We have found the information in these two articles reputable and helpful in answering some of our questions and/or just explaining Castleman Disease. The articles are a bit long but do a good job explaining about the disease. The first one is from the American Cancer Society. The second article is from the International Castleman Disease Organization and is a bit more technical. Hope you find these articles as informative as we have.
Article One from the American Cancer Society: "Castleman disease (CD) is a
disease of lymph nodes and related tissues. It was first described by Dr.
Benjamin Castleman in Boston in 1956. CD is also known as giant lymph node
hyperplasia and angiofollicular lymph node hyperplasia. Although not officially
a cancer, this disease can act very much like lymphoma (cancer of lymph nodes).
In fact, many people with this disease eventually develop lymphomas. This is
why it is included in the American Cancer Society’s cancer information database.
Instead of being called a cancer, CD is often called a lymphoproliferative
disorder. This means there is an abnormal overgrowth of cells of the lymph
system that is similar in many ways to lymphomas. Like lymphoma, CD is
frequently treated with chemotherapy or radiation therapy.
Lymphoid tissue, also known as lymphatic tissue, is the main part of the
immune system. It is formed by different types of cells that work together to
resist infections. Lymphoid tissue also has a role in the body's resistance
against some forms of cancer. The main cell of lymphoid tissue is the
lymphocyte. There are 2 types of lymphocytes: B cells and T cells.
Lymphoid tissue is found in many places throughout the body, including:
• Lymph nodes (bean-sized collections of immune system cells found clustered
in the groin and underarm areas, on the sides of the neck, inside the chest,
and scattered widely though the body including the abdomen)
• Thymus gland
• Spleen
• Tonsils and adenoids
• Bone marrow
Types of Castleman disease:
CD is separated into 2 groups: localized/unicentric and
multicentric/systemic. They affect people very differently.
Localized Castleman disease:
Localized or unicentric CD only affects a single set of lymph nodes. It is
not widespread. The lymph nodes in the chest and abdomen are affected most
often. CD causes these lymph nodes to enlarge. These abnormally large lymph
nodes may press on other organs and tissues inside the chest or abdomen. If
they are in the abdomen, the person might feel pain or pressure in that area.
Enlarged lymph nodes in the chest can press on the windpipe (trachea) or
smaller breathing tubes (bronchi) causing breathing problems. Sometimes the
enlarged lymph nodes are in places such as the neck, groin, or underarms and
can be felt easily. People with localized CD disease are usually cured when the
lymph node is removed with surgery.
Microscopic subtypes of Castleman disease:
Castleman disease can also be classified as either a hyaline vascular type
or a plasma cell type based on how the lymph node tissue appears under a
microscope (these are called microscopic subtypes). Less often, a combination
of both types may occur. The hyaline vascular type is more common and tends to
be localized, while the plasma cell type tends to be multicentric, but
exceptions to this rule often occur. In choosing treatments, doctors feel that
the microscopic type is less important than whether the disease is localized or
multicentric.
We don't know how many people are diagnosed with Castleman disease (CD) each
year. The National Cancer Institute has a program that keeps track of how many
people have each type of cancer. Because CD is not a cancer, it is not included
in these records. We do know that CD disease is very rare in healthy people. In
fact, 2 of the leading cancer centers in the United States, MD Anderson and
Memorial Sloan-Kettering, each see about 2 patients a year.
A risk factor is anything that might change a person's chance of getting a
disease. But risk factors don't tell us everything. Having a risk factor, or
even several, does not mean that you will get the disease. Having no risk
factors doesn't mean you won't get the disease.
Most patients with Castleman disease (CD) have no known risk factors. In
general, most people with localized CD disease feel well otherwise.
No one has discovered the cause of this disease, but many doctors suspect a
virus is involved.
Doctors suspect that problems with the way a patients immune system is
working may contribute to the development of CD. Many people with CD have
abnormally high blood levels of certain substances produced by immune system
cells.
Some scientists believe that CD occurs when there is too much of a protein
called interleukin-6 (IL-6). IL-6 is a protein the body makes to help regulate
immune function. Too much IL-6 seems to cause lymphocytes to reproduce
excessively.
Treatment of localized (unicentric) Castleman disease:
Surgery is the recommended treatment for people with localized Castleman
disease (CD). Removing the abnormal lymph node(s) appears to cure the disease.
Symptoms such as fever and fatigue that are caused by the CD go away when the
lymph node is removed. Relapses are rare. Radiation can also cure localized
disease.
