Scd-ery.babawith Luhanga And Neil

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Paediatric Red Blood Cell  Exchange Introduction Baba Inusa Adult Perspective Neil Westerdale Paediatric and adolescents Luhanga Musumadi 24th September, 2008 HCK

Indications for Transfusion in  Sickle Cell Disease   2.     sickling by lowering Hb S conc.     Prevention of recurrent stroke or                                   first clinical stroke (abnormal TCD)  Chronic debilitating painful crises  Chronic lung disease with arterial       hypoxaemia HCK

Complications of Long­term  Transfusion     Transfusional Iron Overload

­ Delayed growth and sexual maturation               due to endocrine disturbances ­ Diabetes mellitus ­ Cirrhosis ­ Cardiac arrhythmias and heart failure ­ Death >15­20 yr. of chronic transfusion

HCK

Complications of Transfusion Alloimmunization Transfusion­transmitted Infections

Transfusional Iron Overload

HCK

HCK

Risk of Recurrent Stroke    If presented within 24 hours of symptom  onset, patients who received simple  transfusion had a five times greater risk of  recurrent stroke when compared to  children who received exchange  transfusion therapy (95% CI 1.3, 18.6).    No difference in odds of recurrent stroke  was found for any type of treatment after  24 hours of symptoms. HCK

Multivariate analysis

  Risk of recurrent stroke was 8  times higher for patients  receiving simple transfusion  within 24 hours of symptoms  and in the absence of an  antecedent medical event (95%  CI 1.6, 38.8).

HCK

Risk of Recurrent Stroke (n= 81)

Cumulative Stroke-Free Interval

1.0

.8

Type of Transfusioin

.6

exchange transfusion exchange transfusion

.4

-censored simple transfusion

.2

simple transfusion -censored

0.0 0

5

10

15

20

25

Time from first stroke to second stroke (years after stroke) HCK

Pathophysiology

BLOOD VISCOSITY (cP)

7

6

5

4

Transfusion Pre – Post –

3

2 10

20

30

40

50

HAEMATOCRIT (%)

Jan et al, Transfusion,1982, 22(1):19 HCK

150

150

140

140

CBF [mL/100g/min]

CBF [mL/100g/min]

Why would exchange transfusion be better than  simple transfusions for decreased risk of strokes?

130 120 110 100 90 80 8

8.5

9

9.5

10

10.5

11

130 120 110 100 90 80

11.5

Hb [g/dL]

5

10

15 20 25 30 35 40 45 50 55

HbS [%]

Hurlet-Jensen et al, Stroke, 1982, 25(8):1690 HCK

ERYTHROCYTAPHAERESIS  Reduce or prevent iron accumulation                                       while preventing  recurrent stroke in patients with sickle  cell  disease and stroke

HCK

Rationale for Erythrocytaphaeresis  in Reducing Iron Overload With exchange transfusion, RBCs from patients  are removed while donor RBCs are being given,  resulting in fewer RBCs to contribute iron to the  body in comparison with simple transfusion.

HCK

Erythrocytaphaeresis  Goals ◆ Removal of patient’s RBCs: 

­ Reduction of blood viscosity in polycythemia ­ Reduction of excessive iron in non­anemic                                 hemochromatosis

◆ Exchange patient’s abnormal RBCs       with normal RBCs:  ­ Hereditary or acquired RBC disorders HCK

Erythrocytaphaeresis in SCD 

As a treatment or prevention of  acute complications: ●  Acute chest syndrome ●  Stroke

●  Pre­op (when Hb > 10 g/dl)

  As a long­term transfusion therapy

HCK

Long­Term Erythrocytaphaeresis   Sickle Cell Dis with Stroke   < first 3 yr

Pre­Ex Hb S

 < 30%    

 > 3 yr < 50%*

End Hb S (%)        12 ­ 15 

20 ­ 25

End Hct (%)     

27 ­ 36

    27 ­ 36

• Patient must be clinically and neurologically  stable before raising the target Hb S to <50%     from <30%. HCK

Acute Erythrocytaphaeresis              Advantages

◆ Rapid     in Hct level without               volume overload ◆  Rapid    Hb S level without     substantially    the Hct level ◆    rate of transfusional iron loading ◆  Duration of procedure shorter

HCK

Long­term  Erythrocytaphaeresis in SCD Advantages  Reduces the transfusional iron accumulation     ­ Delays onset of transfusional iron­induced         organ damage     ­ Delays initiation of iron chelation therapy     ­ Prevents the need for iron chelation therapy 

HCK

ERYTHROCYTAPHAERESIS               Disadvantages  ◆ Problem with venous access

◆ Increased blood usage

◆ Increased no. of donor exposure ◆ Increased cost for the proc. (?) ◆ Universally not available 

HCK

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