Tc-99m sestamibi bone marrow scintigraphy in Gaucher disease.
OBJECTIVE
No imaging technique has been found to be adequate to assess the severity and extent of bone involvement in patients with Gaucher disease. Marrow involvement, as determined by Tc-99m sulfur colloid, correlated well with the clinical and radiologic changes of the skeleton, but a normal pattern was found in the early stages of the disease. Subsequently, Tc-99m sestamibi (MIBI) has been suggested for direct visualization of glycolipid deposits in the bone marrow. This study was initiated as a pilot using MIBI to detect various forms of bone disease in patients with Gaucher disease of varying severity.


METHODS
Eleven patients (9 men; median age, 39.9; age range, 21 to 61 years) were evaluated. The clinical severity of disease was scored at presentation, and four patients with moderate to severe disease were treated with enzyme replacement therapy. Each patient underwent a radiographic skeletal survey, bone densitometry, and MIBI scintigraphy. The scan included static images of the lower limbs, with a whole-body scan acquired between the early and late acquisition. Tracer uptake in the bone marrow was graded and correlated with clinical and objective variables.


RESULTS
All but one patient had increased MIBI uptake in the bone marrow. No correlation was noted between MIBI uptake and severity score, radiographic changes, densitometry z score, or treatment status.


CONCLUSIONS
MIBI scanning is a sensitive technique for detecting bone marrow deposits in Gaucher disease, but it is inadequate for early identification of patients at high risk for skeletal complications or for the follow-up of patients treated with enzyme replacement.
Aharoni
Dvora
D
Gaucher Clinic, Shaare Zedek Medical Center, Jerusalem, Israel.
Krausz
Yodphat
Y
Elstein
Deborah
D
Hadas-Halpern
Irith
I
Zimran
Ari
A
D000328:Adult; D015519:Bone Density; D001847:Bone Diseases / Q000000981:diagnostic imaging* / Q000209:etiology / Q000378:metabolism; D001853:Bone Marrow / Q000000981:diagnostic imaging*; D005260:Female; D005776:Gaucher Disease / Q000150:complications / Q000000981:diagnostic imaging*; D006801:Humans; D007867:Leg Bones / Q000000981:diagnostic imaging* / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D011877:Radionuclide Imaging; D019275:Radiopharmaceuticals / Q000493:pharmacokinetics; D017256:Technetium Tc 99m Sestamibi / Q000493:pharmacokinetics; D014915:Whole-Body Counting
D019275:Radiopharmaceuticals; D017256:Technetium Tc 99m Sestamibi