Safety and efficacy of plasma exchange therapy for Kawasaki disease in children in intensive care unit: case series

Satoko Noguchi,Tomoyuki Kudo,Eiji Hashiba,Kazuyoshi Hirota,Junichi Saito

doi:10.1186/s40981-018-0156-3

Satoko Noguchi, Tomoyuki Kudo + Show 3 more

Open Access

https://doi.org/10.1186/s40981-018-0156-3

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Abstract

BackgroundWe have reviewed four cases of Kawasaki disease treated with plasma exchange with 5% albumin in electrolyte-balanced solution, according to the recommended guidelines for Kawasaki disease in the intensive care unit, as their responses to intravenous immunoglobulin therapy were poor.Case presentationThe four cases were aged between 5 months and 3 years and weighted between 6.4 and 15.6 kg. The plasma levels of C-reactive protein were significantly decreased after plasma exchange (p < 0.05). The dilatations of the coronary artery were found in two cases, but both of them were ameliorated until 1 month after the onset and the other cases recovered without any complications. However, we recognized that one case showed marked decreases in coagulation factors, especially in fibrinogen after each plasma exchange even with a transfusion of fresh frozen plasma.ConclusionsPlasma exchange with 5% albumin was effective for refractory Kawasaki disease. However, as there was a possibility of coagulation disorder, attention should be given to changes in coagulation factors like fibrinogen, especially in small patients who need frequent plasma exchange.

Highlights

We have reviewed four cases of Kawasaki disease treated with plasma exchange with 5% albumin in electrolyte-balanced solution, according to the recommended guidelines for Kawasaki disease in the intensive care unit, as their responses to intravenous immunoglobulin therapy were poor.Case presentation: The four cases were aged between 5 months and 3 years and weighted between 6.4 and 15.6 kg
Plasma exchange with 5% albumin was effective for refractory Kawasaki disease
As there was a possibility of coagulation disorder, attention should be given to changes in coagulation factors like fibrinogen, especially in small patients who need frequent plasma exchange

Summary

Conclusions

We report four cases of KD that required PE therapies performed with 5% albumin instead of fresh frozen plasma (FFP), according to the guidelines for KD [1]. There were no severe complications, one case showed a marked disturbance of coagulation factors, such as fibrinogen even with FFP transfusion after PE. Suppressing the inflammatory response at an early stage was considered to prevent coronary complications. Taguchi et al suggested a probability of coagulation disturbance after PE with albumin [7], there was no detailed data before and after each PE in their report. We did not experience hemorrhagic complications in our cases, but it is important to observe the coagulation state around PE therapy with albumin. Selective PE using a special plasma separator, which can remove albumin with antibodies and cytokines, but retaining coagulation factors like fibrinogen, is an alternative method.

Background