CASTLEMAN'S
DISEASE
POEMS
SYNDROME
Edema and Castleman's Disease
Related
Terms and Synonymns: Angiofollicular hyperplasia, Giant lymph
node hyperplasia, Angiofollicular Lymph Node Hyperplasia,
Angiomatous
Lymphoid, Giant Benign Lymphoma, Hamartoma of the Lymphatics,
Poems
Syndrome
II am including
a page on Castleman's disease for
two main reasons. First, it does involve the lymph system and
as an
information portal, I want to cover as many lymphatic
conditions as I can. Secondly, there are a number of our
members who have
both lymphedema
and Castleman's, so they need information on both conditions.
The condition was first described by Dr. Benjamin Castleman in 1956.
Pat
Classification: Also known as giant lymph node hyperplasia and angiofollicular lymph node hyperplasia,
Lymphoproliferative
Disorder, Castleman disease is classified as a lymphoproliferative disorder.
Basic Definition:
A pathological
condition
characterized by large, benign,
hyperplastic
lymph nodes containing concentric
perivascular
aggregates of lymphocytes.
(3)
Discussion:
This condition
was first described by Dr. Benjamin
Castleman in 1956. His study involved 13 patients presenting
with
localized non-malignant
mediastinal lymphadenopathy.
The
non-cancerous growths involved in Castleman's generally
occur in the chest, stomach, and/or neck with the localized
form. Other
sites may include the armpit, pelvis and pancreas.
National Organization for Rare Disorders:
Castleman's
Disease is a rare disorder characterized by
non-cancerous (benign) growths (tumors)
that may develop in the
lymph node
tissue
throughout the body (i.e., systemic disease [plasma
cell type]). Most
often, they occur in the chest, stomach, and/or neck (i.e., localized
disease
[hyaline-vascular type]). Less common sites include the armpit
(axilla), pelvis,
and pancreas. Usually the growths represent abnormal enlargement of the
lymph
nodes normally found in these areas (lymphoid hamartoma).
There
are two
main types of Castleman's Disease:
hyaline-vascular type and plasma
cell type.
The
hyaline vascular
type accounts for approximately 90 percent of
the cases. Most
individuals exhibit no symptoms of this form of the disorder
(asymptomatic) or
they may develop non-cancerous growths in the lymph nodes.
About
70% of patients are under 30 years old
and males affected more than females. (3)
The
plasma cell type
of Castleman's Disease may be associated with fever, weight
loss,
skin rash,
early destruction of red blood cells, leading to unusually low levels
of
circulating
red blood cells (hemolytic anemia), and/or abnormally
increased
amounts of certain immune factors in the blood (hypergammaglobulinemia).
A
third type of Castleman's Disease has
been reported in the medical literature. This type may affect more than
one area
of the body (multicentric or generalized Castleman's Disease).
Many
individuals with Multicentric
Castleman's Disease
may exhibit an
abnormally large
liver and
spleen
(hepatosplenomegaly).
Researchers' opinions in
the medical literature differ as to whether Multicentric Castleman's
Disease is
a distinct entity or a multicentric form of the plasma cell type of
Castleman's
Disease.
A
third type of Castleman's disease
has been reported in the medical literature. This type may affect more
than one
area of the body (multicentric or generalized Castleman's disease).
Many
individuals with Multicentric Castleman's disease may exhibit an
abnormally
large liver and spleen (multicentric or generalized Castleman's
disease).). Researchers' opinions in the
medical literature differ as to whether Multicentric Castleman's
disease is a
distinct entity or a multicentric form of the plasma cell type of
Castleman's
disease.
Etiology:
Exact cause is
unknown, however there is speculation that
increased production of interleukin-6 (IL-6) may be involved in the
development
of Castleman's Disease. IL-6 is a substance produced by structures
within the
lymph nodes.
Symptoms: (2)
An enlarged
lymph node, usually inside the chest or abdomen, is the only
symptom for most people with the localized form of Castleman's disease.
People with
multicentric Castleman's disease have more than one enlarged lymph
node. The involved nodes may be in the chest or abdomen. More often,
lymph nodes
near the surface of the body are involved. For example, multicentric
Castleman's
disease often affects lymph nodes in the groin, the underarm area, and
on the
sides of the neck. Multicentric Castleman's disease can also affect
lymphoid
tissue of internal organs, causing the liver, spleen, or other organs
to
enlarge.
