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Blood Transfusion in Sickle Cell Disease
- 1. Transfusion in Sickle CellDisease
- 2. Learning objectives Learning objectives Youshould be able to You should be able to Discuss the indications for blood transfusion in sickle Discuss the indications for blood transfusion in sickle cell disease cell disease Compare and contrast ‘top up’ and exchange Compare and contrast ‘top up’ and exchange transfusion transfusion Explain the disadvantages of blood transfusion in this Explain the disadvantages of blood transfusion in this patient group patient group Explain what specific precautions are necessary if Explain what specific precautions are necessary if patients with sickle cell disease require transfusion patients with sickle cell disease require transfusion Discuss the role of a chronic transfusion programme Discuss the role of a chronic transfusion programme in the prevention of stroke in sickle cell disease in the prevention of stroke in sickle cell disease
- 3. Case Case 30-year-old Fknown HbSS 30-year-old F known HbSS Moderately frequent painful crises Moderately frequent painful crises 1 previous chest crisis requiring exchange 1 previous chest crisis requiring exchange transfusion transfusion Diffuse lymphadenopathy for months Diffuse lymphadenopathy for months Admitted as day case under surgeons for Admitted as day case under surgeons for biopsy of cervical lymph node biopsy of cervical lymph node Hb 6.2 g/dl Hb 6.2 g/dl TRANSFUSE??.... TRANSFUSE??....
- 4. Case … Case … Baseline Hb 5-6 g/dl Baseline Hb 5-6 g/dl Well preoperatively Well preoperatively Not transfused, managed with good Not transfused, managed with good hydration and oxygenation hydration and oxygenation Discharged well post op Discharged well post op
- 5. Case … Case … Readmitted via A+E 5 days later with swelling Readmitted via A+E 5 days later with swelling of neck and fevers of neck and fevers Seen by ENT team, started antibiotics and Seen by ENT team, started antibiotics and steroids because of concerns re airway steroids because of concerns re airway compromise compromise Later O Later O2 2 sats 89% on air, increasingly sats 89% on air, increasingly breathless but sats improve with oxygen breathless but sats improve with oxygen Hb 6.1 g/dl Hb 6.1 g/dl TRANSFUSE??.... TRANSFUSE??....
- 6. Case … Case … Transfused 2 units Transfused 2 units Later in day sats dropping on oxygen, tiring, Later in day sats dropping on oxygen, tiring, CXR shows bilateral patchy shadowing CXR shows bilateral patchy shadowing Concerns re airway compromise Concerns re airway compromise Worsening respiratory parameters Worsening respiratory parameters Reviewed by ITU > for semi-elective intubation Reviewed by ITU > for semi-elective intubation On ITU intubated On ITU intubated Hb 8.4 g/dl Hb 8.4 g/dl TRANSFUSE??.... TRANSFUSE??....
- 8. Case … Case … HbS 46% HbS 46% Treated as chest crisis Treated as chest crisis Aiming for what HbS percentage??.... Aiming for what HbS percentage??....
- 9. Case … Case … HbS 46% HbS 46% Treated as chest crisis Treated as chest crisis Aiming for HbS <30% Aiming for HbS <30% Exchanged 3 units Exchanged 3 units Over following days improving from sepsis Over following days improving from sepsis point of view point of view Weaned from ventilator Weaned from ventilator HbS maintained at <30% via intermittent top HbS maintained at <30% via intermittent top up transfusions up transfusions
- 10. Types/Aims of Transfusion Types/Aimsof Transfusion 1. 1. Simple/ “top up” transfusion Simple/ “top up” transfusion Used to treat severe anaemia in SCD Used to treat severe anaemia in SCD Examples of situations??.... Examples of situations??....
