A case of reversible cerebral vasoconstriction syndrome developing during treatment of adult aplastic anemia
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Reversible cerebral vasoconstriction syndrome (RCVS) refers to a group of disorders characterized by “thunderclap headaches” and diffuse narrowing of the cerebral arteries . RCVS sometimes develops after the use of immunosuppressants that are frequently used to treat hematological diseases. However, only two pediatric cases of RCVS in patients with a hematological disease have been reported [2, 3]. Herein, we report the first adult case of RCVS in a patient with aplastic anemia (AA).
It is important to reduce or stop a drug that is causing RCVS, such as CsA and rabbit-ATG [2, 3]. The hypertension and thunderclap headaches ceased immediately after CsA was stopped, but the possibility that rabbit-ATG induced RCVS cannot be discounted.
Magnesium sulfate  and calcium antagonists [3, 5] are useful RCVS therapies, reducing blood pressure and dilating the cerebral vessels. Retreatment of AA with CsA is sometimes inevitable; Ueki et al. retreated AA by CsA with lomerizine without relapsing RCVS . As the hypertension improved after CsA was stopped, we did not use calcium antagonists.
We commenced CsA at a low dose and controlled the blood level because side-effect development depends on the CsA blood concentration . A switch to tacrolimus is one possible strategy when posterior reversible encephalopathy syndrome (PRES) develops . Tacrolimus also causes RCVS , but is effective against AA . The class effect of the various calcineurin inhibitors on RCVS remains poorly known; these materials may be useful treatment options.
A diagnosis of RCVS requires MRA, but MRA findings are sometimes not initially apparent; repeat MRA is recommended ≥ 1 week after onset . Only two cases of RCVS in patients with hematological diseases have been reported despite many such patients taking drugs that can induce RCVS. The condition may be misdiagnosed, because MRA is not routine; possible erroneous diagnoses include PRES. The prognosis of RCVS is thought to be good, but severe complications such as cerebral hemorrhage and infarctions can develop. Accurate diagnosis via MRA is essential; more cases are required to study whether to restart CsA or switch to tacrolimus to treat AA with a history of RCVS.
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Conflict of interest
The authors declare that they have no conflict of interest.
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