Atypical haemolytic uremic syndrome secondary to acute pancreatitis: a unique presentation

Abstract

Atypical haemolytic uraemic syndrome (aHUS) is a disease of complement dysregulation and can be fatal if not treated in a timely manner. Although normally associated with triggers such as infection or pregnancy, this case demonstrates acute pancreatitis as the triggering event. The patient’s initial presentation of thrombocytopaenia and acute renal failure was first attributed to a systemic inflammatory response syndrome due to pancreatitis, but with detailed history and further laboratory investigation, we were able to show that patient was having symptoms associated with aHUS. On early recognition of aHUS, this patient was able to receive the proper standard of care with eculizumab and had a full recovery while preventing renal failure. When patients present with thrombocytopaenia and renal failure in acute pancreatitis, we want to ensure physicians keep aHUS on the differential.

Rare Presentation of Atypical Hemolytic Uremic Syndrome in a Child With Acute Lymphocytic Leukemia and Pancreatitis

“…HUS or aHUS are rarely reported as a complication in pediatric ALL and unlike our patient, mostly in the context of active disease. Literature review revealed 6 pediatric cases in which aHUS preceded the diagnosis of acute leukemia,2,4,5,8–10 1 case of simultaneous presentation with ALL,1 2 cases where aHUS occurred during ALL induction chemotherapy,3,6 and 2 cases of HUS during maintenance therapy 1,7. One reported patient presented with Shiga toxin producing Escherichia coli O157:H, a hallmark of typical HUS, followed by a diagnosis of ALL 9…”

“…Case reports of aHUS that were considered to be associated with ALL therapy include a fatal case of aHUS in a 4-year-old girl during ALL induction therapy, postulated to have been triggered by l -asparaginase. That patient had also received vincristine and dexamethasone 7. Kanchi and colleagues described a child with ALL who developed recurrent aHUS at 23 and 47 weeks of chemotherapy, thought to be a complication from vincristine 8.…”

“…We present a four-year-old boy with a history of developmental delay, diagnosed with standard risk B-cell acute lymphoblastic leukemia (B-ALL),1–10 who underwent treatment per Children’s Oncology Group (COG) AALL0932 protocol with induction chemotherapy of dexamethasone, vincristine, and polyethylene glycol (PEG)-asparaginase and intrathecal cytarabine and methotrexate 11. On day 24 of induction, he presented with severe abdominal pain, emesis and constipation.…”

Atypical Hemolytic Uremic Syndrome Secondary to Pancreatitis: A Case Report

“…There are various reports of autoimmune pancreatitis with autoimmune hemolytic anemia [ 11 ], TTP [ 12 ], HUS [ 13 ], and aHUS [ 6 ] in TMA secondary to pancreatitis, and drug-induced TMA [ 14 ] is also a possibility when drugs potentially causing TMA are used before or during hospitalization. However, TMA, including aHUS, must be assumed when pancreatitis is accompanied by elevated creatinine, hemolytic anemia, and low platelets, and peripheral blood smears or assay of ADAMTS13 levels, for example, should be employed for further differentiation of TMA.…”

“…In addition to the fact that aHUS is an extremely rare disease, with a prevalence of 4.96 per million total population [ 5 ], cases of aHUS triggered by acute pancreatitis are extremely rare in the literature, having been reported only by Barish et al [ 6 ]. This is a case report of an aHUS that appears to have been triggered by acute pancreatitis.…”