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.

PFO and attributable risk with RoPE score

                        cryptogenic stroke, CS (n= 3,023)                                           

 

RoPE score          No patients   Prevalence PFO   PFO attributable   

 

0-3                         613               23(19-26)             0                          

4                            511               35(31-39)             38 (25-48)              

5                            516               34 (30-38)            34 (21-45)             

6                            482               47(42-51)             62 (54-68)         

7                            434               54(49-59)             72 (66-76)            

8                            287               67(62-73)             84 (79-87)         

9-10                       180                73(66-79)             88(83-91)         

 

 

                       cryptogenic stroke (CS) with PFO (N=1,324)

                            No CS+PFO    est 2 yr TIA/CVA (Kaplan-Meier) 

 

                                                                                   

0-3                         108                20(12-28)

4                            148                12(6-18)

5                            186                  7 (6-18)

6                            236                  8 (4-12)

7                            263                  6 (2-10)

8                            233                  6 (2-10)

9-10                       150                   2(0-4)

attributable risk PFO based on Bayes' theorem

 

derivation of data used to make RoPE database

RoPE score for PFO

RoPE score for PFO (Risk of Paradoxical Embolus)

Characteristic    Points

No history of
DM                         1
stroke/TIA              1
HTN                       1
nonsmoker             1
cortical infarct        1

Age
18-29                      5
30-39                      4
40-49                      3
50-59                      2
60-69                      1

total score  -- add sum of parts
cut score used in many articles is 7

source Neurology 2013 au Thaler
 

RoPE score, PFO and CS

Thaler DE, Ruthazer R, Weimar C., et al.  Recurrent stroke predictors differ in medically treated patients with pathogenic v. other PFO's.  Neurology 2014; 83: 221-226.

 

The RoPE score, "Risk of Paradoxical Embolism" score estimates the probability that a PFO discovered in a cryptogenic stroke (CS) patient is incidental or pathogenic, based on Bayes theorem.  Patients with high RoPE score (younger, no vascular risk factors, and a superficial infarct) are more likely to have pathogenic PFO's while patients with low RoPE scores (older, vascular risk factors) are probably incidental.  Authors hypothesized that predictors of recurrent stroke should differ among high and low RoPE score patients and PFO characteristics should be les important in low RoPE score individuals. 

 

Risk factors for recurrence with 1-2.2 yrs of followup  included LOW RoPE scores (one year followup, 7 v. 4 %; two year followup 10 v. 5 %) with 4/5 recurrences being in low score subgroup 

 

Patients with TIA had more recurrent events (HR 1.69) but there was no interaction with RoPE score.

 

Variable associated with risk in low RoPE group include older age, those treated with antiplatelet drugs after initial event.

Variables associated with recurrence in highRoPE score group include history of stroke or TIA, hypermobile interatrial septum, and a small shunt, but not shunt at rest. 

 

Comment of blogger

The article confirms / validates the RoPE score to some extent.  However, the point of closure is to prevent lifetime risk of paradoxical embolus, not 2 year risk.  As such, I am skeptical of the claim that risk of recurrence is lower in high RoPE score group

DJ