Correspondence

of the classification criteria for definite antiphospolipid syndrome

(APS). Journal of Thrombosis and Haemostasis, 4, 295306.
Pengo, V., Tripodi, A., Reber, G., Rand, H., Ortel, L., Galli, M. &
De Groot, P.G. (2009) Update of the guidelines for lupus anticoagulant detection. Journal of Thrombosis and Haemostasis, 7,
17371740.
Rosti, V. (2000) The molecular basis of paroxysmal nocturnal hemoglobinuria. Haematologica, 85, 8287.
Socie, G., Gramont, A., Rio, B., Leporrier, M., Rose, C., Heudier, P.,
Rochant, H., Cahn, J.Y. & Gluckman, E. (1996) Paroxysmal

nocturnal haemoglobinuria: long term follow-up and prognostic

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Keywords: paroxysmal nocturnal haemoglobinuria, thrombotic complication, antiphospholipid antibodies, anticardiolipin antibodies, lupus anticoagulant.
First published online 15 June 2010
doi:10.1111/j.1365-2141.2010.08230.x

The myths about platelet transfusions in immune-mediated

thrombocytopenias

Platelet transfusions were shown to reduce mortality from

haemorrhage in patients with acute leukaemia in the 1950s.
Since then, their use has become an essential part in the
management of thrombocytopenic and thrombocytopathic
individuals with significant bleeding. However, despite any
substantial evidence, platelet transfusions are withheld inappropriately for the risk of fear of adding fuel to the fire and
worsening the thrombotic complications in conditions like
thrombotic thrombocytopenic purpura (TTP) and heparininduced thrombocytopenia (HIT). In addition, transfusion of
platelets is deemed ineffective (due to rapid immune destruction) in patients with very low platelet counts secondary to
immune thrombocytopenia (ITP). At the same time, many
patients with these conditions may require platelet transfusions
at least occasionally, to prevent catastrophic bleeding, before an
interventional procedure or a definitive diagnosis is made.
Several reviews and the British Society for Standards in
Haematology guidelines state that platelet transfusions should
only be considered in patients with TTP in the event of lifethreatening haemorrhage. TTP, itself being a thrombotic
disorder, is rarely associated with serious haemorrhage.
However, these patients may require invasive procedures, such
as central line insertion for plasma exchange, which can be
associated with bleeding. Lozano et al (2005) reported the case
of a 45-year-old woman who required a central venous
catheter for plasma exchange because of poor peripheral
access. Her platelet count was 6 109/l and attempts at the
insertion of a femoral line by an experienced anaesthetist were
unsuccessful but resulted in a haematoma. However, a jugular
line was successfully placed after five buffy-coat platelet
transfusions (platelet increment was 86 109/l) and plasma
exchange was initiated promptly with no reported complications. Platelet concentrates may also be necessary in critically ill
patients with thrombocytopenia where the diagnosis of TTP
has been considered but not confirmed. The most compre494

hensive literature review in this context was undertaken by

Swisher et al (2009) who performed a search for patient
outcomes after platelet transfusions in 77 case series of 25 or
more patients with TTP published since 1970. They identified
only 12 case series that described patient outcomes after
platelet transfusions concluding that serious deterioration of
the clinical condition related to the transfusion would have
been unlikely. The authors also reported on the 33 patients
from the extensive Oklahoma TTP-HUS (Haemolytic uremic
syndrome) registry who received platelet transfusions. There
was no demonstrable relationship between transfusions and
neurological or thrombotic abnormalities with surprisingly any
difference among patients who received the transfusions before
or after the initiation of plasma exchange.
HIT is an increasingly recognised condition often noted in
the postoperative setting and in seriously ill patients in the
Intensive Care Unit. In these clinical situations, the definitive
diagnosis of HIT can be difficult due to possible other reasons
for thrombocytopenia, which also increases the risk of
bleeding. If HIT has been suspected in these cases, platelet
transfusion is considered a contraindication for the concern of
promoting platelet aggregation. However, as most clinicians
would appropriately stop any form of heparin if HIT was a
diagnostic possibility, it is worth noting that the trigger factor
for further platelet aggregation and thrombosis with HIT has
been eliminated. Also the alternate anticoagulation that would
have been commenced should prevent any further thrombotic
complications, which are theoretically possible from further
platelet transfusions. The notion that transfused platelets will
increase the chance of thrombosis with HIT is based on a
single report by Babcock et al (1976), which described one
patient who developed an arterial thrombosis after platelet
transfusion. A recent report of platelet transfusions in four out
of 13 patients who tested positive for HIT by serotonin release
assay demonstrated no thrombotic consequences (Hopkins &

