, Li ZY, Cao XX, Feng J, Zhou DB, Zhong DR, Fajgenbaum DC, Li J. Phase 2 study using oral thalidomide-cyclophosphamide-prednisone for idiopathic multicentric Castleman disease. Blood. 2019;133(16):1720-1728.
Author:Linlin CD Patient Casterman Home Manager
Reviews: Zhang Deputy Chief Physician, Department of Hematology, Peking Union Medical College Hospital
Editor:Casamine Tea< /p>
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