Cerebrovascular manifestations in hematological diseases: an update

José M Ferro,Joana Infante

doi:10.1007/s00415-021-10441-9

Abstract

Patients with hematological diseases often experience cerebrovascular complications including ischemic stroke, intracerebral and subarachnoid hemorrhage, microbleeds, posterior reversible encephalopathy syndrome, and dural sinus and cerebral vein thrombosis (CVT). In this update, we will review recent advances in the management of cerebrovascular diseases in the context of myeloproliferative neoplasms, leukemias, lymphomas, multiple myeloma, POEMS, paroxysmal nocturnal hemoglobinuria (PNH), thrombotic thrombocytopenic purpura (TTP), and sickle-cell disease. In acute ischemic stroke associated with hematological diseases, thrombectomy can in general be applied if there is a large vessel occlusion. Intravenous thrombolysis can be used in myeloproliferative neoplasms and sickle-cell anemia, but in other diseases, a case-by-case evaluation of the bleeding risks is mandatory. Patients with sickle-cell disease and acute stroke need very often to be transfused. In PNH, acute ischemic stroke patients must be anticoagulated. Most patients with CVT can be treated with low-molecular weight heparin (LMWH) acutely, even those with leukemias. Prevention of recurrence of cerebral thrombotic events depends on the control of the underlying disease, combined in some conditions with antithrombotic drugs. The recent introduction of specific monoclonal antibodies in the treatment of PHN and TTP has dramatically reduced the risk of arterial and venous thrombosis.

Highlights

In registries of busy comprehensive stroke centers, hematological diseases are a very rare cause of stroke, in particular of ischemic stroke [1, 2]
Hematological diseases are a rare cause of stroke, but cerebrovascular complications are frequent in patients with hematological diseases
The evidence supporting decisions in stroke acute treatment and secondary prevention varies in quality from high to very low

Summary

Introduction

In registries of busy comprehensive stroke centers, hematological diseases are a very rare cause of stroke, in particular of ischemic stroke [1, 2]. Patients with multiple myeloma (MM) are at increased risk of ischemic and hemorrhagic stroke and cerebral venous thrombosis [28, 33]. The spectrum of cerebrovascular disease in PNH comprises cerebral venous thrombosis (CVT), single or multiple TIA and cortical [53] or lacunar [54] ischemic stokes (Fig. 3), and other rarer manifestation such as Moyamoya syndrome [55] and PRES [56]. Therapeutic strategies for PNH are bone marrow transplantation as the only curative treatment for the bone marrow failure component of disease, and monoclonal antibody complement inhibitors, such as eculizumab (anti-C5 monoclonal antibody), which controls complement-mediated intravascular hemolysis, reduces the risk of recurrent arterial and venous thrombosis, and prolongs survival [51]. PRES has a lower occurrence in the auto-transplant population (0.86%) [87]

Conclusions and future directions

Findings

Compliance with ethical standards