Extracorporeal photochemotherapy (ECP; photopheresis), an immunomodulatory therapy developed for cutaneous T-cell lymphoma, has shown promise in treating chronic graft-versus-host disease (cGvHD) in uncontrolled studies. The purpose of this study was to further examine the effects of ECP on cGvHD. ECP (administered initially 3 times weekly on alternating days) was retrospectively evaluated in 14 patients with extensive cGvHD following allogeneic hematopoietic stem cell transplantation. The median time from transplantation to ECP initiation was 29 months (range, 5-96 months). The median number of concomitant baseline treatments per patient was 3 (range, 0-5). During a median ECP duration of 17 months (range, 3-44 months), 3 patients (21%) achieved a complete cutaneous response (100% improvement), 4 patients (29%) achieved a partial cutaneous response (> or =50% improvement), and 7 patients (50%) had stable skin disease. The median time to response was 6 months (range, 2-15 months), and the median response duration was 5 months (range, 1-31 months). At endpoint, responses were ongoing in 4 patients. Resolution or improvement was noted in arthralgia (5/7 patients), oral changes (3/7), elevated liver enzymes (3/5), dry eyes (2/5), joint stiffness (3/3), pulmonary disease (1/3), and thrombocytopenia (1/1). Because of a favorable response, 11 of 13 patients (85%) who received prednisone at baseline were able to taper (7/13; 54%) or discontinue (4/13; 31%) this medication, and 12 of 14 patients (86%) were able to taper (11/14; 79%) or discontinue (1/14; 7%) ECP. Five-year posttransplantation survival was 77%. Our results suggest that adjunctive ECP improves cutaneous and extracutaneous manifestations of cGvHD and has a steroid-sparing effect.

Summary:Extracorporeal photochemotherapy (ECP; photopheresis), an immunomodulatory therapy, has previously demonstrated promising results in treating chronic graftversus-host disease (cGvHD). We treated six patients (ages 33-54 years) with long-standing refractory extensive-stage cGvHD. ECP was performed thrice weekly initially in all patients. Concomitant therapies included prednisone (n ¼ 6), tacrolimus (n ¼ 5), cyclosporin A (n ¼ 2), hydroxychloroquine (n ¼ 2), mycophenolate mofetil (n ¼ 1), and psoralen plus ultraviolet A radiation (n ¼ 1). After an average of 7.2 months (range, 2-13 months) of ECP, all patients experienced either improvement or stabilization in sclerodermatous skin changes, as well as partial improvements in liver enzyme levels. Skin softening occurred in four patients and was noted as early as 3-8 weeks into treatment. Two patients were able to taper steroid therapy, and two patients were able to taper ECP to twice weekly. ECP was well tolerated. Our results support those of previous studies, suggesting that ECP may be beneficial in patients with refractory cGvHD.

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The use of immunosuppressive therapy has markedly increased over the past several years, and concomitant with its use has been an increased frequency of associated neoplasia. The patient presented is a 22-year-old white male who, following two renal transplants and prolonged immunosuppressive therapy with azathioprine and methylprednisolone, developed chronic granulocytic leukemia. Chromosome karyotyping demonstrated the somewhat unusual development of a Philadelphia chromosome with translocation to the # 7 of the C group. A review of transplantation centers revealed that five cases of chronic granulocytic leukemia have occurred in a population of 25,000 renal transplant patients, a 5-fold increased incidence over the general population. Possible etiologies that may be responsible for the development of chronic granulocytic leukemia in patients on immunosuppressive therapy are discussed. It is our hope that by the introduction of these reports of chronic granulocytic leukemia into the medical literature, the need for caution in the use of immunosuppressive drugs in nonmalignant disease will again be emphasived. increased. This increase encompasses a wide variety of conditions, including prevention of homograft rejection and the suppression of autoimmune symptoms. Although acute leukemia has been reported in patients on cytotoxic therapy following transplantation, and in patients with myeloma, lo lymphoma, and CLL, l 1 reports of CGL occurring in this setting have not been reported in the literature. We believe the patient described in this report represents the first reported association between a renal transplant recipient on immunosuppressive therapy and chronic granulocytic leukemia with the development of a Philadelphia chromosome. CASE REPORTThe patient, a 22-year-old white male, was admitted 4 years ago (1973) transplant and was treated with azathioprine 125 mg and methylprednisolone 16 mg daily. Two weeks following the transplant, an acute rejection occurred despite irradiation (150 Rad) to the transplant site and increased amounts of immunosuppressive drugs. Hemodialysis was reinstituted. In January 1976, the original rejected graft was removed and he received a second renal transplant from a cadaver donor. Autopsy findings on the 15-year-old cadaver donor showed the cause of death to have been massive head trauma. There was no evidence of any leukemia or other white cell disorders. The patient achieved normal renal function and was discharged on azathioprine 150 mg and methylprednisolone 12 mg daily. H e failed to show any evidence of rejection and he did not receive any anti-lymphocyte globulin as immunosuppressive therapy. The leukocyte count ranged from 17,000 to 23,000 until 9 months later, September, 1976, when increased numbers of bands, eosinophils and basophils were noted in addition to a progressive thrombocytosis. For the next three months, his thrombocytosis was accompanied by a progressive leukocytosis and hyperuricemia while on maintenance azathioprine and methylprednisolone (Table 1). A...

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