ISCHEMIC STROKE / CLASSIFICATION AND ETIOLOGY
Hematologic disorders associated with ischemic stroke
Created 02/05/2023, last revision 04/05/2023
- hematologic disorders cause a relatively small proportion of all ischemic strokes (∼ 1% in the general population and ∼ 4% in young patients)
- in contrast to cerebral venous thrombosis, the association between coagulation abnormalities and arterial stroke is not always clear
- if found responsible, the hematologic disorders are classified as TOAST 4
Pathophysiology
Main mechanisms by which hematologic disease cause stroke
- increased coagulation or aggregation with thrombus formation intravascularly or in cardiac cavities with subsequent embolization (typically inherited coagulation disorders)
- hyperviscosity syndrome (corpuscular or noncorpuscular), in which thrombosis and stasis in the microcirculation lead to hypoxia
- polycythemia vera or another myeloproliferative disease
- macroglobulinemia, cryoglobulinemia, etc.
- polycythemia vera or another myeloproliferative disease
Overview of hematologic disorders associated with ischemic stroke
Classification according to the etiology of the hematologic disorder
| Congenital disorders | Acquired disorders |
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Hereditary thrombophilia
1. protein S deficiency Hemoglobinopathy
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Classification based on the likelihood of causal association with stroke
| Hematologic disorder | Possible or probable association with stroke | Association uncertain |
| Hereditary coagulopathies (coagulation-inhibiting factor deficiency) |
antithrombin III deficiency protein C deficiency APC resistance (Leiden) |
protein S deficiency heparin cofactor II deficiency factor II (prothrombin) mutation |
| Hereditary fibrinolysis disorders | dysfibrinogenemia hyperfibrinogenemia |
plasminogen deficiency plasminogen activator deficiency factor XII deficiency prekallikrein deficiency excess of PAI-1 |
| Increased concentrations of coagulation factors | factor IX factor XI Thrombin Activatable Fibrinolysis Inhibitor (TAFI) |
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| Autoimmune diseases |
antiphospholipid syndrome (APS) |
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| Erythrocyte disorders | polycythemia vera sickle cell disease (hemoglobinopathy) |
secondary polycythemia paroxysmal nocturnal hemoglobinuria thalassemia |
| Platelet disorder |
essential thrombocythemia thrombocythemia in myeloproliferative diseases |
secondary thrombocythemia acquired platelet hyperaggregability (“sticky” platelets) |
| Other prothrombotic conditions with uncertain or multifactorial association with the occurrence of ischemic stroke |
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