DEAR DR. GOTT: I’m a 70-year-old woman. My astute physician’s assistant noticed I had a low red-blood-cell count and referred me to a hematologist. I was subsequently diagnosed with hereditary stomatocytosis. Because there is no history of Mediterranean heritage, he enlisted the help of the renowned Stanford Medical Center, so I don’t doubt the diagnosis.
I am writing to you because I am a die-hard fan and trust your opinion. Could you please tell me whether or not I should undertake any dietary changes, since I’m told that this condition causes deposits of iron in the primary organs?
DEAR READER: Hereditary stomatocytosis is a disorder that affects the permeability of red blood cells. There are several varieties of this disorder, with the two most common being overhydrated hereditary stomatocytosis (OHS or hydrocytosis) and dehydrated hereditary stomatocytosis (DHS or xerocytosis).
Both OHS and DHS cause the red blood cells to absorb and excrete abnormal amounts of sodium and potassium. OHS causes the absorption of too much sodium, which leads the cell to excrete too much potassium. DHS is the opposite with too much potassium taken in and too much sodium removed.
These lead to swelling (OHS) or shrinking (DHS) of the red blood cells that then causes the membrane to disintegrate, releasing hemoglobin. This process is known as hemolytic anemia.
These syndromes are extremely rare and usually occur in Caucasians of European descent. Both disorders are genetic, meaning they are caused by a mutated gene and usually run in families; however, sporadic cases with no apparent genetic link have been reported.
I urge you to see a hematologist (blood-disorder specialist). He or she will be your best source for what to expect and possible treatment options.
To provide related information, I am sending you a copy of my Health Report “Blood – Donations and Disorders.” Other readers who would like a copy should send a self-addressed stamped No. 10 envelope and a check or money order for $2 to Newsletter, P.O. Box 167, Wickliffe, OH 44092. Be sure to mention the title.
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