A 66 year -old woman presented with three days of worsening abdominal pain. She described the pain as severe sharp epigastric pain which radiated to her back and both lower abdominal quadrants. She endorsed daily alcohol use. Admission labs were significant for: Lipase 10,401 units/L, hemoglobin 13.7 g/dL, platelets 272 10e9/L, and creatinine of 1.3 mg/dL. CT of the abdomen revealed pancreatitis without any focal fluid collection. The patient was admitted for treatment of alcoholic pancreatitis. On hospital day three, her lab results had drastically changed to hemoglobin 7 g/dL, platelets 40 10e9/L, and creatinine 4.8 mg/dL. Heparin Induced Platelet antibody was negative. She developed hallucinations and fevers, but had improving abdominal pain. She did not have petechiae or areas of ecchymosis. LDH was 860 units/L; haptoglobin was low normal at 49 mg/dL; Direct Antiglobulin Test was negative ruling out autoimmune hemolysis; fibrinogen was 701mg/dL ruling out disseminated intravascular coagulation; schistocytes were seen on peripheral smear. She was diagnosed with Thrombotic Thrombocytopenic Purpura and underwent plasmaphoresis. Mental status, hemoglobin, and platelet count all eventually recovered. Unfortunately, her renal function improved but did not return to baseline. ADAMST13 activity was 74%; results returned several days after plasmaphoresis was initiated.
Microangiopathic Hemolytic Anemia (MAHA) is diagnosed when non-immune mediated hemolysis is found with schistocytes. Additional findings include elevated LDH, low haptoglobin, anemia and elevated direct bilirubin. Negative Coombs tests confirms that the hemolysis is non-immune mediated. The vast majority of MAHA is classified as Thrombotic Thrombocytopenic Purpura (TTP) or Hemolytic-Uremic Syndrome (HUS). The classic pentad for diagnosis of TTP includes: MAHA, thrombocytopenia, fever, acute renal insufficiency, and neurologic changes. It is uncommon to have all five criteria fulfilled. TTP should be considered when MAHA and thrombocytopenia are encountered. Confirmatory testing for ADAMST13 should not delay evaluation for plasma exchange. It should be noted that while ADAMST13 activity is decreased in a large majority of TTP cases, it can be within normal limits.
Pancreatitis is a commonly encountered diagnosis by the Hospitalist. The acute illness can lead to bone marrow suppression resulting in anemia and thrombocytopenia. Although rare, pancreatitis can cause MAHA. Hospitalists should be vigilant for the development of MAHA with pancreatitis as it will likely be a part of either TTP or HUS. The failure to recognize and promptly treat TTP-HUS can be fatal.