oxalates and Pancytopenia

Systemic oxalosis is a condition in which calcium oxalate crystals deposit into various bodily tissues. Although this may occur as the result of a rare primary syndrome in which an error of glyoxylate metabolism causes an overproduction of oxalate, it is more often seen as a secondary process characterized by increased enteric oxalate absorption. Here, we describe a patient with short bowel syndrome on long-term parenteral nutrition support who developed a unique manifestation of systemic oxalosis, leading to deposition of oxalate crystals within the bone marrow contributing to pancytopenia. In this report, in addition to reviewing the literature on this presumably rare manifestation of oxalosis, we also discuss its pathogenesis in the setting of short bowel syndrome and its management, including prevention.

Topics: Bone Marrow; Humans; Hyperoxaluria; Oxalates; Pancytopenia; Short Bowel Syndrome

Topics: Adult; Bone Marrow; Crystallization; Female; Humans; Hyperoxaluria, Primary; Oxalates; Pancytopenia

Topics: Birefringence; Bone Marrow; Crystallization; Female; Giant Cells, Foreign-Body; Humans; Hyperoxaluria, Primary; Kidney Failure, Chronic; Oxalates; Pancytopenia; Renal Dialysis; Young Adult

Combined liver and kidney transplantation is the ideal treatment for patients with end-stage renal failure secondary to primary hyperoxaluria and systemic oxalosis, with a functioning liver providing replacement of the deficient enzyme and a functioning kidney providing the route of excretion for the oxalate crystals. Pancytopenia from bone marrow infiltration of oxalate crystals is a rare complication of primary hyperoxaluria, and its reversal following transplant has not been described. We report the first case of pancytopenia from marrow infiltration by oxalate crystals reversing following a successful kidney transplant alone. Although kidney alone transplants do not provide the best chance of survival or quality of life as compared to a combined kidney and liver transplant, a well functioning kidney transplant is able to take care of the systemic oxalate load and ameliorate, at least for a period of time, the systemic complications of oxalosis.

Topics: Adult; Bone Marrow; Crystallization; Humans; Hyperoxaluria, Primary; Kidney Failure, Chronic; Kidney Transplantation; Male; Oxalates; Pancytopenia; Recurrence