High incidence of silent cerebral infarcts in adult patients with beta thalassemia major.
OBJECTIVE
Survival of beta thalassemia major (TM) patients has improved significantly over the past few decades. Consequently, less commonly reported complications are now being recognized. An incidence as high as 60% of silent cerebral infarcts (SCI) has been demonstrated by brain Magnetic Resonance Imaging (MRI) studies in beta thalassemia intermedia (TI). The aim of this study was to determine whether regularly transfused TM adult patients experience less SCI, as compared to the incidence described in TI.


METHODS
In this observational study, 28 transfusion dependent TM patients, >18years of age underwent brain MRI studies.


RESULTS
Focal bright foci in the cerebral white matter were demonstrated in 17 (60.7%) patients; most of them had multiple lesions. Elevated serum ferritin (SF), primarily 5years Area Under the Curve, was found to have a significant association with the presence of SCI (p<0.031). Similar results were found when 4 patients with intact spleen and 2 patients with splenules were excluded (p=0.027). There was no significant association between number of SCI and clinical or other laboratory parameter evaluated.


CONCLUSIONS
The present study demonstrates a high rate of SCI in regularly transfused TM adult patients. Effective continuous iron chelation, preventive low dose aspirin and routine periodical brain MRI are recommended.
Comprehensive Center of Thalassemia, Hemoglobinopathies & Rare Anemias, Institute of Hematology, Beilinson Hospital, Rabin Medical Center, Petah Tikva, Israel; Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
Idit
I
Pazgal
Section of Neuroradiology, Department of Radiology, Beilinson Hospital, Rabin Medical Center, Petah Tikva, Israel; Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
Edna
E
Inbar
Section of Neuroradiology, Department of Radiology, Beilinson Hospital, Rabin Medical Center, Petah Tikva, Israel; Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
Maya
M
Cohen
Comprehensive Center of Thalassemia, Hemoglobinopathies & Rare Anemias, Institute of Hematology, Beilinson Hospital, Rabin Medical Center, Petah Tikva, Israel; Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
Ofer
O
Shpilberg
Comprehensive Center of Thalassemia, Hemoglobinopathies & Rare Anemias, Institute of Hematology, Beilinson Hospital, Rabin Medical Center, Petah Tikva, Israel; Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel. Electronic address: starkpinhas@gmail.com.
Pinhas
P
Stark
D000328:Adult; D001241:Aspirin / Q000627:therapeutic use; D002544:Cerebral Infarction / Q000097:blood / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000209:etiology*; D054022:Cerebrum / Q000000981:diagnostic imaging* / Q000187:drug effects / Q000473:pathology; D005260:Female; D005293:Ferritins / Q000097:blood; D005343:Fibrinolytic Agents / Q000627:therapeutic use; D006801:Humans; D015994:Incidence; D007502:Iron Chelating Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D065227:Transfusion Reaction; D055815:Young Adult; D017086:beta-Thalassemia / Q000097:blood / Q000150:complications* / Q000628:therapy
D005343:Fibrinolytic Agents; D007502:Iron Chelating Agents; D005293:Ferritins; D001241:Aspirin
Adult patients; Beta thalassemia major; Iron chelation; Silent cerebral infarcts; Transfusion dependent