Cerebrovascular manifestations in hematological diseases: an update

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.

Keywords: Cerebral venous thrombosis; Essential thrombocythemia; Intracerebral hemorrhage; Leukemia; Lymphoma; Myeloma; Myeloproliferative neoplasm; POEMS; PRES; Paroxysmal nocturnal hemoglobinuria; Polycythaemia vera; Sickle cell disease; Stroke; Thrombotic thrombocytopenic purpura.

Fig. 1

Thrombosis of the left lateral sinus (arrow) with a 67 year old male with essential thrombocythemia, presenting with headache and papilledema

Fig. 2

Stroke in acute leukemias: multiple hemorrhages in acute myeloid leukemia with hyperleukocytosis (up); cerebral venous thrombosis in acute monocytic leukemia (bottom)

Fig. 3

Acute stroke in a patient with paroxysmal nocturnal hemoglobinuria, showing an right hemispheric acute and a left frontal chronic infarct on MR DWI sequence

Fig. 4

Uncountable microbleeds in a fatal case of thrombotic thrombocytopenic purpura

Fig. 5

Posterior reversible encephalopathy syndrome in a young patient with T-cell lymphoblastic lymphoma, presenting with headache and seizure after first chemotherapy cycle (pegylated l-asparaginase, vincristine, doxorubicin, and methotrexate): bilateral asymmetrical parieto-frontal hyperintensities on FLAIR sequence