In addition,
people with either type of Castleman's disease may have other
symptoms. The most common include:
- Fever
- Weakness
- Night
sweats
- Weight
loss
- Loss
of appetite
- Nausea
and vomiting
- Nerve
damage that leads to numbness and weakness
These symptoms
occur about twice as often in multicentric than in localized
Castleman disease.(1)
Diagnosis:
Nodal
biopsies are one method of
diagnosis. The biopsies may be either
small needle or
excisional.
In
addition to the nodal biopsies radiology
tests which can include
CT
scans, MRI,
Chest
x-ray and/or gallium scan may be indicated.
The condition
is often difficult to diagnose because most
patients present
asymptomatic
(58%-97%). Generally follow up testing is done
when a large mass is noted in a x-ray..
Complications:
Pericardial and
pleural effusions, axillary,
inguinal and abdominal lymphadenopathy,
and
splenomegaly.
Patient may experience infections as
well.
Unicentric Castleman disease
People
with unicentric Castleman disease usually do well once the affected
lymph node is removed, although having Castleman disease may increase
your risk of lymphoma.
Mulricentric Castleman disease
On
the other hand, multicentric Castleman disease is much more sserious
and often life-threatening. Death from multicentric disease usually
occurs due to a serious infection, failure of multiple organs, cancer,
such as lymphoma or Kaposi's sarcoma. The presence of HIV/AIDS
tends to worsen the outcome. (CNN International Sept. 3, 2011)
Treatment:
Treatment programs will ne based on whether the patient has unicentric Castlemans or multicentric Castlemans.
For the
hyaline vascular sub-type,
surgical excision is the usual treatment with a low recurrence rate.
Treatment
for the plasma cell variant may include
radiation therapy
and
chemotherapy.
Drugs used in
the
chemotherapy treatment involve doxorubicin,
vincristine, cyclophosphamide, melphalan and chlorambucil. They may be
used
invidiually or in combination (CHOP).
Other treatments may include corticosteroids, antiviral drugs and immune modulators.
Prognosis:
For localized
Castleman's, the prognosis is
actually quite good. However, there may be a recurrence of
the condition.
For
multicentric Castleman's, the prognosis is not so
good. The morbidity rate for multicentric Castleman's was 50%
in two and a
half years. Other factors that dim the prognosis is if the
condition
accompanies AIDS. Also, approximately 20% of people with
Castleman's will
develop lymphoma.
------------------------------------------
Abstracts and
Studies:
------------------------------------------
Surgical treatment of unicentric plasma cell histological type Castleman's disease].
Sept 2011
[Article in Serbian]
Source
Vojnomedicinska akademija, Klinika za grudnu hirurgiju, Beograd, Srbija.
Abstract
INTRODUCTION:
Castleman's disease or angiofollicular lymph hyperplasia is a rare disease with two identified clinical forms. Unicentric or localized form is characterized by isolated growth of lymph nodes, most often in mediastinum, and multicentric form is expressed as systemic disease with spread lymphadenopathy, organomegaly and presence of general symptoms of the disease. Histological types are hyalovascular, plasma-cell and transitive (mixed) cell.
CASE REPORT:
This case report shows a woman, 59 years old, with unicentric form of plasma-cell type of Castleman'sdisease.
Unicentric form is usually shown as hyalovascular histological type,
extremely rare as plasma-cell type, and transitive (mixed) cell type
was never described in literature as localized clinical form. The disease was
manifested with chest pain, loss of body weight, exhaustion and
weakness of legs. Further diagnostic procedures found the presence of
enlarged lymph nodes paratracheally right, in a close contact with vena cava superior. The disease was confirmed by histopathological analysis of bioptated mediastinal lymph node after mediastinoscopy. Surgical treatment included extirpation of enlarged lymph nodes. After the regular postoperative condition, a full therapy effect was confirmed.
CONCLUSION:
Unicentric form of Castleman's disease is expressed with enlarged lymph nodes on predilected places, usually in mediastinum. Surgical treatment is best method for the management of the disease and brings a full recovery of patient.
http://www.ncbi.nlm.nih.gov/pubmed/22046887
---------------------------------------------
Multicentric plasma cell variant Castleman's disease presenting
with cutaneous vasculitis and pulmonary parenchymal involvement in a
patient with ankylosing spondylitis: case report and review of the
literature.
