Clin. lab. Haemat. 1992, 14, 209-21 1
Hydroxyethyl starch induced acquired von Willebrand’s disease M . D A L R Y M P L E - H A Y , M B , C hB *, R. A I T C H I S O N , M R C P , M R C P a t h , P. C O L L I N S , M R C P , M. SEKHAR, MRCP & B. COLVIN, FRCP, FRCPath Departments of Haematology and *Surgery, Royal London Hospital, London E l 1BB Accepted for publication 3 1 January 1992 Summary Hydroxyethyl starch ( H E S ) (Hespan, DuPont) is a widely used synthetic volume expander which in standard doses of up to 1.5 1 in 24 h has no significant effect on coagulation (Munsch et al. 1988). However, the data sheet states that in large volumes HES may alter the coagulation mechanism. We now report a case of HES induced acquired von Willebrand’s disease (vWD) in which severe bleeding occurred. Keywords: hydroxyethyl starch, von Willebrand’s disease
A 33-year-old man presented in 1976 with a cerebellar astrocytoma. This was treated with excision and radiotherapy but recurred in 1980. He developed steroid induced avascular necrosis of the femoral heads and had bilateral hip arthroplasties performed in 1988 without any abnormal bleeding although he was noted to have a prolonged activated partial thromboplastin time (APTT) with normal thrombin time (TT) and prothrombin time (PT) (see Table 1). This was not further investigated at presentation. In November 1990 an acute right sided sensori-neural hearing loss occurred so it was decided to use HES in an attempt to improve the blood supply to the right ear. After 3 litres of HES given over 3 days he had an episode of epistaxis, and then complained of weakness and pain in his left leg. On examination he had some sensory loss in the leg with bilateral brisk reflexes and extensor plantar responses. At this time his APTT was significantly prolonged at 81 sec again with normal PT and TT. A further 1.5 litres of HES was given over the next 36h. However, the neurological signs worsened and the clotting screen was still abnormal so it was thought possible that the weakness was due to bleeding into his spinal cord. The HES was therefore stopped. The day after, his APTT was 74 sec with marked reduction in F VIIIC, vWF Ag, vWF and Factor XI1 (see Table 1). von Willebrand multimer analysis showed a generalized reduction in all sizes of multimer. Over the next few days his clotting parameters improved; so that 4 days after the HES was stopped the VIIIC, vWFAg and vWF had returned to normal while Factor XI1 remained low at 0.08 iu/ml. A myelogram was performed which was normal. His neurological deficit slowly improved Correspondence: M. Dalrymple-Hay.
209
210
M . Dalryrnple-Hay
et
al.
Table 1. Haemostatic parameters ~~
Date
APTT (sec) Factor VlIIC VWF : Ag VWF Factor XI1
~~~~~
1988 30.11.90 2.12.90 3.12.90 4.12.90 5.12.90 6.12.90 11.12.9021.12.90 31.1.91 51
81
97
74 0.11
Hydroxyethyl starch induced acquired von Willebrand’s disease M . D A L R Y M P L E - H A Y , M B , C hB *, R. A I T C H I S O N , M R C P , M R C P a t h , P. C O L L I N S , M R C P , M. SEKHAR, MRCP & B. COLVIN, FRCP, FRCPath Departments of Haematology and *Surgery, Royal London Hospital, London E l 1BB Accepted for publication 3 1 January 1992 Summary Hydroxyethyl starch ( H E S ) (Hespan, DuPont) is a widely used synthetic volume expander which in standard doses of up to 1.5 1 in 24 h has no significant effect on coagulation (Munsch et al. 1988). However, the data sheet states that in large volumes HES may alter the coagulation mechanism. We now report a case of HES induced acquired von Willebrand’s disease (vWD) in which severe bleeding occurred. Keywords: hydroxyethyl starch, von Willebrand’s disease
A 33-year-old man presented in 1976 with a cerebellar astrocytoma. This was treated with excision and radiotherapy but recurred in 1980. He developed steroid induced avascular necrosis of the femoral heads and had bilateral hip arthroplasties performed in 1988 without any abnormal bleeding although he was noted to have a prolonged activated partial thromboplastin time (APTT) with normal thrombin time (TT) and prothrombin time (PT) (see Table 1). This was not further investigated at presentation. In November 1990 an acute right sided sensori-neural hearing loss occurred so it was decided to use HES in an attempt to improve the blood supply to the right ear. After 3 litres of HES given over 3 days he had an episode of epistaxis, and then complained of weakness and pain in his left leg. On examination he had some sensory loss in the leg with bilateral brisk reflexes and extensor plantar responses. At this time his APTT was significantly prolonged at 81 sec again with normal PT and TT. A further 1.5 litres of HES was given over the next 36h. However, the neurological signs worsened and the clotting screen was still abnormal so it was thought possible that the weakness was due to bleeding into his spinal cord. The HES was therefore stopped. The day after, his APTT was 74 sec with marked reduction in F VIIIC, vWF Ag, vWF and Factor XI1 (see Table 1). von Willebrand multimer analysis showed a generalized reduction in all sizes of multimer. Over the next few days his clotting parameters improved; so that 4 days after the HES was stopped the VIIIC, vWFAg and vWF had returned to normal while Factor XI1 remained low at 0.08 iu/ml. A myelogram was performed which was normal. His neurological deficit slowly improved Correspondence: M. Dalrymple-Hay.
209
210
M . Dalryrnple-Hay
et
al.
Table 1. Haemostatic parameters ~~
Date
APTT (sec) Factor VlIIC VWF : Ag VWF Factor XI1
~~~~~
1988 30.11.90 2.12.90 3.12.90 4.12.90 5.12.90 6.12.90 11.12.9021.12.90 31.1.91 51
81
97
74 0.11
