Pearls on strokes associated with hematologic diseases

This is based on an OLD article by Martin Samuels, so newer drugs for condition are not included

Samuels MA, Thalinger K.  Cerebrovascular manifestations of selected hematologic diseases.  Seminars in Neurology 11:4 1991.

1.  Anemia-- examine the EYE.  First sign is pallor, then spindle shaped retinal hemorrages with cotton wool spots.

2.  Blood transfusions can trigger stroke in patients with Beta thallasemia (cites Logothetis J. et al. Neurology 1972;22:294-304.

3. In thrombocytopenia, intracranial hemorrhages occurs as small ring shaped hemorrhages in the gray and white matter due to capillary bleeding.Subdural, subarachnoid and epidural hemorrhages are rare in this condition.  Peripheral nerve and spinal cord hemorrhage also are rare.

4. In thrombotic thrombocytic purpura (TTP), consists of triad of thrombotic purpura, hemolytic anemia and neurologic manifestations.  Fever and renal disease are "invariably" present.  Diagnosis depends on tissue of skin, lymph node, bone marrow or spleen that show hyalinization of arterioles and platelet thrombi with small foci of parenchymal necrosis and petecchiae."Gray matter" symptoms include headache, confusion, aphasia, hemiparesis,visual changes, dysarthria, seizures, coma, vertigo. Exchange transfusion helps dramatically. Other treatments are heparin, steroids, splenectomy.

5.  In hemolytic uremic syndrome, similar to TTP, caused by immune deposits, exchange transfusion can result in dramatic improvement.  FFP without albumen also helps.

6.  Henoch-Schoenlein purpura (anaphylactoid purpura) is characterized by serosanguineous effusions into subcutaneous, submucous, and subserous tissues, esp in young adults.  Treatment is supportive.  ICH and SAH occur rarely.IgA mediated, also get renal deposits

7.  Ischemic strokes occur in 15-32 percent of patients with polycythemia vera.  The annual incidence of TIA/ stroke is 4-5 percent even in those treated with phlebotomy.  Conversely, risk of stroke is rare in secondary polycythmia.  For example, someone with congenitally cyanotic heart disease and Hct of 60 has very low risk, none occurred in a small series cited with more than 200 patient years of followup.

8.  Hyperviscosity syndrome is a major cause of stroke in patients with myeloma.  Clinical presentation is stupor, coma, drowsiness, inattention,delirium.  Fundoscopic changes include "sausage veins,"  retinal hemorrhages and exudates.  Focal events can occur in Waldenstrom's macroglobulinemia.  Plasma exchange can dramatically improve symptoms due to heavy proteins.