Jul-Aug 2011
Source
Department
of Immunology, Allergology and Rheumatology, Antwerp University
Hospital, Faculty of Medicine, University of Antwerp, Edegem, Belgium.
michael.wolf@uza.be
Abstract
We present a case of the multicentric plasma cell variant of Castleman's disease (CD)
with two rare manifestations. The patient consulted us because of
cutaneous vasculitis of the lower limbs, while constitutional symptoms
were nearly absent. Imaging studies also revealed pulmonary parenchymal
involvement. Furthermore, our patient is the first case in whom
association of ankylosing spondylitis with CD is reported. In addition,
we present a review of the literature with emphasis on the clinical
presentation of CD and its difficult discrimination from autoimmune and
infectious disorders. An overview of the therapeutic options is also
provided.
http://www.ncbi.nlm.nih.gov/pubmed/21938988
---------------------------------------------
PUBMED List of Castleman Abstracts and Studies
http://www.ncbi.nlm.nih.gov/pubmed?term=castlemans%20disease---------------------------------------------
Successful treatment with bortezomib and thalidomide for POEMS syndrome associated with multicentric mixed-type Castleman's disease.
Oct. 2011
http://jjco.oxfordjournals.org/content/41/10/1221.long
---------------------------------------------
Castleman's disease of the pleura.
Aug 2011
http://www.ncbi.nlm.nih.gov/pubmed/21850586
---------------------------------------------
A case of Castleman disease with status epileptics originating from focal cortical dysplasia].
Jul 2011
http://www.ncbi.nlm.nih.gov/pubmed/21823508
---------------------------------------------
An unusual case of cutaneous hyaline-vascular Castleman's disease with multicentric involvement and systemic symptoms.
Aug 2011
http://onlinelibrary.wiley.com/doi/10.1111/j.1365-4632.2009.04442.x/abstract
---------------------------------------------
Comparison of plasma cell type of Castleman's disease and IgG4-related sclerosing disease: a histopathological and immunohistochemical study.
2011
Key Words: Castleman’s disease; IgG4-related sclerosing disease; Plasma cell; Immunohistochemistry
http://content.karger.com/produktedb/produkte.asp?DOI=000327357&typ=pdf
---------------------------------------------
Improved outcome with rituximab in patients with HIV-associated multicentric Castleman disease.
Sept. 2011
http://bloodjournal.hematologylibrary.org/content/118/13/3499.long
---------------------------------------------
Meningeal
Castleman's disease with
multifocal involvement: a case report and review of literature.
J
Neurooncol.
2008 May
SpringerLink
---------------------------------------------
Fluorodeoxyglucose-positron
emission tomography/computed tomography in the staging and evaluation
of
treatment response in a patient with Castleman's disease: a case report
J
Med Case Reports. 2008 Apr
PubMed
Central
---------------------------------------------
Multicentric
Castleman's Disease in HIV
Infection: a Systematic Review of the Literature.
AIDS
Rev.
2008 Jan-Mar
Permanyer
Publication
---------------------------------------------
Local
Castlemanos Disease: A Report of 17
Cases witn Literature Review
Ai
Zheng. 2008 Mar
Chinese
Journal of Cancer
---------------------------------------------
Acute
renal failure due to
thrombotic microangiopathy associated with Castleman's disease
An
Med Interna. 2007 Dec
PubMed
---------------------------------------------
Castleman's
disease and related disorders.
Frizzera G.
Department of Laboratory Medicine and Pathology, University of
MinnesotaMedical
School, Minneapolis 55455.
Three disorders that bear the eponym of Castleman's disease (CD) are
discussed.
The localized CD of hyaline-vascular (HV) type features an
architecturally
abnormal,
hypervascular lymphoid tissue with burned-out germinal
centers, and
presents as an asymptomatic, slowly growing mass. It may represent a
lymphoid
hyperplasia associated with excessive angiogenesis.
