Letter on Sickle Cell Disease--Amendment/Clarification
The author has highlighted one of the potential serious complications
of transfusion seen in sickle cell disease(SCD).
Death due to Hyperhaemolysis syndrome(HS) has been reported in both SCD
(1)and patient with Myelofibrosis(2).
The author has mentioned that "subsequent cautious transfusions should be
covered with intravenous immunoglobulin (IVIG)[high dose and low dose
The doses should read 1g/kg/day for 2 days(for high dose regime) and
0.4g/kg/day for 5 days(low dose regime). Not 1mg/kg/day for 2 days or
0.4mg/kg/day for 5 days mentioned in the letter.
High index of suspicion and awareness of HS is important as additional
transfusion may further exacerbate haemolysis and may lead to chronic
protracted course (3)or
even death(1,2). Several approaches have been implemented in managing HS.
In mild form, additional transfusion should be avoided and oral
prednisolone (1-2 mg /kg/day) should be tried(3).
If presented with severe and rapid haemolysis, the patient may require
additional transfusion with IVIG and steroids cover.
We have recommended IVIG low dose regime(0.4g/kg/day for 5 days). The use
of IVIG should be selective because it has been associated with renal
and an estimated 0.6 per cent risk of stroke and patients with SCD may be
predisposed to such adverse events. Therefore we prefer the low dose
regime rather than providing high dose IVIG. We prescribed IV
methylprednisolone 0.5g to 1g for 2 days in conjunction with IVIG which
appears to have synergistic effect in suppressing haemolysis(4,5).
1)Friedman DF, Kim HC, Manno CS. Hyperhaemolysis associated with red cell
transfusion in sickle cell disease. Abstract. Transfusion 1993;33Suppl:14S
2)Treleaven JG, Win N. Hyperhaemolysis syndrome in a patient with
myelofibrosis. Haematology 2004;9:147-9
3)Petz L D, Calhoun L, Shulman I, Johnson C, Herron R. The sickle cell
haemolytic transfusion reaction syndrome. Transfusion 1997;37:382-92
4) Win N, New H, Lee E, de la Fuente. Hyperhaemolysis syndrome in sickle
cell disease: case report (recurrent episode)and literature review.
5)Win N, Tullie Y, Needs M, Chen FE, Okpala I. Use of intravenous
immunoglobulin and intravenous methylprednisolone in hyperhaemolysis
syndrome in sickle cell disease. Haematology 2004;9:433-6
Dr Nay Win
Consultant (Transfusion Medicine)
NHS - Blood & Transplant,
National Blood Service (Tooting Centre),
75 Cranmer Terrace,
London SW17 0RB
Competing interests: No competing interests