PET 7 year-old female with a left thigh mass. History of cardiac transplantation in infancy.
PTLD Post-Transplant Lymphoproliferative Disorder Associated with Epstein-Barr virus infection Heterogeneous group of diseases after solid organ transplant WHO classification: 1.Early (reactive plasmacytic hyperplasia) 2.Polymorphic PTLD 3.Monomorphic PTLD (lymphomas)
PTLD Post-Transplant Lymphoproliferative Disorder Related to degree and duration of immunosuppression, type of organ transplanted, CMV status Fever, adenopathy Extranodal disease in >2/3 of cases (muscle involvement extremely rare) Excisional biopsy, multiple core needle biopsies, bone marrow biopsy, CT chest/abd/pelvis Rx: Reduction of immunosuppression, antiviral, anti-B-lymphocyte antibodies (rituximab), interferon, chemotherapy Reported rates of cure/survival variable because of heterogeneity of disease
Nephrogenic Systemic Sclerosis (Nephrogenic Fibrosing Dermopathy) Rare multisystemic disorder in patients with renal insufficiency first described in 1997 (200 cases reported to ICNFDR) Unknown etiology IV gadolinium may serve as a trigger in the setting of acute hepatorenal syndrome or dialysis-dependent chronic renal insufficiency Skin induration of extremities and trunk Myalgia, weakness Skin biopsy: proliferation of fibroblasts adjacent to collagen bundles and absence of inflammatory cells
Nephrogenic Systemic Sclerosis (Nephrogenic Fibrosing Dermopathy) Systemic fibrosis with involvement of the skeletal muscle, bone, pleura, pericardium, myocardium, kidney, testes, dura At LLUMC 12 patients 2000-2006 Skin fibrosis and joint contractures within 2- 11 weeks following double-dose iv gadodiamide AJR 2007: 188, February 2007
Neprogenic Systemic Sclerosis Long term outcome: Severe inability to ambulate due to contractures and myopathy, cane required for ambulation, no disability. Skin changes persist. Plasmapheresis may have some effect. Dialysis probably of no help. FDA issued a public health advisory in June 2006 with regards to high-dose gadolinium-containing agents in patients with renal failure At LLUMC: no iv gadodiamide in acute renal failure, hepatorenal syndrome, dialysis patients or ESRD with creatinine clearance of <15 ml/min, no double/triple dose
Neprogenic Systemic Sclerosis Screen patients with serum creatinine and calculated creatinine clearance prior to MRI with contrast if history of kidney disease, diabetes, or >60 y.o