The localized CD of plasma
cell (PC) type, instead, features an architecturally recognizable lymph
node
with solid sheets of PCs and presents with systemic manifestations of
inflammation and
B cell
hyperreactivity. It appears as a localized chronic
reaction to unknown antigens. "Multicentric" CD indicates a
clinicopathologic entity characterized by the histology of CD of
"mixed" type, a predominantly lymphadenopathic presentation
consistently involving peripheral nodes, manifestations of multisystem
involvement, and an idiopathic nature. It is best considered as a
systemic B
cell lymphoproliferation, which probably arises in a setting of
immunoregulatory
deficit, and may result in the outgrowth of clonal B cell populations.
An
attempt is presented to place the latter two forms of CD in the larger
perspective of idiopathic PC disorders, by taking into account both the
nature
of the diseases (hyperplastic, dysplastic, neoplastic)
and the lymphocyte
traffic system that is involved (bone marrow-bound, mucosa-associated
or
peripheral-node-bound). In such a scheme, localized CD, PC type, and
multicentric CD appear as a hyperplastic and a dysplastic disorder,
respectively, of peripheral-node-bound B cells, related to, and often
associated
with, primary nodal plasmacytoma and osteosclerotic myeloma (so-called
POEMS,
Takatsuki's or Crow-Fukase's syndrome).
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=2464187
---------------------------------------------
Angiofollicular
lymph node hyperplasia (Castleman's
disease).
McCarty MJ, Vukelja SJ, Banks PM, Weiss RB.
Department of Medicine, Brooke Army Medical Center, Fort Sam Houston,
TX 78234,
USA.
This review provides a comprehensive assessment of angiofollicular
lymph node
hyperplasia (ALNH) or Castleman's disease including pathogenesis,
clinical
presentation, histomorphologic and immunophenotypic findings,
laboratory
results, treatment, and prognosis. A division of ALNH into clinically
relevant
subtypes provides a framework for the consideration of the disorder. A
comprehensive search of the medical literature involving ALNH using
Medline was
performed. Reports judged to be significant for the understanding of
the
disorder were analyzed and their findings incorporated into this
review. ALNH is
divided into localized/unicentric ALNH and generalized/multicentric
ALNH due to
the profound clinical differences seen between these variants.
Localized/unicentric
ALNH is separated by clinical and histomorphologic criteria into
hyaline-vascular (HV) and plasma-cell (PC) subtypes.
Generalized/multicentric
ALNH may be divided by clinical criteria into generalized/multicentric
ALNH
without
neuropathy (non-neuropathic) and generalized/multicentric
ALNH with
neuropathy (POEMS-associated or neuropathic). The dichotomy between
these two
subtypes is not absolute, with considerable clinical overlap occurring
among
patients presenting with generalized disease. Immunophenotypic and
molecular
probe studies demonstrate clonal
B-cell
lymphocyte populations in some cases,
particularly those with generalized/multicentric ALNH. However, the
finding of
clonal populations is of no value in predicting malignant clinical
progression.
We conclude that using this division of ALNH, patients presenting with
symptoms
and histomorphology consistent with ALNH can be subdivided into the
appropriate
category of ALNH. Localized or unicentric disease, either HV or PC
subtype, has
an excellent prognosis with surgery being curative in the majority of
cases.
Generalized or multicentric disease indicates a poor prognosis with
short
survival, with the neuropathic variant possessing resistance to
steroids and
chemotherapy and a corresponding worse prognosis.
Pub Med
National
Library of Medicine
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=7585657&itool=iconabstr/
---------------------------------------------
Castleman's
disease
Larroche C, Cacoub P, Godeau P.
Service de medecine interne, hopital de la Pitie-Salpetriere, Paris,
France.
Castleman's disease, also called angiofollicular lymph node hyperplasia
was
first described as a distinct entity by Castleman et al in 1956. Two
forms are
now described a localized and a multicentric. The clinical and
biological signs
are varied and heterogeneous, and the diagnostic is made on the
histologic
examination. This atypical lymphoproliferative disorder is of unknown
origin,
but interleukin 6 play a central part in this disease. Despite the
benignity of
this "prelymphoma state", an aggressive course with poor prognosis
occur usually in the multicentric form. Malignant lymphomas and
Kaposi's sarcoma
have been associated with Castleman's disease.
