Fulminant mixed humoral and cellular rejection in a cardiac transplant recipient: a review of the histologic findings and literature.

Mixed acute cellular and humoral rejection is diagnosed uncommonly among heart transplant recipients and usually occurs within the first post-transplant month. We report a case of fatal, fulminant, mixed, acute cellular and humoral rejection in a 33-year-old woman 6 weeks after orthotopic heart transplantation. She had been treated with intravenous methylprednisolone for International Society for Heart and Lung Transplantation (ISHLT) Grade 2 rejection at post-operative Day 28. Despite intensification of immunosuppression therapy, she developed fever and progressive hemodynamic instability. Autopsy results revealed ISHLT Grade 4 mixed cellular and humoral rejection. Cellular rejection is a well-described mechanism of graft failure early after heart transplantation. Although humoral rejection also is recognized as contributing to early graft failure, its characteristics and clinical implications are not as well characterized. We describe a patient with fulminant mixed rejection, despite intensified immunosuppression therapy, early after orthotopic heart transplantation who presented with high-grade fever. We include a review of the literature on humoral and mixed rejections.

Survival and incidence of acute rejection in heart transplant recipients undergoing successful withdrawal from steroid therapy.

BACKGROUND: Steroid-free immunosuppression is feasible in selected patients after heart transplantation. Survival and incidence of acute rejection are important parameters to evaluate when weighing risks and benefits of steroid withdrawal. METHODS: One hundred thirty-seven patients were retrospectively reviewed who underwent heart transplant at Emory University between January 1988 and April 1994 and survived >1 year. Standard immunosuppression (cyclosporine, azathioprine and prednisone) without induction therapy was used. Weaning from steroids was attempted in all patients. Scheduled endomyocardial biopsy was used for long-term surveillance screening. RESULTS: Seventy-two patients (52.5%) underwent successful prednisone withdrawal (Group P0) at an average of 13 months after heart transplant, whereas 65 patients (47.5%) did not achieve steroid-free immunosuppression (Group P1). Group P0 had a mean of 1.3 treated rejection episodes (ISHLT Grade > or = 1b) during the first post-transplant year and Group P1 a mean of 2.3 (p <0.0001). In Group P0, 40 patients (55.6%) suffered a subsequent acute rejection with an ISHLT Grade > or = 1b, resulting in treatment. Of these, 15% were ISHLT Grade 1b, 47.5% Grade 2, 35% Grade 3a and 2.5% Grade 3b. The estimated risk of suffering from acute rejection of at least Grade 1b after achieving steroid-free immunosuppression was 50% at 21 months. Estimated survival at 5 years after heart transplant was 92.9% in Group P0 and 72.3% in Group P1 (p <0.01). Cox proportional hazard modeling revealed black recipient race as effect modifier of group status with decreased survival time in both groups. CONCLUSION: Steroid-free immunosuppression in white heart transplant recipients is associated with improved survival. A low acute rejection score during the first year predicts successful steroid withdrawal. Black recipient race appears to be negatively associated with survival and deserves further detailed study. Long-term surveillance screening using endomyocardial biopsy is recommended.

Takayasu's arteritis mimicking aortic intramural hematoma in a female patient with chest pain.

We report the case of a young female patient who presented with chest pain, having a background of systemic complaints and a history of essential thrombocytosis. Transesophageal echocardiography (TEE) was suggestive of an intramural hematoma of the distal ascending aorta and transverse arch, confirming computed tomography findings. However, surgery revealed only diffuse aortic wall thickening without hematoma or dissection, despite an epiaortic ultrasonogram corroborating the TEE findings. Histopathology demonstrated inflammation involving all layers of the aortic wall, with intimal fibrosis, disrupted elastic fibers in the media, and marked fibrous thickening of the adventitia with multiple necrobiotic foci; the latter were apparently the cause of the markedly hypodense region mimicking intramural hematoma on imaging. The findings were consistent with Takayasu's arteritis, a large vessel vasculitis of unknown etiology that predominantly affects females.