Differential diagnosis of oedema

Prof. Paola Romagnani, Dr. Calogero Cirami, Prof. Maurizio Salvadori: A 31-year-old woman came to our observation because of persisting oedema. Three years before, the patient underwent allogenic stem cell transplantation from her HLA identical brother because she suffered from an acute lymphoblastic leukaemia. The leukaemia was FAB L1 peroxidase negative and 60% of the cells in the bone marrow were CD34+, CD13+, CD33+, CD15+, CD4+, HLA-DR+ blasts. Conventional cytogenetic analysis was unsuccessful and Bcr/Abl detection by FISH was negative. After disease remission following one course of induction therapy, the patient completed one cycle of consolidation therapy with cytarabine and mitoxanthrone, although meanwhile she underwent an acute respiratory distress syndrome that required intensive treatment. Cyclosporine A and methotrexate were administered as prophylaxis for graft-versus-host disease (GVHD). The post-transplant period was uneventful. Periodic controls always yielded negative results. As could be expected, the above-mentioned treatments generated an ovarian failure, which manifested through a persisting amenorrhea. Thus, 2 years after allogenic stem cell transplantation, the patient started hormone replacement therapy (HRT) with estradiol valerate (2 mg) and medroxyprogesterone acetate (10 mg). After 2 weeks of HRT, she began to suffer from generalised oedema, mainly periorbital and pretibial, which were more evident in the morning and resulted in weight increase. HRT was stopped, which resulted in an improvement of oedema. After a few days, HRT was administered again, but a significant increase in the oedema occurred. Therefore, HRT was again stopped and treatment with diuretics was introduced. The severity of the oedema worsened, and its preferential localisation to periorbital areas suggested a possible renal origin, even though the laboratory tests performed immediately before beginning the HRT had shown normal serum albumin levels, normal renal function (creatinine 1 mg/dl or 88 μmol/l), and the absence of either proteinuria or alterations of urine sediment.