Blood Transfusion Therapy for Thalassemia:Frequency,Complications

4th Pediatric Infectious Diseases Conference

ISSN 0973 - 0958

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Hemoglobin Variants Detection by High Performance Liquid Chromatography Method

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Results

MODERN TRENDS IN MANAGEMENT OF THALASSEMIA

Related Topics

Thalassemia Epidemiology and Pathophysiology

b-thalassemia Clinical Manifestations,Diagnosis and Management

Iron Overload and Chelation Therapy

Splenectomy,Bone Marrow Transplantation and Neocyte Transfusion

Pharmacological Methods To Increase Gamma Chain Production and Gene Manipulation

Dr.M.R.Lokeshwar
Consultant Pediatric Hematologist,
Imperial Mahal, 3rd floor, Dadar TT, Mumbai - 400014.

Continued...

TRANSFUSION THERAPY :

Amount and Frequency of transfusion :

It is desirable that patients receive not more than 10 cc packed cells/kg/day, which raises Hb level by about 3.5 gm/dl. In most of the patients, transfusion of about 10cc of packed RBC/kg every third week is adequate to maintain Pre-transfusion baseline Hb level at desired 10-11 gm/dl.
Rate of transfusion should be 5-7 ml/kg body weight/hour to avoid sudden increase in blood volume.
In patients with cardiac insufficiency, transfusions may have to be given every second week and sometimes every week. The duration of transfusion should be prolonged by decreasing the rate to 1-3 ml/kg/hr and not more than 5 ml/kg/hour.

How often to transfuse :

Transfusions should preferably be given on an out-patient basis, at intervals of 2-4 weeks.
Blood to be transfused should be crossmatched using Coomb's sera to minimize reactions. Blood should be taken from a voluntary donor and should be screened for hepatitis B antigen, HCV, Syphilis, malaria and HIV.

The patients should be assessed annually for mean hemoglobin levels maintained overall blood requirement, physical growth and development, evidence of hypersplenism, antibody development and iron overload.

On an average, the annual blood requirement is 180-200 ml of blood/kg. However, if the requirement exceeds this level, hypersplenism or development of anti-red cell antibodies have to be considered.

Complications of transfusions :

Febrile Reactions :
- It is seen in 3-20% of patients and may be due to leucocyte or platelet antibodies, antibodies against RBC antigens, allergic reactions to other plasma or blood proteins or due to pyrogens present in transfused blood. Chills followed by fever may occur within an hour of transfusion or may be delayed for 24 hours. Headache, nausea, and vomiting may be associated.
- Febrile reactions usually respond to anti-pyretic and anti-histaminic agents. Leucocyte filters are also effective in eliminating neutrophils, which are responsible for pyrogenic reactions.
Hemolytic Transfusion Reactions : It occurs in 5-15% of cases.
- These are due to major or minor blood group mismatch and are characterized by fever, chills, tachycardia, nausea, vomiting,pallor, restlessness, anxiety, flushing of face, precordial oppression and pain, increased pulse rate and respiratory rate, generalized tingling sensation, pain in back and thigh, shock with cold and clammy skin, cyanosis and collapse. Delirium and convulsions may develop. Most feared complications are acute renal failure, hemorrhagic diathesis due to DIC and anaphylactic shock, all of which may be of life threatening proportions.
- Patients may develop indirect hyperbilirubinemia, hemoglobinemia, hemoglobinuria, reticulocytosis and positive Coomb's test and can occasionally go into acute renal failure and shock.
- On onset of any early symptoms, transfusion should be stopped immediately. Anything unusual that alarms the mother should be respected. Older children (with Thalassemia) sometimes can even sense that something has gone wrong. As the mother always remembers the blood group of the child, it is a sound policy to show her the label on the bottle before starting the transfusion. For the first fifteen minutes, transfusion should be given slowly so as to detect any reactions at the earliest moment.
- If facilities are available, all patients should be typed for the common RBC antigens ie Rh, Cc, Dd, Ee, Kell, Kidd and Duffy (before starting regular transfusion). Monitoring of the antibodies must be continued life long.
Other reactions uncommonly seen and thought to be of uncertain etiology are sudden development of hypertension, convulsions, cerebral hemorrhage and edema after multiple transfusions.

Transfusion transmitted diseases like malaria, syphilis, hepatitis B, Hepatitis C, Cytomegalovirus, and HIV infection can occur. All thalassemics who are negative for the hepatitis B surface antigen and antibody, should receive hepatitis b vaccine -4 doses at day 0, 1 month, 2nd month and 12th month intramuscularly. It also can be given intradermally in the dose of 0.1 cc, thus reducing the cost. This has been found to be effective by some workers. However, efficacy is not well proven.