Some patients with localized CD develop secondary amyloidosis, a condition
in which abnormal proteins build up in the kidneys, skin, and some other
organs. This protein build-up stops once the lymph node affected by CD is
removed.
The outlook for localized CD is very good if the affected lymph node can be
removed with surgery. But sometimes the surgeon cannot safely remove all the
disease. This doesn't necessarily mean it will come back. Even partial removal
may help and the disease may not grow back.
Frequent follow-up exams are very important for several years after the
treatment for Castleman disease (CD) is finished. The doctors will continue to
watch you for signs of recurrent disease."
Article Two from the International Castleman Disease Organization: "Castleman disease is a benign disorder first described by
Dr. Benjamin Castleman in 1956. Castleman disease is also referred to as
angiofollicular hyperplasia, and is non-clonal disease of the lymphnodes. As
the name angiofollicular hyperplasia (1) implies there is a follicular
hyperplasia of lymph nodes with abnormally increased interfollicular
vascularity. Castleman disease can be classified as a) unicentric vs.
multicentric, based on clinical and radiological findings, b) hyaline vascular
vs. plasmacytic vs. mixed cellularity variety based on histopathology.
Unicentric Castleman Disease is usually a slow growing
solitary mass typically located in the mediastinium or mesenteries. There are
no constitutional symptoms and no elevation of acute phase reactants
(Interleukin 6, ESR and CRP). Symptoms
if present are due to a mass effect of bulky lymphadenopathy. In 90-95% cases
surgical resection is curative and usually there is no progression to lymphoma
or association with other tumors. The prognosis is excellent with a 5 year
survival of close to 100%.
The histopathology of hyaline vascular Castleman disease
shows that the lymphnode germinal centers are poorly formed with dysplastic/
atrophic CD21+ follicular dendritic cell networks surrounded by an expanded
mantle zone consisting of rims of small CD20+ lymphocytes arranged in an onion
skin manner. There is increased
interfollicular vascularity with capillary proliferation and endothelial
hyperplasia.
Classically it is thought that Unicentric Castleman disease
is usually of the hyaline vascular variety and multicentric disease of the
plasmacytic type or mixed cellularity variety. These relationships are based on
the analysis of the small numbers of patients since Castleman disease is rare.
Review of 37 patients with Castleman disease treated at UAMS, which is the
largest single institution experience in the world with Castleman Disease. We
found that patients with Unicentric Castleman disease indeed have as pathology
the hyaline vascular variety. However, the histopathology of multicentric
disease can be evenly divided between hyaline vascular variety on one hand and
plasmacytic type and mixed cellularity variety on the other hand. Mixed
cellularity clinically behaves more like plasmacytic type rather than hyaline
vascular disease.
Surgical excision is the preferred treatment in most cases
of unicentric Castleman Disease and adjuvant therapy e.g. steroids and/or
rituxan before surgery is very useful to shrink bulky or inoperable disease. In
some cases, radiotherapy has proven effective.
In most cases of the hyaline-vascular type of Castleman
Disease, individuals exhibit no symptoms of this type of the disorder
(asymptomatic) or may develop a non-cancerous (benign) growth in the lymph
tissue; most frequently in the chest. Symptoms with this type are usually
secondary to the size and location of the growth.
The hyaline vascular sub-type accounts for 90% of cases of
Castleman disease and patients are generally asymptomatic (58% to 97% of
patients are asymptomatic). Most patients come to clinical attention when a
solitary middle or posterior mediastinal mass (adenopathy) is detected
incidentally on chest radiographs. In order of frequency, the intrathoracic
sites involved include the anterior mediastinum, particularly right
paratracheal, hilar nodes, and the posterior mediastinum. About 70% of affected
patients are less than 30 years old, and males are affected more than females. Symptomatic
patients may complain of dry cough, dyspnea, or recurrent infection due to
airway compression. The treatment is surgical with a low recurrence rate if the
resection is subtotal. Rare cases have been complicated by the development of
vascular neoplasms that resemble Kaposi's sarcoma, or Hodgkin's lymphoma .
The exact cause of Castleman Disease is not known. Some
researchers speculate that increased production of interleukin-6 (IL-6) may be
involved in the development of Castleman Disease. IL-6 is a substance produced
by structures within the lymph nodes.
The treatment of Castleman Disease is directed toward the
specific symptoms that are apparent in each individual. Specific therapies for
the treatment of this disorder are symptomatic and supportive. Surgical removal
(excision) of the growth is the preferred treatment in most cases of localized
Castleman Disease. In some cases, ionizing radiation (radiotherapy) has proven
effective."
.jpg)
.jpg)
.jpg)