Successful Rechallenge with Darbepoetin Following Immunosuppressive Therapy in a Dialysis Patient with Erythropoietin-Induced Pure Red Cell Aplasia / 대한내과학회지

Patients with erythropoiesis-stimulating agent (ESA)-induced pure red cell aplasia (PRCA) should not routinely be switched to an alternative ESA or to darbepoetin-alpha because anti-erythropoietin (anti-EPO) antibodies cross-react with all kinds of recombinant ESAs. We present a case of ESA-induced PRCA in a 69-year-old man on hemodialysis whose anemia improved with reintroduction of darbepoetin-alpha following immunosuppressive therapy. The patient developed severe anemia after 15 months of subcutaneous administration of erythropoietin-alpha. After the diagnosis of PRCA, erythropoietin-alpha was discontinued and immunosuppressive therapy with a combination of prednisolone and oral cyclophosphamide was initiated. After 4 months of immunosuppressive therapy, the anti-EPO antibody titer was markedly decreased; however, esophageal candidiasis developed. Additional therapy with cyclosporine alone instead of prednisone and cyclophosphamide was performed, and anti-EPO antibody was subsequently not detected. Darbepoetin-alpha was then reintroduced, and the patient's anemia improved without red cell transfusion. In conclusion, ESA-induced PRCA was successfully treated with reintroduction of darbepoetin-alpha following immunosuppressive therapy.

A Case of P-ANCA-Positive Anti-Glomerular Basement Membrane Antibody Disease / 대한내과학회지

Up to 40% of patients with anti-glomerular basement membrane (GBM) disease, which is a rare autoimmune disorder usually manifesting as rapidly progressive glomerulonephritis (RPGN), are positive for circulating anti-neutrophil cytoplasmic antibody (ANCA). Many previous reports showed poor outcomes in these "double-positive" patients. We report a patient with perinuclear (p)-ANCA positive anti-GBM disease who presented with RPGN and required hemodialysis. Plasmapheresis and steroid and cyclophosphamide therapy were initiated following renal biopsy and resulted in normalization of anti-GBM antibody and p-ANCA titers, recovery of renal function, and discontinuation of hemodialysis. This case suggests that aggressive immunosuppression with plasmapheresis in patients who are p-ANCA positive with anti-GBM disease should be considered, even in those with severe renal dysfunction.

Acute oxalate nephropathy caused by ethylene glycol poisoning

Ethylene glycol (EG) is a sweet-tasting, odorless organic solvent found in many agents, such as anti-freeze. EG is composed of four organic acids: glycoaldehyde, glycolic acid, glyoxylic acid and oxalic acid in vivo. These metabolites are cellular toxins that can cause cardio-pulmonary failure, life-threatening metabolic acidosis, central nervous system depression, and kidney injury. Oxalic acid is the end product of EG, which can precipitate to crystals of calcium oxalate monohydrate in the tubular lumen and has been linked to acute kidney injury. We report a case of EG-induced oxalate nephropathy, with the diagnosis confirmed by kidney biopsy, which showed acute tubular injury of the kidneys with extensive intracellular and intraluminal calcium oxalate monohydrate crystal depositions.

Strangulated Small Bowel Obstruction Following Endoscopic Retrograde Cholangiopancreatography

No abstract available.

A Case of Giant Cell Arteritis Accompanied by Acute Myeloid Leukemia

Giant cell arteritis (GCA), the most common form of systemic vasculitis in adults, preferentially involves large and medium-sized arteries in patients over the age of 50. The classic manifestations are headaches, jaw claudication, polymyalgia rheumatica, and visual symptoms. Acute myeloid leukemia (AML) is a hematopoietic stem cell disorder characterized by a block in the differentiation of hematopoiesis, resulting in the growth of a clonal population of neoplastic cells or blasts. This malignant alteration in hematopoietic stem cells leads to a loss of normal hematopoietic function, which, if left untreated, typically leads to death within weeks to months of its clinical presentation. Although there have been reports of CLL or CML accompanied by several kinds of autoimmune vascular diseases, such as polymyalgia rheumatica, GCA, or necrotizing temporal arteritis, no studies have reported a case of AML with GCA. We experienced an 80-year-old male patient who developed AML 6 years after the diagnosis of GCA. He was under the use of oral glucocorticoid, hydroxychloroquine, and methotrexate at the time of the diagnosis of the AML. This is the first case in Korea to report GCA accompanied by AML.