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$Unique_ID{BRK03637}
$Pretitle{}
$Title{Cryoglobulinemia, Essential Mixed}
$Subject{Cryoglobulinemia Essential Mixed EMC Raynaud's Disease and
Phenomenon Purpura Vasculitis Wegener's Granulomatosis}
$Volume{}
$Log{}
Copyright (C) 1992 National Organization for Rare Disorders, Inc.
910:
Cryoglobulinemia, Essential Mixed
** IMPORTANT **
It is possible that the main title of the article (Essential Mixed
Cryoglobulinemia) is not the name you expected. Please check the SYNONYM
listing to find the alternate names and disorder subdivisions covered by this
article.
Synonyms
EMC
Information on the following diseases can be found in the Related
Disorders section of this report:
Raynaud's Disease and Phenomenon
Purpura
Vasculitis
Wegener's Granulomatosis
General Discussion
** REMINDER **
The Information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or treatment
purposes. If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.
Essential Mixed Cryoglobulinemia is a rare autoimmune disorder that
affects the blood and various other body systems. Major symptoms may include
unusual response to cold, skin abnormalities, weakness and blood problems.
There may also be joint pain, inflamed blood vessels and kidney problems.
Symptoms
Essential Mixed Cryoglobulinemia is characterized by extreme physical
reactions to cold such as loss of feeling in the hands or feet. In people
with this disorder, blood becomes thicker when they are is exposed to cold.
Brown spots may appear under the skin. The kidneys may become inflamed and
produce unusual levels of protein or even blood in the urine. There may also
be arthritis type joint pain, weakness and even central nervous system
problems. Gastrointestinal symptoms usually occur and the liver and spleen
may be affected. Problems in the intestines and colon may cause chronic
diarrhea.
Causes
The exact cause of Essential Mixed Cryoglobulinemia is not known. However,
it is considered to be an autoimmune disorder. (Autoimmune disorders are
caused when the body's natural defenses such as antibodies, lymphocytes,
etc.) against invading organisms mistakenly attack healthy tissue. In this
condition the immune system appears to be triggered by cold temperatures.
Cryoglobulins are proteins in the blood that become apparent when the blood
is cooled. These Cryoglobulins can affect many different bodily systems
causing dysfunction.
Affected Population
Essential Mixed Cryoglobulinemia is a rare autoimmune disorder that can
affect many body functions; however, it is usually not apparent until middle
age and it affects females more often than males.
Related Disorders
Symptoms of the following disorders can be similar to those of Essential
Mixed Cryoglobulinemia. Comparisons may be useful for a differential
diagnosis:
Raynaud's Disease and Phenomenon are vascular disorders. They are caused
by spasms of blood vessels when a person is exposed to either hot or cold
temperatures. Symptoms occur more often in cold temperatures than in hot
weather, and can also be triggered by emotional upset. Patients may
experience sensory changes such as aching pain, a tingling feeling or
throbbing in fingers or toes when exposed to extreme temperatures. There may
be the sensation of tightness or pins and needles. The feeling of coldness
or numbness and a loss of color or turning blue may also occur. (For more
information on this disorder, choose "Raynaud" as your search term in the
Rare Disease Database).
Purpura means a red or purple color of the skin. It results from the
fusion of blood and plasma into surfaces under the skin, mucous membranes and
serous membranes. Purpura is the most common symptom of a vascular bleeding
disorder which is characterized by increased bruising and fragile blood
vessels. It can be caused by inflammation of the blood vessels affecting the
skin, joints, gastrointestinal system and kidneys. (For more information on
this disorder, choose "Purpura" as your search term in the Rare Disease
Database).
Vasculitis is a common disorder characterized by an inflammation of the
blood vessel walls. Arteries and veins of all sizes and in all parts of the
body may be affected. Depending on the system involved there may be muscle
pain, joint pain, fever, weight loss, loss of appetite, headache, or
generalized weakness. The skin, eyes, stomach and kidneys may be affected.
(For more information on this disorder, choose "Vasculitis" as your search
term in the Rare Disease Database).
Wegener's Granulomatosis is a rare collagen vascular disorder that begins
as a localized inflammation of the mucous membranes in the upper and lower
respiratory tract and usually progresses into generalized inflammation of the
blood vessels and kidneys. (For more information on this disorder, choose
"Wegener" as your search term in the Rare Disease Database).
Therapies: Standard
Protecting the patient from cold temperatures is a necessary preventive
measure for people with Essential Mixed Cryoglobulinemia.
Treatment of Essential Mixed Cryoglobulinemia usually consists of
combinations of drugs including steroids and cyclophosphamide. These drugs
can alter the immune system and alleviate symptoms in many cases.
Plasmapheresis is a procedure used for removing unwanted substances such
as toxins and plasma parts from the blood. Blood is removed from the patient
and blood cells are separated from plasma. The patient's plasma is then
replaced with other human plasma and the blood is transfused back into the
patient. Cryoblobulins can be removed from the patient's blood by cooling
the blood serum during Plasmapheresis.
Cryofiltration is another form of treatment that can remove the
cryoblobulins from the blood without the need of plasma replacement.
Therapies: Investigational
This disease entry is based upon medical information available through May
1992. Since NORD's resources are limited, it is not possible to keep every
entry in the Rare Disease Database completely current and accurate. Please
check with the agencies listed in the Resources section for the most current
information about this disorder.
Resources
For more information on Essential Mixed Cryoglobulinemia, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
203 746-6518
NIH/National Heart, Lung and Blood Institute
9000 Rockville Pike
Bethesda, MD 20892
(301)496-4236
References
CECIL TEXTBOOK OF MEDICINE, 19th Ed.: James B. Wyngaarden, and Lloyd H.
Smith, Jr., Editors; W.B. Saunders Co., 1990. P. 601.
HEMATOLOGY, 4th Ed,: William J. Williams, et al,; Editors; McGraw-Hill,
Inc., 1990. P. 1437.
THE KIDNEY, 4th Ed.; Barry M. Brenner, M.D. and Floyd C. Rector, Jr.,
M.D., Editors; W.B. Saunders Company, 1991. Pp. 1311-1312.
CHRONIC DIARRHEA IN ESSENTIAL MIXED CRYOGLOBULINEMIA: A MANIFESTATION OF
VISCERAL VASCULITIS?, Jones, MP., et al.; Am J Gastroenterol, April, 1991,
(issue 86 (4)). Pp. 522-524.
RAPID IMPROVEMENT IN A PATIENT WITH LEUKOCYTOCLASTIC VASCULITIS WITH
SECONDARY MIXED CRYOGLOBULINEMIA TREATED WITH CRYOFILTRATION., Sawada, K., et
al.; J Rheumatol, January, 1991, (issue (1)). Pp. 91-94.