==================================================
Poems Syndrome - Related medical Condtions
Synonyms of POEMS Syndrome
Disorder Subdivisions
General Discussion
POEMS syndrome is an extremely rare multisystem disorder. POEMS is an
acronym that stands for (P)olyneuropathy, disease affecting many
nerves; (O)rganomegaly, abnormal enlargement of an organ;
(E)ndocrinoapthy, disease affecting certain hormone-producing glands
that help to regulate the rate of growth, sexual development, and
certain metabolic functions (endocrine system); (M)onoclonal gammopathy
or M proteins; and (S)kin defects. Common symptoms include progressive
weakness of the nerves in the arms and legs, an abnormally enlarged
liver and/or spleen (hepatosplenomegaly), abnormally darkening of the
skin (hyperpigmentation) and excessive hair growth (hypertrichosis).
Endocrine abnormalities such as failure of the ovaries and testes
(gonads) to function properly (primary gonadal failure) and diabetes
mellitus type I may be present. Specific endocrine abnormalities
associated with POEMS syndrome vary from case to case.
POEMS syndrome is associated with a group of disorders known as
monoclonal gammopathies or plasma cell dyscrasias. These disorders are
characterized the uncontrolled growth of a single clone (monoclonal) of
plasma cells, which results in the abnormal accumulation of M-proteins
(also known as immunoglobulin M or IgM) in the blood. M-proteins are
supposed to fight foreign substances in the body such as viruses and
bacteria, but researchers suspect that they play a role in the
development of POEMS syndrome. However, the specific role M-proteins
play and the exact cause of POEMS syndrome is unknown.
.
Organizations related to
POEMS Syndrome
------------------------------------------------------------------------
|
Poems syndrome
|
|
|
 |
A
combination of plasma
cell dyscrasia, chronic progressive polyneuropathy, and
endocrine abnormalities such as diabetes
mellitus. This syndrome is of unknown cause and takes its
name from the typical features: polyneuropathy, organomegaly,
endocrinopathy, M proteins and skin changes. Numerous other
characteristics can be noted. However, patients do not have an
increased prevalence of amyloidosis and rarely reveal Bence Jones
proteinuria. Bone proliferation occurs at entheses (sites of tendon and
ligament attachment to bone). Particularly characteristic are irregular
bony excrescences on the posterior elements of the spine and about the
sacroiliac and costovertebral joints (Fig.1).
http://www.amershamhealth.com/medcyclopaedia/medical/Volume%20III%201/POEMS%20syndrome.asp
|
---------------------------------------------------------------------------------------
POEMS
syndrome: definitions and long-term outcome.
Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM,
Larson DR, Greipp PR, Witzig TE, Basu R, Suarez GA, Fonseca R, Lust JA,
Gertz MA.
Division of Hematology and Internal Medicine, Mayo Clinic, Rochester,
MN 55905, USA. dispenzieri.angela@mayo.edu
The POEMS syndrome (coined to refer to polyneuropathy,
organomegaly, endocrinopathy,
M protein, and skin changes) remains poorly understood. Ambiguity
exists over the features necessary to establish the diagnosis,
treatment efficacy, and prognosis. We identified 99 patients with POEMS
syndrome. Minimal criteria were a sensorimotor
peripheral neuropathy and evidence of a monoclonal
plasmaproliferative disorder. To distinguish POEMS from neuropathy
associated with monoclonal gammopathy of undetermined significance,
additional criteria were included: a bone lesion, Castleman disease,
organomegaly (or lymphadenopathy),
endocrinopathy,
edema (peripheral edema, ascites,
or effusions), and skin changes. The median age at presentation was 51
years; 63% were men. Median survival was 165 months. With the exception
of fingernail clubbing (P =.03) and extravascular volume overload (P
=.04), no presenting feature, including the number of presenting
features, was predictive of survival. Response to therapy (P
<.001) was predictive of survival.
Pulmonary hypertension, renal failure, thrombotic events, and
congestive heart failure were observed and appear to be part
of the syndrome. In 18 patients (18%), new disease manifestations
developed over time. More than 50% of patients had a response to
radiation, and 22% to 50% had responses to prednisone and a combination
of melphalan and prednisone, respectively. We conclude that the median
survival of patients with POEMS syndrome is 165 months, independent of
the number of syndrome features, bone lesions, or plasma cells at
diagnosis. Additional features of the syndrome often develop, but the
complications of classic multiple myeloma rarely develop.
PMID: 12456500 [PubMed - indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12456500&dopt=Abstract
------------------------------------------------------------
Treatment
of POEMS syndrome.
