Hereditary ovalocytosis
Ovalocytosis - hereditary
Hereditary ovalocytosis is a rare condition passed down through families (inherited). The blood cells are oval-shaped instead of round. It is a form of hereditary elliptocytosis .
Hereditary elliptocytosis
Hereditary elliptocytosis is a disorder passed down through families in which the red blood cells are abnormally shaped. It is similar to other bloo...
Causes
Ovalocytosis is mainly found in Southeast Asian populations.
Symptoms
Newborn infants with ovalocytosis may have anemia and jaundice . Adults most often do not show symptoms.
Anemia
Anemia is a condition in which the body does not have enough healthy red blood cells. Red blood cells provide oxygen to body tissues. Different type...
Jaundice
Jaundice is a yellow color of the skin, mucus membranes, or eyes. The yellow coloring comes from bilirubin, a byproduct of old red blood cells. Jau...
Exams and Tests
An exam by your health care provider may show an enlarged spleen.
This condition is diagnosed by looking at the shape of blood cells under a microscope. The following tests may also be done:
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Complete blood count
(CBC) to check for anemia or red blood cell destruction
Complete blood count
A complete blood count (CBC) test measures the following:The number of red blood cells (RBC count)The number of white blood cells (WBC count)The tota...
- Blood smear to determine cell shape
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Bilirubin level
(may be high)
Bilirubin level
The bilirubin blood test measures the level of bilirubin in the blood. Bilirubin is a yellowish pigment found in bile, a fluid made by the liver. Bi...
- Lactate dehydrogenase level (may be high)
- Ultrasound of the abdomen (may show gallstones)
Treatment
In severe cases, the disease may be treated by removal of the spleen ( splenectomy ).
Splenectomy
Spleen removal is surgery to remove a diseased or damaged spleen. This surgery is called splenectomy. The spleen is in the upper part of the belly, ...
Possible Complications
The condition may be associated with gallstones or kidney problems.
Gallstones
Gallstones are hard deposits that form inside the gallbladder. Gallstones may be as small as a grain of sand or as large as a golf ball.
References
Gallagher PG. Hemolytic anemias. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine . 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 161.
Segel GB. Hereditary elliptocyctosis. In: Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics . 20th ed. Philadelphia, PA: Elsevier; 2016:chap 459.
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Ovalocytoses - illustration
Red blood cells (RBCs) are normally round. In ovalocytosis, the cells are oval. Other conditions that produce abnormally shaped RBCs include spherocytosis and eliptocytosis.
Ovalocytoses
illustration
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Blood cells - illustration
Blood is comprised of red blood cells, platelets, and various white blood cells.
Blood cells
illustration
-
Ovalocytoses - illustration
Red blood cells (RBCs) are normally round. In ovalocytosis, the cells are oval. Other conditions that produce abnormally shaped RBCs include spherocytosis and eliptocytosis.
Ovalocytoses
illustration
-
Blood cells - illustration
Blood is comprised of red blood cells, platelets, and various white blood cells.
Blood cells
illustration
Review Date: 2/1/2016
Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.