- 11. Types/Aims of Transfusion Types/Aimsof Transfusion 1. 1. Simple/ “top up” transfusion Simple/ “top up” transfusion Used to treat severe anaemia in SCD Used to treat severe anaemia in SCD – In situations as in general population eg. GI In situations as in general population eg. GI bleeding, trauma bleeding, trauma – Aplastic crisis – parvovirus B19 Aplastic crisis – parvovirus B19 – Haemolytic crises Haemolytic crises – Splenic (and liver) sequestration > transfusion Splenic (and liver) sequestration > transfusion can be life saving can be life saving – Anaemia associated with renal failure in older Anaemia associated with renal failure in older patients with sickle cell disease patients with sickle cell disease Used to dilute HbS % if anaemic Used to dilute HbS % if anaemic
- 13. Types/Aims of Transfusion2 Types/Aims of Transfusion 2 2. Exchange Transfusion 2. Exchange Transfusion Used to reduce HbS % in patients who are not Used to reduce HbS % in patients who are not significantly anaemic significantly anaemic – Stroke/neurological syndromes Stroke/neurological syndromes – Chest crisis Chest crisis – Persistent or recurrent priapism Persistent or recurrent priapism – Pre-surgery (not necessary for minor operations) Pre-surgery (not necessary for minor operations) Keep normovolaemic Keep normovolaemic Manual or cell separator Manual or cell separator
- 15. Types/Aims of Transfusion3 Types/Aims of Transfusion 3 3. Transfusion/exchange transfusion 3. Transfusion/exchange transfusion programmes programmes Regular transfusion/exchange aiming to Regular transfusion/exchange aiming to suppress HbS % in the longer term suppress HbS % in the longer term – Severe anaemia Severe anaemia – Frequent crises esp. if severe e.g. Chest crises Frequent crises esp. if severe e.g. Chest crises – CVA/abnormal brain scan CVA/abnormal brain scan – In children to prevent stroke in those found to be In children to prevent stroke in those found to be at high risk by transcranial doppler ultrasound at high risk by transcranial doppler ultrasound screening screening
- 16. Reminders Reminders In HbSShomozygotes, normal Hb 6-9 g/dl, In HbSS homozygotes, normal Hb 6-9 g/dl, sometimes even lower sometimes even lower No need to transfuse to a “normal Hb” unless No need to transfuse to a “normal Hb” unless symptomatic, large drop in Hb or other indication to symptomatic, large drop in Hb or other indication to do so do so Anaemia is chronic and very well tolerated – why??... Anaemia is chronic and very well tolerated – why??...
- 17. Reminders Reminders In HbSShomozygotes, normal Hb 6-9 g/dl, In HbSS homozygotes, normal Hb 6-9 g/dl, sometimes even lower sometimes even lower No need to transfuse to a “normal Hb” unless No need to transfuse to a “normal Hb” unless symptomatic, large drop in Hb or other indication to symptomatic, large drop in Hb or other indication to do so do so Anaemia is chronic and very well tolerated, partly due Anaemia is chronic and very well tolerated, partly due to decreased O to decreased O2 2 affinity of HbS affinity of HbS All HbSS patients should be on long term folic acid All HbSS patients should be on long term folic acid Dose??.... Dose??.... Why??.... Why??....
- 18. Reminders Reminders In HbSShomozygotes, normal Hb 6-9 g/dl, In HbSS homozygotes, normal Hb 6-9 g/dl, sometimes even lower sometimes even lower No need to transfuse to a “normal Hb” unless No need to transfuse to a “normal Hb” unless symptomatic, large drop in Hb or other indication to symptomatic, large drop in Hb or other indication to do so do so Anaemia is chronic and very well tolerated, partly due Anaemia is chronic and very well tolerated, partly due to decreased O2 affinity of HbS to decreased O2 affinity of HbS All HbSS patients should be on long term folic acid All HbSS patients should be on long term folic acid – Dose 5 mg OD Dose 5 mg OD – Can easily become deficient due to increased rate of red cell Can easily become deficient due to increased rate of red cell turnover turnover Don’t forget>> sickle cell patients can become more Don’t forget>> sickle cell patients can become more anaemic for the same reasons as other patients too anaemic for the same reasons as other patients too – Do not overlook possibility of GI bleeding etc. Do not overlook possibility of GI bleeding etc.
- 19. Complications of Transfusion Complicationsof Transfusion As with any transfusion As with any transfusion – Transmission of viruses and ??prions Transmission of viruses and ??prions – Acute or delayed haemolytic transfusion reactions Acute or delayed haemolytic transfusion reactions – Bacterial contamination Bacterial contamination – TRALI TRALI – Fluid overload Fluid overload – Acute allergic reaction/anaphylaxis Acute allergic reaction/anaphylaxis – PTP PTP Increased viscosity (more later…) Increased viscosity (more later…) Iron overload (more later…) Iron overload (more later…) Alloimmunisation (more later…) Alloimmunisation (more later…)
- 20. Increased Viscosity Increased Viscosity Can exacerbate problems in sickle cell crisis Can exacerbate problems in sickle cell crisis Blood transfused has significantly higher Blood transfused has significantly higher haematocrit then blood in circulation haematocrit then blood in circulation Topping up sickle cell patients with a relatively Topping up sickle cell patients with a relatively good Hb during a crisis is hazardous good Hb during a crisis is hazardous Aim to maintain haematocrit at reasonable Aim to maintain haematocrit at reasonable level level – Also give crystalloids/colloid if necessary Also give crystalloids/colloid if necessary – Exchange transfuse as alternative Exchange transfuse as alternative – Monitor haematocrit regularly during exchange Monitor haematocrit regularly during exchange – As a rough guide, post transfusion Hb should be As a rough guide, post transfusion Hb should be less than 12 g/dl less than 12 g/dl