Dispenzieri A, Gertz MA.
Division of Hematology and Internal Medicine, Mayo Clinic, 200 First
Street SW, Rochester, MN 55905, USA. dispenzieri.angela@mayo.edu
POEMS (polyneuropathy, organomegaly,
endocrinopathy, M protein, and skin changes) syndrome is a rare
multisystemic paraneoplastic syndrome driven by an underlying plasma
cell dyscrasia. More than 95% of patients will have monoclonal lambda
sclerotic plasmacytoma(s) or bone marrow infiltration. Recognition of
the complex of a combination of peripheral neuropathy, organomegaly,
endocrinopathy, monoclonal plasmaproliferative disorder, skin changes,
sclerotic bone lesions, Castleman disease, thrombocytosis,
papilledema, peripheral edema,
pleural effusions, ascites, fingernail clubbing, and white
nails is the first step in effectively managing the disease. Once a
patient has been completely evaluated, each component of the disease
should be addressed, while finalizing a treatment plan for the
underlying plasma cell proliferative disorder. In patients with a
dominant sclerotic plasmacytoma, first-line therapy should include
radiation to the lesion. Retrospective analysis and personal experience
would dictate that systemic therapy be considered for patients with
diffuse sclerotic lesions or absence of any bone lesion and for
patients who have not demonstrated stabilization of their disease 3 to
6 months after completing radiation therapy. Treatments with
demonstrated benefit include corticosteroids, low-dose alkylator
therapy, and high-dose chemotherapy with peripheral blood stem cell
transplant. Until the pathogenesis is fully understood, these are the
mainstays of treatment for patients with POEMS syndrome.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=15115653&itool=iconabstr
---------------------------------------------------------------------------------------
Treatment of POEMS Syndrome
POEMS
syndrome is a very rare type of bone marrow disorder whose cause is
unknown. It involves an overgrowth of bone marrow cells called plasma
cells, which produce chemicals that damage other parts of the body. It
is not inherited, nor is it contagious.
Patients
sometimes confuse POEMS syndrome with multiple myeloma. The symptoms
and potential complications of these diseases are very different. The
average expected life span is better in patients with POEMS (who may
have osteosclerotic myeloma) than it is in patients
with
multiple myeloma.
The
diagnosis of POEMS syndrome is made by combining the information from a
thorough history, physical examination, and blood, urine, X-ray and
bone marrow tests. The overgrowth of
plasma cells can often be detected by finding an abnormal
protein in the blood or urine (a monoclonal protein), by an abnormality
on a bone X-ray and/or a bone marrow biopsy. POEMS is a syndrome, which
means that several features must be present to make the diagnosis.
Because it is so rare, the diagnosis is often missed. Initially, people
may have been told that they have a nerve problem without any known
cause. Or they may have been told that they have multiple myeloma, a
type of bone marrow cancer.
Mayo
Clinic approach
Mayo
Clinic uses an integrated approach involving a multispecialty team of
physicians in caring for patients with POEMS syndrome. Working
together, Mayo clinicians develop a treatment plan based on the
patient's individual needs and health issues.
Treatment
Options
People
with POEMS suffer many symptoms;
most commonly, the worst is caused by nerve damage. Patients can
develop extreme, progressive weakness (neuropathy) of their extremities
(feet, legs, hands and arms). The most effective method of stopping the
progression or advancement of this weakness is to kill the plasma cell
clone (the osteosclerotic myeloma). The doctor may recommend radiation
therapy, corticosteroids, chemotherapy or even a bone
marrow transplant. If there are only one or two spots of
osteosclerotic myeloma, the doctor may recommend radiation therapy
alone. The choice of treatment must be made with care, balancing the
risks and benefits of treatment.
If
there are hormonal abnormalities, hormone replacement may be needed.
Physical therapy is highly recommended if there has been significant
disability caused by the neuropathy. As the osteosclerotic myeloma is
treated, many of the other symptoms will improve, including the skin
changes, the swelling of the extremities and the enlarged organs. The
hormonal abnormalities may or may not improve. The nerve problem may or
may not improve, depending on how advanced the disease is by the time
treatment begins.
