Castleman Disease (CD)

What is Castleman Disease?

Castleman disease is a rare, nonclonal lymphoproliferative disorder, meaning immune cells in the lymph nodes multiply abnormally without being a true cancer. It can affect lymph nodes in the neck, chest, abdomen or pelvis and can look like other benign or malignant conditions.^[1]

It is divided into unicentric disease (one lymph node area) and multicentric disease (several areas), and the multicentric type includes forms linked to the virus HHV-8 (also called KSHV), forms with unknown cause (idiopathic) and forms linked to POEMS syndrome.^{[2, 3]}

Common symptoms

Unicentric disease usually causes a solitary enlarged lymph node with mild symptoms.^[2] Multicentric disease is more often systemic, and the HHV-8 and idiopathic forms share many features such as constitutional symptoms and organ dysfunction.^[4]

• Enlarged lymph nodes in one or several areas
• Fever, night sweats, fatigue and weight loss
• Anemia, low platelets, fluid build-up or swelling
• Enlarged liver or spleen, and kidney problems in some people^{[4, 5]}

Causes and risk factors

The cause of idiopathic multicentric disease is not fully known, and a cytokine storm that often involves interleukin-6 is thought to drive it.^[5] The HHV-8 form often occurs with HIV infection or another cause of immune deficiency.^[2]

How it is diagnosed and treated conventionally

Diagnosis needs a multidimensional approach that combines clinical findings, imaging, lymph node biopsy and, where relevant, tests for the virus.^[2] International consensus criteria exist for the idiopathic multicentric form, and they also require excluding infections, cancers and autoimmune disorders that can mimic it.^[5]

Surgical removal of the affected node is often curative in unicentric disease.^[2] For HHV-8 multicentric disease, antibody therapy against B cells such as rituximab is standard care, and for idiopathic multicentric disease, anti-IL-6 therapy is the recommended first-line treatment and the only one approved by the US FDA and EMA.^[2] Refractory cases need additional therapies.^[3] The anti-IL-6 antibody siltuximab was approved for idiopathic multicentric disease in the past decade.^[6]

Why Castleman Disease calls for a whole-person approach

Castleman disease is not a single illness, and its type decides the treatment, so medical classification always comes first. Beyond the disease itself, people often live with fatigue, pain, poor sleep and the side effects of treatment.

Supportive care aims at those symptoms. It does not change the disease course.

Systemic inflammation

In multicentric disease, inflammatory signaling, especially interleukin-6, drives fever, fatigue and organ problems.^{[2, 5]} This is why anti-IL-6 treatment is used, and why constitutional symptoms are taken seriously.

Fatigue and weight loss

Fatigue, night sweats and weight loss are among the most common complaints in the multicentric forms.^[4] Fatigue also occurs in many people with cancer, and it has been studied in that setting (see the acupuncture section).

Immune suppression and infection risk

Some forms occur in people with HIV or other immune deficiency, and treatment can further affect immune function.^[2] This matters for any complementary therapy, because infection risk and drug interactions need to be discussed with your physicians.

Sleep, pain and emotional strain

A rare, serious diagnosis can bring pain, disturbed sleep and worry. These symptoms have been studied in people with cancer, and the findings are described below as indirect evidence only.^[7]

Acupuncture as supportive care in Castleman Disease

What the research shows

A PubMed search found no clinical trials of acupuncture for Castleman disease, so there is no evidence that it affects the disease. What exists is evidence for symptoms in people with cancer, which is a different group and may not apply.

An overview of 51 systematic reviews of acupuncture for cancer-related conditions found reported benefit for cancer pain, fatigue, insomnia, nausea and vomiting and quality of life. The authors rated nearly all of the reviews as low or very low in methodological quality and called for better trials.^[7] A meta-analysis of 17 trials found acupuncture or acupressure was associated with less cancer pain, including in 7 high-quality sham-controlled trials, although the evidence was graded moderate because results varied widely between studies.^[8]

An updated meta-analysis of 9 trials (809 participants) found lower fatigue scores with real acupuncture than with sham and than with usual care.^[9] A review of cancer-related insomnia found improved sleep compared with a wait-list in women with breast cancer on treatment, but real and sham acupuncture improved sleep to a similar degree, and the authors called for larger, more rigorous trials.^[10]

How acupuncture may work

How acupuncture might ease these symptoms is not established, and no study has examined its effect on lymph node disease or interleukin-6 in Castleman disease. We do not claim that acupuncture changes the immune process behind the condition.

What treatment involves

Treatment uses fine, sterile, single-use needles, typically left in place for about 20 to 40 minutes, with a short course of visits and reassessment. Points are chosen conservatively when blood counts are low or the immune system is affected.

