Indian Journal of Nephrology最新文献

Posttransplant thrombotic microangiopathy (PT-TMA) can be caused by calcineurin inhibitors (CNIs), ischemic injury, infections, or antibody-mediated rejection (ABMR). Delayed recognition can result in allograft loss. We describe the first reported case of successful reversal of refractory PT-TMA with eculizumab in India. It highlights the importance of prompt diagnosis and benefit from an early initiation of eculizumab therapy in refractory cases.

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition due to extensive and uncontrolled immune activation. There is sparse literature on HLH in kidney transplant recipients. We report a case of a 27-year -old male kidney transplant recipient who presented with dengue fever and acute allograft dysfunction. Following improvement in allograft function with supportive treatment, he was found to have worsening pancytopenia with unusually high serum ferritin levels. Bone marrow aspiration performed for pancytopenia revealed hemophagocytosis. A diagnosis of HLH secondary to dengue viral infection was made based on the modified HLH diagnostic criteria (2009). He received supportive treatment and steroids and was discharged in a stable condition with normal kidney allograft functions. To our knowledge, this is the first case report of HLH secondary to dengue viral infection in a kidney allograft recipient managed successfully with timely diagnosis and appropriate treatment.

Proliferative glomerulonephritis in myelofibrosis is a very rare. Mesangial proliferation and sclerosis with changes of chronic thrombotic microangiopathy have been reported, but pauci-immune focal crescentic glomerulonephritis has not been described so far. Herein, we present a 68-year-old male who was a known case of myelofibrosis and presented with rapidly progressive glomerulonephritis and nephrotic range proteinuria. He was diagnosed as anti-neutrophil cytoplasmic antibody (ANCA)-negative focal crescentic glomerulonephritis, and he responded well to a course of intravenous methylprednisolone and cyclophosphamide. Pauci-immune focal crescentic glomerulonephritis may occur in myelofibrosis without ANCA and may be related to unknown pathogenetic mechanisms in myeloproliferative disorders or suggest any superimposed pathology that might respond well to immunosuppressants.

Collapsing focal segmental glomerulosclerosis (FSGS) a heterogeneous group of disorders, rather than a single disease entity. Kidney biopsy shows segmental or globally collapsed, sclerotic glomerular capillaries. There is also hypertrophy and hyperplasia of overlying glomerular epithelial cells. Immuno-fluorescence is negative or has non-specific deposits of immunoglobulins and C3. We present two cases of C3 dominant collapsing FSGS. Both the cases were non-responsive to therapy and had a poor outcome. This calls for research to study the role of the complement pathway in the pathogenesis of FSGS.

Gemcitabine-induced thrombotic micro-angiopathy (GiTMA) is a very rare pathology of micro-vascular occlusion with a poor prognosis. In this case report, we present a young male with pancreatic carcinoma who received gemcitabine as adjuvant chemotherapy and developed thrombotic micro-angiopathy (TMA) manifesting as nephrotic syndrome with renal dysfunction and posterior reversible encephalopathy syndrome (PRES). The case was successfully managed with discontinuation of the drug and conservative management. The pathogenesis of GiTMA might be direct endothelial dysfunction with consequent activation of the clotting system. The role of plasma exchanges and monoclonal antibodies is unclear in drug-induced TMA.