Anemia Due to Hemolysis

Hemolytic anemias from changes in the RBC’s	Hereditary elliptocytosis and Hereditary spherocytosis
Hemolytic anemias from changes in the RBC’s	Erythropoietic protoporphyria
Metabolic RBC disorders	Glucose-6-Phosphate Dehydrogenase Deficiency(G6PD deficiency)
Hemoglobinopathies	Sickle cell anemia
Hemoglobinopathies	Thalassemia
Hemolytic anemias from changes outside of the RBC	Autoimmune hemolytic anemias
	Traumatic hemolytic anemia
	Splenomegaly and Hypersplenism

Introduction

Hemolysis is a process that dissolves red blood cells, so that they live shorter than the normal 120 days. This can occur because of changes in the red blood cells themselves or because of factors outside of the red blood cells. Damaged RBC’s are removed by phagocytic cells, called “reticulocytes”, in the liver, spleen and in the bone marrow. These reticulocytes are equipped to dismantle the RBC’s. However, this is done by reutilizing the iron of the hemoglobin molecule and rescuing the heme part as bilirubin. Normally there are a number of enzymes that take care of this automatically. The iron is transported to the bone marrow for reutilization of the iron so that new RBC’s can be made there. The hemoglobin is converted by the liver into bilirubin and bound to glucuronide. Bilirubin glucuronide is excreted by the liver into the bile ducts and ends up in the stool where further enzymatic reactions change it into stercobilin. This gives the stool its brown color. Each enzymatic step is known to be able to be interrupted by a congenital abnormality. Some of these abnormalities are of clinical significance, others are minor.

Symptoms

The symptoms are similar to other forms of anemia. However, hemolysis may present with more pronounced symptoms such as chills, an elevated body temperature, pain in the abdomen and in the back with general malaise. In severe cases of hemolysis the patient may have excruciating pain all over and even get into shock. Jaundice and splenomegaly (enlarged spleen) are often also present.

Diagnostic tests

When blood tests of a patient with these symptoms show anemia and reticulocytosis (meaning large numbers of reticulocytes in the blood), the physician would suspect hemolysis as a possible cause of the illness. A blood smear is obtained and the hematologist looks for possible causes of hemolysis. For instance with spherocytosis the pathologist would find spherically shaped red blood cells. An enlarged spleen is found and there is a family history of similar findings. Osmotic fragility tests show an increased fragility of the red blood cell membrane. The Coombs’ tests shows whether there are autoimmune antibodies that cause autoimmune hemolytic anemia.

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Anemia
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Disclaimer:

This outline is only a teaching aid to patients and should stimulate you to ask the right questions when seeing your doctor. However, the responsibility of treatment stays in the hands of your doctor and you

References:

1. Merck Manual (Home edition): Anemia

2. Noble: Textbook of Primary Care Medicine, 3rd ed., Mosby Inc. 2001

3. Goldman: Cecil Medicine, 23rd ed., Saunders 2007: Chapter 162 – APPROACH TO THE ANEMIAS

Last Modified: Feb. 4, 2008