All
patients need lifelong follow-up for this condition because relapses
are possible
http://www.mayoclinic.org/poems-rst/
------------------------------------------
External Links:
------------------------------------------
National Organizations and Web
Sites
National
Organization for Rare Disorders
(NORD)
55 Kenosia Avenue
PO Box 1968
Danbury, CT 06813
Toll-free:
(800) 999-6673 (voicemail only)
Telephone: (203) 744-0100
Internet
address:http://www.rarediseases.org/
-------------------------------------------
Castleman's
Disease Homepage - International Castleman's Disease Organizatino (3)
http://www.castlemans.org/
Patient Resources:
http://www.castlemans.org/patient-resources
-------------------------------------------
Castleman's
Disease (2)
American
Cancer Society
http://www.cancer.org/docroot/CRI/CRI_2_3x.asp?dt=70
http://www.cancer.org/acs/groups/cid/documents/webcontent/003093-pdf.pdf
--------------------------------------------
Castleman's
disease Information
http://www.diseasesdatabase.com/ddb2165.htm
---------------------------------------------
Castleman's Disease - Signs and Symptoms (1)
http://www.cancer.org/acs/groups/cid/documents/webcontent/003093-pdf.pdf
----------------------------------------------
Alleviation of Systemic Manifestations of
Castleman's Disease by Monoclonal Anti-Interleukin-6 Antibody
New
England Journal of Medicine
-----------------------------------------------
----------------------------------------------
-----------------------------------------------
An unusual presentation of
Castleman's Disease:a case report
-----------------------------------------------
-----------------------------------------------
NIH/National
Heart, Lung and Blood Institute Information Center
P.O. Box 30105
Bethesda, MD 20824-0105
Tel: (301)592-8573
Fax: (301)251-1223
Email: nhlbiinfo@rover.nhlbi.nih.gov
-----------------------------------------------
-----------------------------------------------
International Castleman's Disease Organization
4 Brazos Court
Santa Fe, NM 87508
Tel: (505)424-7947
Fax: (505)424-7948
Email: HMConsult@msn.com
Internet: http://www.castlemans.org
===========================
ICD-10 and ICD-9
ICD-10
Poems Syndrome -
Orpha
number: Poems Syndrome ORPHA2905
ICD-10
Castleman's
Disease
Hyaline
vascular types D21.9; Connective and other soft tissue unspecified
M8000/0; Plams cell type where the histology is difficult to prefidct
D47.9; Neoplasm of uncertain or unknown behaviour of lymphoid
haematoporietic and related tissue, unspecified M8000/1
ICD-9
Castleman's
(mediastinal lymph node hyperplasia) 785.6
===========================
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http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema\
For Information about
Lymphedema Complications
http://www.lymphedemapeople.com/wiki/doku.php?id=complications_of_lymphedema
For Lymphedema Personal Stories
http://www.lymphedemapeople.com/phpBB2/viewforum.php?f=3
For information about How to Treat a
Lymphedema Wound
http://www.lymphedemapeople.com/wiki/doku.php?id=how_to_treat_a_lymphedema_wound
For information about
Lymphedema Treatment
http://www.lymphedemapeople.com/wiki/doku.php?id=treatment
For information about Exercises
for Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=exercises_for_lymphedema
For information on Infections
Associated with Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=infections_associated_with_lymphedema
For information on Lymphedema
in Children
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_in_children
Lymphedema Glossary
http://www.lymphedemapeople.com/wiki/doku.php?id=glossary:listing
===========================
Lymphedema
People - Support
Groups
-----------------------------------------------
Children with Lymphedema
The time has come
for families, parents, caregivers to have a support group of their own.
Support group for parents, families and caregivers of chilren with
lymphedema. Sharing information on coping, diagnosis, treatment and
prognosis. Sponsored by Lymphedema People.
http://health.groups.yahoo.com/group/childrenwithlymphedema/
Subscribe: childrenwithlymphedema-subscribe@yahoogroups.com
......................
Lipedema Lipodema Lipoedema
No matter how you spell it, this is another very little understood and
totally frustrating conditions out there. This will be a support group
for those suffering with lipedema/lipodema. A place for information,
sharing experiences, exploring treatment options and coping.
Come join, be a part of the family!
http://health.groups.yahoo.com/group/lipedema_lipodema_lipoedema/?yguid=209645515
Subscribe: lipedema_lipodema_lipoedema-subscribe@yahoogroups.com
......................