- 21. Iron Overload Iron Overload A big problem! A big problem! Can lead to Can lead to – Cardiomyopathy, heart failure, arrhythmias, death Cardiomyopathy, heart failure, arrhythmias, death – Hepatic dysfunction, cirrhosis, hepatocellular Hepatic dysfunction, cirrhosis, hepatocellular carcinoma carcinoma – Endocrine dysfunction Endocrine dysfunction Diabetes mellitus Diabetes mellitus Hypogonadism Hypogonadism Hypothyroidism/hypoparathyroidism Hypothyroidism/hypoparathyroidism – Arthropathy (chondrocalcinosis with Arthropathy (chondrocalcinosis with pyrophosphate deposition), osteoporosis pyrophosphate deposition), osteoporosis
- 22. Increased ironin > no mechanism for Increased iron in > no mechanism for increased excretion increased excretion More transfusions > more iron overload More transfusions > more iron overload Monitoring iron overload Monitoring iron overload – Tests of body iron burden (total body iron) Tests of body iron burden (total body iron) Ferritin – simple test, imprecise, useful to Ferritin – simple test, imprecise, useful to monitor changes monitor changes Serum iron, % transferrin saturation Serum iron, % transferrin saturation
- 23. – Tests oforgan iron loading Tests of organ iron loading Liver iron Liver iron – Biopsy Biopsy – SQUID SQUID – MRI T2* MRI T2* Cardiac iron Cardiac iron – MRI T2* MRI T2* – Tests of function of sensitive organs Tests of function of sensitive organs ECG, echocardiography, 24 hour monitor, MUGA ECG, echocardiography, 24 hour monitor, MUGA Liver function tests Liver function tests DEXA scan DEXA scan Glucose, thyroid and other endocrine function Glucose, thyroid and other endocrine function tests tests
- 24. No clearnational guidelines on when to No clear national guidelines on when to start treatment start treatment Generally aim for ferritin <1000 mcg/l Generally aim for ferritin <1000 mcg/l Deferoxamine (desferrioxamine, Desferral) Deferoxamine (desferrioxamine, Desferral) – Not absorbed orally, very short t1/2 Not absorbed orally, very short t1/2 – Given sc by infusion overnight (8-12 hrs) most Given sc by infusion overnight (8-12 hrs) most nights a week, inconvenient, affects quality of nights a week, inconvenient, affects quality of life life – Also sometimes given iv at time of blood Also sometimes given iv at time of blood transfusion or continuously if severe cardiac transfusion or continuously if severe cardiac disease disease
- 25. – Give withascorbic acid (vitamin C) – increases Give with ascorbic acid (vitamin C) – increases urinary iron excretion urinary iron excretion – Removal of liver iron more rapid than Removal of liver iron more rapid than removal of cardiac iron removal of cardiac iron – Can reverse much of the damage over time, Can reverse much of the damage over time, endocrine dysfunction often permanent endocrine dysfunction often permanent – Improvement of cardiac function and survival Improvement of cardiac function and survival – Compliance is a MAJOR problem Compliance is a MAJOR problem – Turin study Turin study 95% compliant patients alive after 30 years 95% compliant patients alive after 30 years 12% non-compliant 12% non-compliant
- 27. – Side effects Sideeffects Sensitivity reactions, pain at infusion site Sensitivity reactions, pain at infusion site Exacerbation of infections esp. Yersinia Exacerbation of infections esp. Yersinia – Consider this in patients with diarrhoea on Consider this in patients with diarrhoea on deferoxamine deferoxamine Auditory toxicity Auditory toxicity Visual and neurotoxicity Visual and neurotoxicity Growth and bone defects Growth and bone defects
- 28. Deferiprone Deferiprone – Givenorally 3 times daily Given orally 3 times daily – Effective with very high starting levels (eg Effective with very high starting levels (eg ferritin >3000) ferritin >3000) – Less so if starting at lower iron load Less so if starting at lower iron load – Side effects can be serious Side effects can be serious Agranulocytosis – need for freq monitoring Agranulocytosis – need for freq monitoring Joint pains Joint pains GI side effects GI side effects – Can give in combination with deferoxamine Can give in combination with deferoxamine
- 29. Deferasirox, recentlylicensed Deferasirox, recently licensed – Given orally once daily Given orally once daily – As effective as deferriprone for unloading As effective as deferriprone for unloading liver iron liver iron – Little data yet re effect on cardiac iron Little data yet re effect on cardiac iron – No need for regular blood count No need for regular blood count monitoring monitoring
- 30.
- 31. Alloimmunisation Alloimmunisation Blood donorpopulation genetically very different from Blood donor population genetically very different from sickle population sickle population Frequent transfusion increases risk of development of Frequent transfusion increases risk of development of alloantibodies to minor antigens alloantibodies to minor antigens Sickle cell population more prone to developing Sickle cell population more prone to developing alloantibodies alloantibodies Can become a big problem for transfusion in the future Can become a big problem for transfusion in the future Use “phenotyped” blood in all patients with sickle cell Use “phenotyped” blood in all patients with sickle cell disease disease Matched for Matched for – ABO group, Rh D group (as with all blood) ABO group, Rh D group (as with all blood) – E E – C C – Kell Kell