Pseudo-thrombotic thrombocytopenic purpura presenting as multi-organ dysfunction syndrome: A rare complication of pernicious anemia

We present a rare case of pernicious anemia presented as multi-organ dysfunction syndrome, later found to have pseudo-thrombotic thrombocytopenic purpura.

An 86-year-old female presented with respiratory distress, altered mental status, acute renal failure and was intubated in emergency room. She was found to have severe anemia, thrombocytopenia, high lactate, high lactate dehydrogenase and low haptoglobin. Peripheral smear revealed multilobulated neutrophils with schistocytes, poikilocytes and anisocytes.

She was admitted to intensive care unit for altered mental status, multi-organ dysfunction syndrome with severe metabolic acidosis in setting of hemolysis. She was intubated and managed with intravenous antibiotics and blood transfusion. Patient improved significantly after blood transfusion. Lactic acid normalized, acute kidney injury resolved and mentation improved after transfusion. Laboratory investigation revealed low vitamin B12, high methylmalonic acid, high homocysteine, high lactate dehydrogenase, low haptoglobin, high anti-parietal antibody and high anti-intrinsic factor antibody. Patient was diagnosed with pernicious anemia and pseudo-thrombotic thrombocytopenic purpura with concomitant intramedullary hemolysis. Her hematological parameters and her clinical condition improved significantly after starting therapy with cyanocobalamin.

Pernicious anemia is a chronic disease with subtle presentation but may present as life-threatening complications. Hemolysis and pseudo-thrombotic thrombocytopenic purpura may present as multi-organ dysfunction syndrome which has dramatic response to appropriate therapy.