MEN WITH LYMPHEDEMA
If you are a man with lymphedema; a man with
a loved one with lymphedema who you are trying to help and understand
come join us and discover what it is to be the master instead of the
sufferer of lymphedema.
http://health.groups.yahoo.com/group/menwithlymphedema/
Subscribe: menwithlymphedema-subscribe@yahoogroups.com
......................
All About Lymphangiectasia
Support group for parents, patients, children who suffer from all forms
of lymphangiectasia. This condition is caused by dilation of the
lymphatics. It can affect the intestinal tract, lungs and other
critical body areas.
http://health.groups.yahoo.com/group/allaboutlymphangiectasia/
Subscribe: allaboutlymphangiectasia-subscribe@yahoogroups.com
......................
Lymphatic Disorders Support Group @ Yahoo
Groups
While we have a number of support groups for lymphedema... there is
nothing out there for other lymphatic disorders. Because we have one of
the most comprehensive information sites on all lymphatic disorders, I
thought perhaps, it is time that one be offered.
DISCRIPTION
Information and support for rare and unusual disorders affecting the
lymph system. Includes lymphangiomas, lymphatic malformations,
telangiectasia, hennekam's syndrome, distichiasis, Figueroa syndrome,
ptosis syndrome, plus many more. Extensive database of information
available through sister site Lymphedema People.
http://health.groups.yahoo.com/group/lymphaticdisorders/
Subscribe: lymphaticdisorders-subscribe@yahoogroups.com
===========================
Lymphedema People New Wiki Pages
Have you seen our new
“Wiki” pages yet? Listed
below are just a sample of the more than 140 pages now listed in our
Wiki section. We are also working on hundred more.
Come and take a stroll!
Lymphedema Glossary
http://www.lymphedemapeople.com/wiki/doku.php?id=glossary:listing
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema
Arm Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=arm_lymphedema
Leg
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=leg_lymphedema
Acute
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=acute_lymphedema
The Lymphedema
Diet
http://www.lymphedemapeople.com/wiki/doku.php?id=the_lymphedema_diet
Exercises for
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=exercises_for_lymphedema
Diuretics are not for
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=diuretics_are_not_for_lymphedema
Lymphedema People
Online Support Groups
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_people_online_support_groups
Lipedema
http://www.lymphedemapeople.com/wiki/doku.php?id=lipedema
Treatment
http://www.lymphedemapeople.com/wiki/doku.php?id=treatment
Lymphedema and Pain
Management
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_and_pain_management
Manual
Lymphatic Drainage (MLD) and Complex Decongestive Therapy (CDT)
http://www.lymphedemapeople.com/wiki/doku.php?id=manual_lymphatic_drainage_mld_complex_decongestive_therapy_cdt
Infections Associated
with Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=infections_associated_with_lymphedema
How to Treat a
Lymphedema Wound
http://www.lymphedemapeople.com/wiki/doku.php?id=how_to_treat_a_lymphedema_wound
Fungal Infections
Associated with Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=fungal_infections_associated_with_lymphedema
Lymphedema in
Children
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_in_children
Lymphoscintigraphy
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphoscintigraphy
Magnetic Resonance
Imaging
http://www.lymphedemapeople.com/wiki/doku.php?id=magnetic_resonance_imaging
Extraperitoneal
para-aortic lymph node dissection (EPLND)
http://www.lymphedemapeople.com/wiki/doku.php?id=extraperitoneal_para-aortic_lymph_node_dissection_eplnd
Axillary
node biopsy
http://www.lymphedemapeople.com/wiki/doku.php?id=axillary_node_biopsy
Sentinel Node
Biopsy
http://www.lymphedemapeople.com/wiki/doku.php?id=sentinel_node_biopsy
Small Needle Biopsy -
Fine Needle Aspiration
http://www.lymphedemapeople.com/wiki/doku.php?id=small_needle_biopsy
Magnetic
Resonance Imaging
http://www.lymphedemapeople.com/wiki/doku.php?id=magnetic_resonance_imaging
Lymphedema
Gene FOXC2
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_gene_foxc2
Lymphedema Gene VEGFC
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_gene_vegfc
Lymphedema Gene SOX18
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_gene_sox18
Lymphedema
and Pregnancy
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_and_pregnancy
Home page: Lymphedema People
http://www.lymphedemapeople.com
Page Updated: Jan. 8, 2012
