Bone complications occur frequently in Gaucher disease (GD) and reduce the quality of life of these patients. Skeletal involvement is an important indication for treatment to ameliorate symptoms and reduce the risk of irreversible and debilitating disease. Bone biomarkers have been used to assess disease status and the response to therapy in a number of bone disorders. Here, we examine the literature for evidence of abnormalities in bone turnover markers in patients with type 1 GD to assess whether they might be useful for the assessment of bone involvement in GD. We have found that bone biomarkers in GD show highly variable results which do not currently support their routine use for clinical assessment of bone status, as an indication for therapy initiation, or for monitoring the response to therapy. A greater understanding of bone markers and their relation to the bone manifestations of GD is required.

Background: Splenectomy is sometimes necessary after abdominal trauma, but splenectomized patients are at risk of sepsis due to impaired immunological functions. To overcome this risk, autotransplantation of the spleen by using a new technique has been proposed, but so far, a demonstration of functionality of the transplanted tissue is lacking.Methods: We therefore evaluated 5 patients who underwent a splenic autotransplant in comparison with 5 splenectomized patients without splenic autotransplant and 7 normal subjects.Results: We confirmed that the patients not undergoing autotransplantation, when compared to normal subjects, had a higher platelet count, higher percentage of micronucleated reticulocytes (p = 0.002), increased levels of naive B lymphocytes (p = 0.01), a defect of class-switched memory (p = 0.001) and class-unswitched memory B cells (p = 0.002), and increased levels of PD1 on T lymphocytes CD8+ (p = 0.08). In contrast, no significant differences for any of the abovementioned parameters were recorded between patients who underwent spleen autotransplantation and normal subjects.Conclusion: These findings suggest that splenic autotransplantation is able to restore an adequate hemocatheretic activity as well as recover the immunological deficit after splenectomy.

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B-cell chronic lymphocytic leukemia (B-CLL) is a heterogeneous hematologic malignancy caused by a clonally expanded B-cell population that recirculates between the blood and tissues. Described is the case of an 81-year-old female B-CLL patient with aberrant expression of a T-cell-associated antigen, CD8, on CD19+ B-CLL cells. Despite chlorambucil therapy and a subsequent salvage regimen, the patient rapidly entered progressive disease and died 9 months later. Flow cytometric immunophenotyping of the B-CLL cells for a number of antigens ruled out a mixed-lineage disorder. The implications of CD8 expression in B-CLL are discussed, and its expression in this case probably reflects an aberrant lineage infidelity. CD8 expression might be considered as a marker of aggressive disease.

4890 Background Myeloid-derived suppressor cells (MDSC) are a heterogeneous population of cells of myeloid origin, and include immature macrophages, granulocytes, dendritic cells (DC) and other myeloid cells. In mice, are phenotypically characterized as CD11b+Gr-1+. Humans MDSC have an immature phenotype, including lineage negative (Lin-), CD14-, HLA-DR-, CD15+, CD34+, CD11b+, CD33+, and CD13+ cells. MDSC impair T-cell functions through secretion immunosuppressive cytokines, perturbation of the arginine metabolism by inducible nitric oxide synthase (iNOS), up-regulation of reactive oxygen species (ROS), therefore are considered an important tumor escape mechanism. These cells, also promote tumor-dependent angiogenesis as well as tumor metastasis, and to provide tumor resistance to antiangiogenic drugs. Their accumulation has been described in the peripheral blood of patients affected by breast, lung, renal and neck carcinoma, melanoma, chronic infections, inflammatory diseases, and traumatic stress. We investigate MSDC in patients with multiple myeloma (MM) and monoclonal gammopathy undetermined significance (MGUS) by flow cytometry Methods We studied 22 patients with MM at diagnosis, 20 patients with MGUS, and 10 healthy controls (HC). Results we observed that patients with MGUS showed the same number of MDSC (CD11b+,CD13+,CD34+,CD14-,CD45+) in the peripheral blood compared to HC (1,90±1,03/mmc, p=0,23). On the contrary, patients affected by MM showed a significant increase of MDSC vs HC (6,80±8,79/mmc vs 1,45± 0,98/mmc, p=0,003). Conclusion Our results suggest that these myeloid-derived suppressor cells, through their mechanisms immunesuppressive and proangiogenesis, could be involved in the progression of MGUS towards overt MM. Disclosures No relevant conflicts of interest to declare.

Background: PET-CTs scans are commonly used in haematological malignancies to stage disease and assess response to therapy. It is not uncommon for PET-CT to reveal incidental findings, the significance of which is unclear. Aims: This study aimed to determine how common incidental findings are in patients with haematological malignancies, and assess the outcome of the investigations into these findings. Methods: Reports from 212 patients who had a PET-CT scan reviewed at the departmental haematology-radiology meeting of the Beatson Oncology Centre, Glasgow, Scotland between May 2015 and May 2018 were reviewed retrospectively. All scans were undertaken at the same site, the West of Scotland PET Centre. For each incidental finding, follow up, diagnosis and outcome was then reviewed. 57% (n = 121) of patients were male and the median age was 57 years. The majority of patients (93%, n = 196) underwent the PET-CT scan due to underlying lymphoma, 122 (43%) of these were Non-Hodgkin and 74 (35%) with Hodgkin lymphoma,16 of the patients had underlying myeloma or other suspected malignancies.Results: The study found that 13% (n = 28) of patients that underwent PET-CT scanning had an incidental finding. The majority of these findings (82%, n = 23) were investigated further. No significant abnormal finding was revealed through further investigation for 43% (n = 10). A pathological finding was found in 57% (n = 13) of scans. Of these 69% (n = 9) had a pre-malignant or malignant diagnosis: 2 adenocarcinomas and 4 precancerous polyps in the colon and 3 Thy3a in the thyroid. The remaining 4 were benign pathologies including gastric reflux, benign adenomas and a fistula. The incidental findings were in a variety of anatomical sites, the most common being the colon (n = 15 findings), followed by the thyroid (n = 4). Regarding outcome patients with pre-malignant polyps and adenocarcinomas underwent complete surgical excision, with no disease remaining post operatively. No patient had evidence of progression / relapse of the incidental finding at time of follow up. Summary/Conclusion: Incidental findings on PET-CT were shown to occur commonly in patients with haematological malignancies. Follow up of these incidental findings is indicated, and appropriate, as it often results in the diagnosis of a treatable, pathological condition. Further investigation in patients with haematological malignancy is particularly important in view of the potential high survival rate from the underlying haematological cancer.

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