The Louisville Team wishes to share with the members of the ASRT what appears to be a post transplant malignancy in their third hand transplant patient.
In October of this year, this patient, who is two years post transplant, presented with abnormally high lymphocyte counts on a routine blood sample. Further analysis revealed a monoclonal B cell clone, in addition to a separate, monoclonal T cell clone. The initial diagnosis was PTLD, and Cellcept was discontinued and the patient was placed on low dose Prograf monotherapy.
Follow up evaluation revealed unique features such as a lack of symptoms, complete absence of nodal or extranodal involvement with the disease confined to the bone marrow and the peripheral blood. The patient is EBV-, and the cells are EBER negative. Bone marrow aspirate showed the same clones, which occupied less than 5% of marrow cellularity. The B cell line is a low grade lymphoma, likely of marginal zone origin The T cell line is CD8+ with restricted V beta usage. The reduction in immunosuppression has not had an effect on the absolute number of either the T or B cell clone in the last 8 weeks.
The team has consulted a number of experts in lymphoma and PTLD at other centers, including the NIH. Phenotypically the B cell is most like a marginal zone lymphoma, which by WHO definition, is not PTLD. Reduction in immunosuppression has not affected the absolute numbers of the clones, consistent with the proposition that these clones are not related to the immunosuppression. As such, there is a high probability that these clones are not PTLD. However, because of the temporal relationship to the transplant, we cannot completely exclude the possibility.
After extensive discussions with the patient regarding potential treatment options including amputation of the hand, the patient has chosen, and we concur with the strategy of managing rejection and maintaining the allograft, with close monitoring of the lymphoma. At this point additional time is needed to resolve the differential diagnosis.