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154. Treatment‐free remission after imatinib discontinuation is possible in paediatric patients with chronic myeloid leukaemia

Author

Giona, Fiorina, primary, Saglio, Giuseppe, additional, Moleti, Maria Luisa, additional, Piciocchi, Alfonso, additional, Rea, Massimiliano, additional, Nanni, Mauro, additional, Marzella, Deborah, additional, Testi, Anna Maria, additional, Mariani, Sabrina, additional, Laurino, Marica, additional, Diverio, Daniela, additional, Gottardi, Enrico, additional, and Foà, Robin, additional

Published
2014
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157. Severity of bone marrow involvement in patients with Gaucher's disease evaluated by scintigraphy with 99mTc-sestamibi

Author

Mariani, Giuliano, Filocamo, Mirella, Giona, Fiorina, Villa, Giuseppe, Amendola, Angela, Erba, Paola, Buffoni, Ferdinando, Copello, Francesco, Pierini, Anna, Minichilli, Fabrizio, Gatti, Rosanna, Brady, Roscoe O, Mariani, G, Filocamo, M, Giona, F, Villa, G, Amendola, A, Erba, P, Buffoni, F, Copello, F, Pierini, A, Minichilli, F, Gatti, R, and Brady, R

Subjects
Adult, Male, Technetium Tc 99m Sestamibi, Adolescent, Knee Joint, Sensitivity and Specificity, Severity of Illness Index, Gaucher, SestaMIBI, MR, Bone involvment, ERT, 99mtc-sestamibi, bone marrow involvement, disease severity, gaucher's disease, scintigraphic score, Bone Marrow, Humans, Child, Radionuclide Imaging, Aged, Gaucher Disease, Reproducibility of Results, Middle Aged, Child, Preschool, Glucosylceramidase, Female, Radiopharmaceuticals
Abstract

Unlabelled: Gaucher's disease is a lysosomal storage disorder due to a genetically transmitted deficiency of the enzyme glucocerebrosidase. In the most common form of the disease (type 1), accumulation of glucosylceramide in the reticuloendothelial cells of liver, spleen, and bone marrow leads to visceromegaly, anemia, thrombocytopenia, and osteopenia. Skeletal manifestations secondary to infiltration of the bone marrow by Gaucher's cells are detectable by radiography only in advanced stages. Imaging of bone marrow involvement can be performed indirectly by magnetic resonance techniques or by bone marrow scintigraphy with radiocolloids. However, both procedures lack specificity because the normal bone marrow, rather than the pathologic process, is imaged. The aim of this study was to assess the reliability of (99m)Tc-sestamibi scintigraphy for direct evaluation of bone marrow involvement. Methods: Seventy-two patients with type 1 and 2 patients with type 3 Gaucher's disease (35 males, 39 females) were enrolled in the study. The mean age +/- SD was 31.9 +/- 16.5 y (range, 3-76 y), and the average duration of the disease manifestations when performing scintigraphy was 12.95 y (median, 10.5 y; range, 0-44 y). Forty-three of 74 patients had never received enzyme replacement therapy (ERT), whereas 31 patients were already being treated with ERT. (99m)Tc-Sestamibi was injected intravenously (6-8 MBq/kg of body weight) and imaging was recorded at the lower limbs 30 min after injection, at the plateau of tracer accumulation in the involved bone marrow. The scans were evaluated visually, assigning a semiquantitative score based on the extension and intensity of uptake in the bone marrow of the lower limbs (0 = no uptake; 8 = maximum uptake). The scintigraphic score was entered into complex statistical analysis, which included a series of clinical and blood chemistry parameters defining overall severity of the disease. Results: (99m)Tc-Sestamibi scintigraphy showed that 71 of 74 patients had some degree of bone marrow involvement. The scintigraphic score was highly correlated with an overall clinical severity score index (SSI) and with various parameters contributing to the SSI, either positively or negatively. The highest correlation of the scintigraphic score was found with an overall biochemical marker of disease severity (serum chitotriosidase). ERT-naive patients showed high correlation of the scintigraphic score with the clinical SSI, with a radiographically based score, and with serum chitotriosidase. In the ERT-treated patients, the scintigraphic score was correlated with the clinical SSI, with hepatomegaly, and with hemoglobin. Conclusion: (99m)Tc-Sestamibi uptake reliably identifies bone marrow infiltration by Gaucher's cells. The scintigraphic score is helpful for defining the severity of bone marrow involvement and for comparing patients. (99m)Tc-Sestamibi scintigraphy, which provides topographic information about the sites involved by the disease, is highly correlated with other parameters of disease severity and appears to correlate with response to ERT.