In prospective studies, about 9 in 100 patients had a minor reaction such as soreness or bleeding at a needle site, and serious events occurred in about 1 in 10,000.^[11] Tell the practitioner first if you have low platelet counts, a bleeding disorder, take blood thinners, have a pacemaker, are pregnant, have a fever or infection, or have swelling from fluid build-up.

Ayurvedic medicine as supportive care in Castleman Disease

The Ayurvedic view

Ayurveda has no classical name for Castleman disease, which was first described in 1954.^[3] A practitioner would instead assess fatigue, digestion, sleep and other symptoms, and might describe them in terms of the doshas and agni (digestive fire). These are traditional frameworks, not biomedical diagnoses.

Therapies that may be used

Care is individualized and may include:

• Diet and daily routine aimed at regular meals, rest and steady sleep
• Gentle oil massage and relaxation practices for stress
• Herbs, if appropriate, prescribed individually after review of your medicines

Panchakarma is not appropriate during acute illness, in frailty, in pregnancy, or when someone has fever, anemia, low platelets, organ involvement or immune suppression, all of which can occur in multicentric disease.^{[2, 4]} It should not be considered for active multicentric disease or during active treatment unless your treating physician agrees.

What the research shows

No clinical studies of Ayurvedic treatment for Castleman disease were found in PubMed. Ayurveda cannot be presented as a treatment for the disease or as a way to avoid medical therapy.

Herbal medicine as supportive care in Castleman Disease

Herbs and formulas that have been studied

The only report found is a single case study from China. It described a patient with recurrent lymph node enlargement treated for nearly three years with a combination of oral, topical and intravenous traditional Chinese medicine aimed at "strengthening the body, resolving phlegm and detoxification".^[12]

What the research shows

A single case report cannot show that a treatment works. The authors reported that the nodes could no longer be felt and symptoms disappeared, but one patient with no comparison group provides no reliable evidence, and we do not carry over its conclusion that the treatment is safe and effective.^[12] Other explanations, such as the natural course of the disease, cannot be ruled out.

No clinical trials of herbal medicine for Castleman disease were found.

Safety and herb-drug interactions

Herbs are active substances and can interact with medicines. Case reports describe bleeding when warfarin was combined with herbs such as dan shen, dang gui, ginseng and Boswellia.^[13] A literature review reports that St John's wort lowered blood levels of the transplant drugs ciclosporin and tacrolimus, and in some cases this led to organ rejection.^[14] Immunosuppressive and targeted drugs used for Castleman disease may be affected by similar mechanisms, so any herb needs review by your physicians first.

Herbal and dietary supplements are a recognized cause of liver injury.^[15] About one fifth of Ayurvedic medicines bought online contained detectable lead, mercury or arsenic.^[16] Please tell us about every medicine and supplement, and whether you are pregnant or breastfeeding.

Translating traditional medicine into modern biological language

This section is interpretation, not equivalence. Chinese medicine might describe the swollen nodes as accumulation of phlegm and toxin with underlying deficiency, as in the case report, and Ayurveda might describe aggravated kapha with weak digestion.^[12] Researchers studying Castleman disease instead measure abnormal lymph node tissue, cytokines such as interleukin-6, viral infection and organ dysfunction.^{[2, 5]}

No study has shown that a traditional pattern corresponds to any of these processes. The meeting point is symptoms such as fatigue, pain and sleep, which have been studied in cancer care.^[7]

How this care fits with your medical care

Castleman disease needs diagnosis and ongoing care from physicians, often a hematologist or oncologist, and sometimes an infectious disease or rheumatology specialist. Complementary care is supportive only and never replaces surgery, siltuximab, rituximab, steroids, antiviral therapy or monitoring. For a condition like this, we ask that you are under a physician's care for it and that your treating team knows you are receiving complementary care.

Please do not stop or change prescribed treatment without your prescriber. Bring your diagnosis and type, recent blood counts, imaging and biopsy reports, and a full list of medicines and supplements.

When to seek urgent medical care

Multicentric Castleman disease can cause severe inflammation and organ dysfunction.^[5]

• Fever with shaking chills, or any fever while on immune-suppressing treatment
• Shortness of breath, chest pain or swelling of the legs or abdomen
• Fainting, confusion or very low blood pressure symptoms such as severe dizziness
• Unusual bleeding, easy bruising or black stools
• Rapidly enlarging lymph nodes, or fast, unexplained weight loss
• Reduced urine output or sudden swelling

If any of these occur, call 911 or go to the nearest emergency department.