GENERAL OBJECTIVES

This study was carried out to identify the clinical, immunological and outcome
characteristics of autoimmune hemolytic anemia patients treated at the Batangas Regional
Hospital in Pediatric Ward.

SPECIFIC OBJECTIVES

To provide information about the signs and symptoms, cause, treatment and management
of hemolytic anemia for the benefits of the patients.

INTRODUCTION

Hemolytic anemia is anemia due to hemolysis, the abnormal breakdown of red blood
cells (RBCs) either in the blood vessels (intravascular hemolysis) or elsewhere in the body
(extravascular). It has numerous possible causes, ranging from relatively harmless to life-
threatening. The general classification of hemolytic anemia is either acquired or inherited.
Treatment depends on the cause and nature of the breakdown. Hemolysis is the
premature destruction of erythrocytes, and it leads to hemolytic anemia when bone marrow
activity cannot compensate for the erythrocyte loss. Clinical presentation depends on
whether the onset of hemolysis is gradual or abrupt and on the severity of erythrocyte
destruction. A patient with mild hemolysis may be asymptomatic. In more serious cases,
the anemia can be life threatening, and patients can present with angina and
cardiopulmonary decompensation. The clinical presentation also reflects the underlying
cause for hemolysis. For example, sickle cell anemia is associated with a painful occlusive
crisis. Hemolytic anemia represents approximately 5% of all anemias.

The overall incidence of death is low in cases of hemolytic anemia. However, older
patients and patients with cardiovascular impairment are at an increased risk. Morbidity is
dependent on the etiology of the hemolysis and the underlying disorder such as sickle cell
anemia or malaria. Most of the disorders that lead to hemolysis are not specific to any
race. Most cases of hemolytic anemia are not sex specific. Autoimmune hemolytic anemia
(AIHA) is slightly more likely to occur in females than in males. G6PD deficiency is an X-
linked recessive disorder. Males are usually affected, and females are carriers. Hemolytic
anemia can occur in persons of any age. Hereditary disorders are usually evident early in
life. Autoimmune hemolytic anemia is more likely to occur in middle-aged and older
individuals.
More than 200 causes for hemolysis exist. The immediate cause of hemolytic anemia is
the early destruction of red blood cells. This means that red blood cells are destroyed and
removed from the bloodstream before their normal lifespan is up. Hereditary disorders
include erythrocyte membrane and enzymatic defects and hemoglobin abnormalities. In
inherited hemolytic anemias, the genes that control how red blood cells are made are
faulty. You can receive a faulty red blood cell gene from one or both of your parents.
Acquired hemolytic conditions can be due to immune disorders, toxic chemicals and drugs,
antiviral agents (eg, ribavirin) physical damage, and infections. In acquired hemolytic
anemias, the body makes normal red blood cells. However, some disease, condition, or
factor destroys the cells too early. Autoimmune hemolytic anemia and hereditary
spherocytosis are classified as examples of extravascular hemolysis because the red
blood cells are destroyed in the spleen and other reticuloendothelial organs. Intravascular
hemolysis occurs in hemolytic anemia due to prosthetic cardiac valves, G6PD deficiency,
thrombotic thrombocytopenic purpura, disseminated intravascular coagulation, and
paroxysmal nocturnal hemoglobinuria (PNH).

Symptoms of hemolytic anemia are diverse and are due to the anemia, the extent of
compensation and previous treatment. Symptoms are chills, dark urine, enlarged spleen,
fatigue, pale skin color (pallor), rapid heart rate, shortness of breath and yellow skin color
(jaundice). The complications depend on the specific type of hemolytic anemia. Severe
anemia can cause cardiovascular collapse. Severe anemias can aggravate heart disease,
lung disease, or cerebrovascular disease.

Treatment depends on the type and cause of the hemolytic anemia. Folic acid, iron
replacement, and corticosteroids may be used. In emergencies, a blood transfusion or
removal of the spleen (splenectomy) may be necessary. Medicines can improve some
types of hemolytic anemia, especially autoimmune hemolytic anemia (AIHA).
Corticosteroid medicines, such as prednisone, are used to limit or prevent your immune
system from making antibodies against red blood cells. Plasmapheresis is a procedure
that removes antibodies from the blood. For this procedure, blood is taken from your body
using a needle inserted into a vein. In some types of hemolytic anemia, such as
thalassemias, the bone marrow doesn't make enough healthy red blood cells. The red
blood cells it does make may be destroyed before their normal lifespan is over. Blood and
marrow stem cell transplants may be used to treat these types of hemolytic anemia. If you
have AIHA with cold-reactive antibodies, try to avoid cold temperatures. This can help
prevent the breakdown of red blood cells. It's very important to protect your fingers, toes,
and ears from the cold.