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Localized lymphoid relapse in the pancreas following allogeneic hematopoietic stem cell transplant for chronic
J M Rossetti1, J Lister, R K Shadduck
1Western Pennsylvania Cancer Institute, The Western Pennsylvania Hospital, 4800 Friendship Avenue, Suite 2303, Pittsburgh, PA 15224, USA. jrossett@wpahs.org
The incidence of isolated extramedullary disease (EMD) following allogeneic hematopoietic stem cell transplant (allo-HSCT) for chronic myelogenous leukemia (CML) is not fully known. One review found the incidence of isolated myeloid EMD, or granulocytic sarcoma (GS), in an allo-HSCT treated CML/myelodysplastic subgroup to be just 0.22%. The incidence of lymphoid EMD in similar patients is extremely rare with only two cases reported in the literature. While the etiology of EMD in the post-transplant setting is not entirely clear, there may be inefficacy of immune surveillance function outside of the bone marrow cavity. Isolated CML GS following allo-HSCT carries a median interval to bone marrow relapse between 7 and 10 months and a median survival of 12 months. Less is known about lymphoid EMD. The treatment in these cases is ill defined with modalities ranging from involved field radiation to second allo-HSCT. We present a case of isolated pancreatic lymphoid EMD diagnosed 15 months after allo-HSCT for CML. Our patient was also treated with withdrawal of his immunosuppressive regimen. Unfortunately, at just over 4 months following pancreatic resection, he developed systemic relapse and died. While EMD can occur anywhere in the body, CML associated pancreatic EMD is not previously reported.
The incidence of isolated extramedullary disease (EMD) following allogeneic hematopoietic stem cell transplant (allo-HSCT) for chronic myelogenous leukemia (CML) is not fully known. One review found the incidence of isolated myeloid EMD, or granulocytic sarcoma (GS), in an allo-HSCT treated CML/myelodysplastic subgroup to be just 0.22%. The incidence of lymphoid EMD in similar patients is extremely rare with only two cases reported in the literature. While the etiology of EMD in the post-transplant setting is not entirely clear, there may be inefficacy of immune surveillance function outside of the bone marrow cavity. Isolated CML GS following allo-HSCT carries a median interval to bone marrow relapse between 7 and 10 months and a median survival of 12 months. Less is known about lymphoid EMD. The treatment in these cases is ill defined with modalities ranging from involved field radiation to second allo-HSCT. We present a case of isolated pancreatic lymphoid EMD diagnosed 15 months after allo-HSCT for CML. Our patient was also treated with withdrawal of his immunosuppressive regimen. Unfortunately, at just over 4 months following pancreatic resection, he developed systemic relapse and died. While EMD can occur anywhere in the body, CML associated pancreatic EMD is not previously reported.
