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Summary:In this case report we describe a novel treatment with two chimeric monoclonal antibodies (MoAb) targeting the autoimmune B cell clone responsible for bullous pemphigoid (BP) as a manifestation of steroid refractory chronic graft-versus-host disease (GVHD) that developed after unrelated cord blood transplantation. Monitoring the BP-specific circulating antibodies and CD25-expressing activated T lymphocyte subset led us to combine anti-CD20 (Rituximab) mediated B cell ablation with anti-CD25 (Daclizumab) therapy to block CD4 + T cell help. Complete clinical and serologic response was achieved within 4 weeks of initiation of therapy allowing global immunosuppression to be dramatically reduced. Bone Marrow Transplantation (2002) 30, 327-329. doi:10.1038/sj.bmt.1703654 Keywords: transplantation; GVHD; bullous pemphigoid; rituximab; daclizumab A 10-year-old boy was diagnosed with B-precursor ALL at 18 months of age. After 6 months of treatment he had CNS recurrence followed shortly by a bone marrow relapse. He received standard chemotherapy for 3 years with craniospinal irradiation. Four years off therapy he sustained a second isolated bone marrow relapse. During chemotherapy he was noted to have a testicular relapse that was treated with involved-field irradiation and additional chemotherapy. He was in his fourth remission when he arrived at our center.Following total body irradiation (1350 cGy total), 3 days of melphalan (135 mg/m 2 total) and ATG (90 mg/kg total) he was transplanted with an O+, male, 4/6 HLA-matched unrelated umbilical cord blood graft in March 1999 and engrafted with full donor chimerism. The patient and donor were mismatched at one HLA-A and HLA-DRB1 loci, During the first 3 months post transplant the patient experienced pancreatitis, acute renal failure secondary to rhabdomyolysis, and pulmonary hemorrhage. He developed grade 3 acute GVHD of the gut 5 weeks after transplant that was controlled with steroids, FK506, and 10 weeks of anti-CD25 monoclonal antibody (daclizumab). The patient had multiple readmissions for pulmonary edema and respiratory failure secondary to renal insufficiency and hemolytic uremic syndrome that continued to require intermittent hemodialysis. Four months after transplant he was readmitted with hypertensive encephalopathy presumed secondary to FK-506 toxicity. While FK-506 was discontinued he developed GVHD of the skin, controlled by the combination of steroids and the addition of azathioprine and mycophenolate mofetil.Despite continued immunosuppressive therapy with mycophenolate mofetil at 30 mg/kg/day and methylprednisolone at 1 mg/kg/day (Table 1), 7 months after transplant pruritic hemorrhagic bullae developed over his entire body. Skin biopsy of a bulla revealed a subepidermal blister with spongiosis, and hemorrhagic infiltration of lymphocytes, and eosinophils. Direct immunofluorescence of tissue sections revealed linear deposits of IgG and C3 at the basement membrane. Indirect immunofluorescence (IDIF) on 1 m NaCl split skin 1 revealed a linear band of I...

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