Hematologic and renal improvement of monoclonal immunoglobulin deposition disease after treatment with bortezomib-based regimens

Monoclonal immunoglobulin deposition disease (MIDD) is characterized by non-organized immunoglobulin-fragments along renal basement membranes with subsequent organ deterioration. Treatment is directed against the immunoglobulin-producing clone. We treated 18 MIDD patients with bortezomib-based regimens (12 received bortezomib-dexamethasone, 6 bortezomib-dexamethasone with cyclophosphamide). Eleven (61%) patients achieved a hematologic response, but only 6 (33.3%) reached to a complete (CR) or very good partial response (VGPR). Regarding renal outcomes 77.8 and 55.6% had ≥30 and ≥50% reduction of proteinuria, respectively, but 33.3% ended up in end-stage renal disease (ESRD). Among patients with CR or VGPR, median eGFR improvement was 7.7 ml/min/1.73 m2 and none progressed to ESRD, but no significant renal recovery was observed in patients achieving a partial response or less, with 50% progressing to dialysis. Pretreatment eGFR seems to influence renal prognosis. Bortezomib-based treatment is considered an effective approach in MIDD and reaching to a deep hematologic response (≥VGPR) conditionally controls further renal declining. © 2016 Informa UK Limited, trading as Taylor & Francis Group

Bone Architecture: Collagen Structure and Calcium/Phosphorus Maps

Bone collagen structure in normal and pathological tissues is presented using techniques of thin section transmission electron microscopy and morphometry. In pathological tissue, deviations from normal fine structure are reflected in abnormal arrangements of collagen fibrils and abnormalities in fibril diameter. The relationships between these bone structural changes and the skeletal calcium/phosphorus ratio are discussed. Calcium/phosphorus ratio is measured by X-ray absorptiometry and computed microtomography