Hematologic disorders associated with ischemic stroke

David Goldemund M.D.
Updated on 05/05/2024, published on 02/05/2023
  • 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 relationship between coagulation abnormalities and arterial stroke is not always clear
  • if hematologic disorders are found to be responsible for an ischemic stroke, it is classified as TOAST 4


Main mechanisms by which hematologic diseases cause stroke

  • increased coagulation or aggregation with thrombus formation intravascularly or in cardiac cavities with subsequent embolization (typically seen in inherited coagulation disorders)
  • hyperviscosity syndrome (corpuscular or noncorpuscular), in which thrombosis and stasis in the microcirculation lead to hypoxia
    • polycythemia vera or other myeloproliferative diseases
    • macroglobulinemia, cryoglobulinemia, etc.

Overview of hematologic disorders associated with ischemic stroke

Classification according to the etiology of the hematologic disorder

Congenital disorders  Acquired disorders
 Hereditary thrombophilia

1. protein S deficiency
2. protein C deficiency
3. APC resistance (Leiden mutation – factor V)

4. antithrombin III deficiency

5,6. dysfibrinogenemia/hyperfibrinogenemia
7. tPA deficiency
8.  PAI-1
9.  prothrombin gene mutation G20210A  (increased prothrombin production)

10. thrombomodulin deficiency
11. plasminogen deficiency


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
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)
Autoimmune diseases
antiphospholipid syndrome (APS)
Erythrocyte disorders polycythemia vera
sickle cell disease  (hemoglobinopathy)
secondary polycythemia
paroxysmal nocturnal hemoglobinuria
Platelet disorders
essential thrombocythemia
thrombocythemia in myeloproliferative diseases
Thrombotic thrombocytopenic purpura (TTP)
secondary thrombocythemia
acquired platelet hyperaggregability (“sticky” platelets)
Other prothrombotic conditions with uncertain or multifactorial association with the occurrence of ischemic stroke
  • pregnancy, puerperium
  • oral contraceptives (OC) and hormone replacement therapy (HRT) → more
  • nephrotic syndrome
  • malignancy, paraneoplastic syndromes → Cancer-related stroke
  • marantic endocarditis
  • chemotherapy-induced thrombosis
  • thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS)
  • DIC
  • macroglobulinemia, cryoglobulinemia
  • antifibrinolytic therapy
  • HIT (Heparin-induced thrombocytopenia with thrombosis) → see here
  • infections (e.g., COVID-19)

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Hematologic disorders associated with ischemic stroke