Case of the Month: Fever and Leg Pain

Case Authors: Caroline Miller, MD and Majed Refaai, MD

Clinical History

A young woman experiencing a sickle cell crisis presented with fever and leg/groin pain.

Past Medical History

Significant for sickle cell disease treated with hydroxyurea and multiple previous pain crises.

Recent History

Two weeks prior to the current vaso-occlusive crisis (VOC), the patient experienced an acute chest crisis, for which she was transfused multiple units of packed red blood cells (RBCs). Her most recent RBC antibody screen was negative.

Upon admission her hemoglobin (Hb) level was 8.6 g/dL. Over a few days, she experienced an acute drop in Hb level, reaching 4.4 g/dL. Despite the transfusion of 10 units of RBCs, her Hb continued to drop to 3.2 g/dL. Other labs included lactate dehydrogenase (LD) of >18,000 U/L, total bilirubin of 32.5 mg/dL, absolute reticulocyte count of 13.3 THOU/uL and markedly elevated liver function tests (AST of 9,976 U/L, ALT of 1,760). A direct antiglobulin test (DAT) was positive. She was treated with methylprednisolone, a monoclonal antibody (toculizimab) and intravenous immunoglobulin G (IVIG). Despite the aggressive treatment, the patient developed coagulopathy, multiorgan failure, intracranial bleeding and ultimately expired.

Next: Diagnosis