Acute skin GVHD following syngeneic BMT for CLL

Abstract

Summary:A 41-year-old woman received a syngeneic BMT for CLL and subsequently developed acute skin GVHD. Transfusion-related allogeneic GVHD was excluded on the basis of an unchanged HLA type in circulating lymphocytes. Short tandem repeat PCR was used to confirm syngeneicity between donor and recipient. The patient had a personal and family history of autoimmune disease which may have made her particularly susceptible to development of syngeneic GVHD. The distinction between allogeneic and syngeneic or autologous GVHD is important because of therapeutic implications. Keywords: GVHD; syngeneic BMT; CLL; chimerism We report the case of a 41-year-old woman who received a syngeneic BMT for CLL and subsequently developed histologically proven acute grade 2 skin GVHD requiring treatment, including high-dose corticosteroids. The HLA type of circulating lymphocytes remained unchanged. Short tandem repeat (STR)-PCR analysis of pre-BMT DNA extracted from donor and recipient indicated identity between donor and recipient supporting the diagnosis of syngeneic GVHD. Syngeneic and autologous GVHD has been described in animal models. 1,2 Mild and self-limiting acute skin GVHD is thought to occur in 8% of patients receiving autologous and syngeneic BMT. 3 Case reportThe patient presented with stage B CLL and Sjogren's syndrome in 1992. There was a strong family history of autoimmune disease. The patient, her mother and identical twin sister all had hypothyroidism and the patient's mother also had SLE. The patient required intermittent treatment for CLL but, by 1994, her disease had advanced to cause bone marrow failure. She showed a good response to six courses of fludarabine with a reduction in bone marrow lymphocytic infiltration to 40%. She subsequently received a syngeneic BMT following CY 120 mg/kg and single fraction TBI (750 cGy at 16 cGy/min) conditioning. Both recipent and donor were CMV seronegative. At the time of transplant, the patient had no evidence of active autoimmune disease. She received irradiated blood products throughout. The transplant was uncomplicated with haemopoietic recovery (Ͼ0.5 × 10 9 /l neutrophils, Ͼ20 × 10 9 /l platelets) at day 25. Over the next week she developed a generalised skin rash which progressed to desquamation. She was systemically well with no evidence of infection. She had not been commenced on any new drugs in the preceding 3 weeks. A skin biopsy showed the appearances of grade II GVHD with basal cell degeneration and a focal lymphocytic infiltration of the epidermis (Figure 1). Although the patient had been designated to receive irradiated blood products, and showed no deterioration in bone marrow function, we felt that transfusion acquired GVHD should be excluded because of therapeutic implications. The HLA type of the circulating lymphocytes was determined and was unchanged. Pre-BMT DNA extracted from the recipient and donor was compared using a panel of STR markers as previously described. 4 Seven markers were tested (see

A case of syngeneic graft-versus-host disease

“…Of note, all but one of these cases failed to report or, similar to our case, did not provide GVHD prophylaxis to patients undergoing sHSCT. 1,4,5,[9][10][11][12][13] The cases used traditionally allogeneic conditioning regimens, while we used BEAM, a regimen widely used to condition autologous transplant, due to its anti-lymphoma efficacy. Type of conditioning regimen that is most appropriate in sHSCT remains unanswered.…”

“…GVHD was not the cause of death in any of the patients reported. 1,4,5,[9][10][11][12][13] We initiated both steroids and tacrolimus in our patient upon GVHD presentation due to the concerning manifestation of symptoms and lack of strong data with treating sGVHD.…”

Syngeneic Acute Graft-versus-Host Disease

“…Sir, Graft-versus-host disease (GVHD) can be seen in recipients of syngeneic or autologous bone marrow transplantation (BMT) (1,2) and occurs in 8% of patients given this type of BMT (3,4). Most cases are self-limiting, have a good prognosis and involve mainly the skin.…”

Syngeneic graft-versus-host disease: a report of two cases and literature review

“…Reports of severe GVHD development after syngeneic bone marrow transplant are becoming more frequent. [8][9][10][11] We also report two cases of severe syngeneic graft-versus-host disease (SGVHD) requiring high-dose steroids and immunosuppressive therapy.…”