2010 Blackwell Publishing Ltd, British Journal of Haematology, 150, 480497

Correspondence
Goldfinger, 2008). Surprisingly although heparin was discontinued in all four patients, alternative anticoagulation was not
initiated in these patients due to active bleeding. The authors
concluded that there was little substantive evidence for
withholding platelet transfusions in patients with HIT when
clinically indicated although they did advise caution in patients
who developed thrombosis related to HIT.
ITP is another immune mediated condition where exogenous platelet replacement has been historically considered to
be ineffective due to rapid immune destruction. As such, the
initial treatment in patients with low platelet count accompanied by mild to moderate haemorrhagic symptoms is usually
steroids or intravenous immunoglobulins with platelet transfusions reserved for those with life-threatening bleeding.
However, it can take at least 24 h for parenteral immunoglobulins, and up to many days for the different forms of
steroids, before a positive effect on the platelet count is
observed. In the meantime, patients (especially adults with
ITP) with very low platelet counts remain at risk of serious
bleeds and may benefit from platelet transfusion to a safe level
(for example; 5 or 10 109/l). A study of the efficacy of
platelet transfusions in 11 patients with idiopathic and
quinine-induced thrombocytopenia demonstrated a successful
platelet increment (>20 109/l) in all the patients (Carr et al,
1986). Although platelet counts decreased rapidly, as would be
expected in an immune-mediated disease, 16% of all transfusions resulted in persistently elevated next-day increments.
In conjunction with this finding, the latest consensus guidance
on the management of ITP supports the appropriateness of
platelet transfusions especially in conjunction with immunoglobulins (evidence level III/IV) (Spahr & Rodgers, 2008;
Provan et al, 2010). Multiple doses of platelets without any
concurrent drugs have also been shown to be effective in
refractory cases of ITP. In a study by Salama et al (2008), 10
patients with refractory ITP and bleeding, or a high bleeding
risk, were consecutively transfused (one unit every 30 min)
with apheresis platelets (37 units). An increase in the platelet
count to an average of 100 109/l was demonstrated in all
cases. In this context, it would be interesting to compare the
incremental response to platelet transfusions in patients with
ITP in whom decreased production from megakaryocytes,
rather than increased peripheral destruction, is the primary
pathophysiological factor.
In summary, the general notion that platelet transfusions are
best avoided in immune-mediated thrombocytopenias like
TTP, HIT or ITP are not supported by substantial evidence.

Although exogenous platelets may very often be unnecessary in

such patients, they should not be withheld if a safe platelet
count is preferable until a definitive diagnosis can be made or
an interventional procedure is required urgently.
Jecko Thachil
Department of Haematology, Roald Dahl Haemostasis and Thrombosis
Centre, Royal Liverpool University Hospital, Liverpool, UK.
E-mail: jeckothachil@yahoo.co.uk

References
Babcock, R.B., Dumper, C.W. & Scharfman, W.B. (1976) Heparin
induced immune thrombocytopenia. New England Journal of Medicine, 295, 237241.
Carr, J.M., Kruskall, M.S., Kaye, J.A. & Robinson, S.H. (1986) Efficacy
of platelet transfusions in immune thrombocytopenia. American
Journal of Medicine, 80, 10511054.
Hopkins, C.K. & Goldfinger, D. (2008) Platelet transfusions in heparin-induced thrombocytopenia: a report of four cases and review of
the literature. Transfusion, 48, 21282132.
Lozano, M., Domingo, A., Pereira, A., Fontanals, J. & Mazzara, R.
(2005) Platelet transfusion in thrombotic thrombocytopenic
purpura: between Scylla and Charybdis. Transfusion, 45, 884.
Provan, D., Stasi, R., Newland, A.C., Blanchette, V.S., Bolton-Maggs,
P., Bussel, J.B., Chong, B.H., Cines, D.B., Gernsheimer, T.B.,
Godeau, B., Grainger, J., Greer, I., Hunt, B.J., Imbach, P.A., Lyons,
G., McMillan, R., Rodeghiero, F., Sanz, M.A., Tarantino, M.,
Watson, S., Young, J. & Kuter, D.J. (2010) International consensus
report on the investigation and management of primary immune
thrombocytopenia. Blood, 115, 168186.
Salama, A., Kiesewetter, H., Kalus, U., Movassaghi, K. & Meyer, O.
(2008) Massive platelet transfusion is a rapidly effective emergency
treatment in patients with refractory autoimmune thrombocytopenia. Thrombosis Haemostasis, 100, 762765.
Spahr, J.E. & Rodgers, G.M. (2008) Treatment of immune-mediated
thrombocytopenia purpura with concurrent intravenous immunoglobulin and platelet transfusion: a retrospective review of 40
patients. American Journal of Hematology, 2008, 83.
Swisher, K.K., Terrell, D.R., Vesely, S.K., Kremer Hovinga, J.A.,
Lammle, B. & George, J.N. (2009) Clinical outcomes after platelet
transfusions in patients with thrombotic thrombocytopenic
purpura. Transfusion, 49, 873887.

Keywords: platelet, thrombocytopenia, transfusions.

First published online 9 May 2010
doi:10.1111/j.1365-2141.2010.08231.x

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