Published
2003

162. 99mTc-sestamibi scintigraphy to monitor the long-term efficacy of enzyme replacement therapy on bone marrow infiltration in patients with Gaucher disease

Author

Erba, P, Minichilli, F, Giona, F, Linari, S, Dambrosia, J, Pierini, A, Filocamo, M, Di Rocco, M, Buffoni, F, Brady, R, Mariani, G, Erba, Paola A, Minichilli, Fabrizio, Giona, Fiorina, Linari, Silvia, Dambrosia, James, Pierini, Anna, Filocamo, Mirella, Di Rocco, Maja, Buffoni, Ferdinando, Brady, Roscoe O, Mariani, Giuliano, Erba, P, Minichilli, F, Giona, F, Linari, S, Dambrosia, J, Pierini, A, Filocamo, M, Di Rocco, M, Buffoni, F, Brady, R, Mariani, G, Erba, Paola A, Minichilli, Fabrizio, Giona, Fiorina, Linari, Silvia, Dambrosia, James, Pierini, Anna, Filocamo, Mirella, Di Rocco, Maja, Buffoni, Ferdinando, Brady, Roscoe O, and Mariani, Giuliano

Abstract

Assessing the skeletal response to enzyme replacement therapy (ERT) in Gaucher disease (GD) is problematic. We investigated the reliability of 99mTc-sestamibi scintigraphy in monitoring changes in bone marrow involvement induced by ERT. Methods: In 52 GD patients, the efficacy of ERT on bone marrow disease was monitored using at least 2 sequential 99mTc-sestamibi scans; 17 patients were receiving ERT at enrollment, and 35 were ERT-naïve. We elaborated a dose-response model by statistical analysis based on linear mixed models. Results: Patients whose marrow disease improved had received a significantly higher ERT dose per month than patients who did not improve. Significantly more patients reached near-disappearance of marrow disease if their disease burden at enrollment had been lower and the duration of clinical signs shorter. The response of the marrow scintigraphic score wasmore pronounced in ERT-naïve patients. No relevant effect of ERT on marrow disease was observed until platelet count and splenomegaly had improved. Conclusion: Although based on localized evaluation, changes in the 99mTc-sestamibi score closely correlated with the main determinants of ERT, with a definite dose-response relationship. The threshold at which ERT induced any improvement in bone marrow disease was 35-36 U/kg/mo; in ERT-naïve patients, the scintigraphic score declined by 1 unit after ERT at 28 U/kg/mo.

Published
2013

163. Can Children and Adolescents With Chronic Myelogenous Leukemia Be Cured Without Stem Cell Transplant? A Single Center Experience

Author

Giona, Fiorina, primary, Siniscalchi, Benedetta, additional, Moleti, Maria Luisa, additional, Rea, Massimiliano, additional, Marzella, Deborah, additional, Nanni, Mauro, additional, Gottardi, Enrico, additional, Iori, Anna Paola, additional, Diverio, Daniela, additional, Testi, Anna Maria, additional, Saglio, Giuseppe, additional, and Foa', Robin, additional

Published
2013
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164. High Dose Imatinib Is Effective In Children and Adolescents With Chronic Myelogenous Leukemia In Chronic Phase. The Italian Experience

Author

Giona, Fiorina, primary, Putti, Maria Caterina, additional, Menna, Giuseppe, additional, Micalizzi, Concetta, additional, Santoro, Nicola, additional, Iaria, Grazia, additional, Ladogana, Saverio, additional, Burnelli, Roberta, additional, Consarino, Caterina, additional, Siniscalchi, Benedetta, additional, Rea, Massimiliano, additional, Marzella, Deborah, additional, Tucci, Francesca, additional, Nanni, Mauro, additional, Messina, Chiara, additional, Varotto, Stefania, additional, Diverio, Daniela, additional, Biondi, Andrea, additional, Pession, Andrea, additional, Zecca, Marco, additional, Locatelli, Franco, additional, Saglio, Giuseppe, additional, and Foa', Robin, additional

Published
2013
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