$Unique_ID{BRK00891}
$Pretitle{Ill Defined Symptoms and Rare Diseases, Blood and Blood Forming
Organs}
$Title{Family Anemia Condition and Spleen Removal}
$Subject{anemia red cells childhood Minkowski-Chauffard}
$Volume{N-7, D-7}
$Log{}

Copyright (c) 1991   Tribune Media Services, Inc.


Family Anemia Condition and Spleen Removal


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QUESTION:  You recently wrote that spleens are rarely removed these days, but
in our family, we have an anemia condition which appears in childhood, and for
which doctors say there is no other treatment.  Does your recent column change
that?  What can you tell me about our problem?

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ANSWER:  I have had to omit much of the information you included in your long
letter, but it is clear that my remarks about removal of the spleen do not
apply to you and your family.  The condition of hereditary spherocytosis (also
known as Minkowski-Chauffard Disease) is one in which there is an increased
destruction of the red cells causing anemia, and associated with jaundice and
an enlarged spleen.  The diagnosis is usually made in childhood, where, in
some cases, the only sign present is an enlarged spleen found during a routine
examination.  The condition is present in people of all races, but is the most
common inherited anemia found in persons of Northern European descent.  It
occurs in about 220 people per million population in the United States.
Usually, as in your case, the family is aware of the inherited characteristics
of the disease, but a good family history can often be the first clue to the
diagnosis.  There is also a tendency for individuals with this disorder to
develop characteristic pigmented gallstones, even in children.  There are
several types of this disease, and two types of inherited patterns, so a
careful evaluation, with all the appropriate laboratory tests is a must.  If
there are no symptoms, such as jaundice or severe anemia, then treatment may
be unnecessary, but in cases where crises do occur, the condition may be
completely resolved by removing the enlarged spleen.  If you haven't done so
already, seek out a good genetic counselor, who along with your physician, can
determine the pattern of inheritance in your family, and help you to
understand more about the manner in which the disease is transmitted, and some
of the important clues that may alert you and your family members of the
presence of this disorder.
ÿ
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The material contained here is "FOR INFORMATION ONLY" and should not replace 
the counsel and advice of your personal physician.  Promptly consulting your 
doctor is the best path to a quick and successful resolution of any medical 
problem.
