Age: 26.0
Sex: F
Vaccine: PPV
Symptoms: Complement factor C3, Complement factor C4 decreased, Complement factor decreased, Computerised tomogram abdomen abnormal, Computerised tomogram head abnormal
Clinical narrative: Information has been received from the authors of the literature report. Learning Objective #1: To diagnose splenic infarction based on radiologic findings and the importance of vaccinations and early antibiotics in asplenic patients. Learning Objective #2: To suspect and perform hypercoagulable workup in patients presenting with evidence of end organ damage (splenic infarcts and abdominal pain) due to ischemia. Case: The authors present the case of a 26 year old female with past medical history significant for stercoral ulcer, complicated by bowel perforation who presented to the Emergency Department (ED) complaining of abdominal pain, vomiting, headache, subjective fever and facial pain. She was alert and oriented in the emergency department on arrival. She was given pain medications and fluids in the ED and basic labs were ordered. Four hours after her initial presentation, the patient was obtunded and minimally responsive to commands and intubated for airway protection. On repeat physical examination, her pupils were noted to be dilated and sluggish, with no corneal, cough or gag reflex. Stat computerized tomography (CT) scan of the head was negative for acute process and she was started on broad-spectrum antibiotics for suspected infection. Stat Magnetic Resonance Imaging (MRI) of the brain was suspicious for meningitis. Lumbar puncture was positive for Streptococcus pneumoniae, with protein 924 mg/dl, glucose <10 mg/dl, and white blood cells 3120 /UL with 86% polynuclear cells. Old records were reviewed. The patient's spleen was small in size compared to prior abdominal CT. On an initial admission CT for bowel perforation, the spleen was 8.5 cm on coronal section with wedge-shaped pattern appearing to be a splenic infarct. Three months later, on admission with meningitis diagnosis and repeat abdominal CT noted the spleen 5.5 cm on coronal section. She had received PCV (manufacturer unknown) (lot # not provided) on her previous admission with low streptococcus pneumoniae antibody response to the vaccine except for a few subsets. Pathologic report findings from her previous bowel perforation (documented as stercoral ulcer) showed ischemic mucosal injury pattern with mesenteric fat necrosis and intravascular organizing thrombi. During the prior admission, hypercoagulable workup was initiated and noted a positive antinuclear antibody (ANA) with a titer of 1:5120 and positive antiphospholipid antibodies (Lupus anticoagulant IgM 22 M units, Beta 2 Glycoprotein IgA 47 A units, Anticardiolipin IgM 39M units). The rest of the hypercoagulable workup including protein C, protein S, antithrombin III, homocysteine, and Factor V Leiden mutation was negative. Further workup revealed positive anti double stranded (ds) DNA antibody; anti Ro/SSA antibody >8 and Anti La/SSB antibody >8. Her C3 was normal and C4 and CH50 were low. The patient underwent 2 weeks of meningitis treatment with ventilator support without any clinical or neurological improvement. Repeat CT head was consistent with cerebral edema and anoxic changes. Goals of care were addressed with family, treatment was withdrawn and unfortunately the patient passed away. Her autopsy findings were significant for a possibly infarcted spleen which weighed 35 g. Discussion: Systemic lupus erythematosus (SLE) is a chronic inflammatory disorder characterized by multiorgan involvement. Antiphospholipid (ApL) antibodies are positive in 33% of patients with SLE. Antiphospholipid antibody syndrome (APS) is an acquired thrombophilia, caused by autoantibodies to beta 2 glycoprotein, lupus anticoagulant or anticardiolipin. Splenic involvement is seen in 9-20% of patients with SLE. Auto splenectomy can occur in patients with SLE due to vasculitis or due to splenic artery thrombosis. Splenic artery thrombosis is more commonly seen in patients with positive ApL antibodies. Thrombocytosis along with presence of Howell Jolly bodies, target cells, ovalocytes and spherocytes on peripheral smear, and evidence of atrophic spleen on imaging studies are imperative tip-offs of autosplenectomy among these patients. Asplenic patients must be vaccinated against encapsulated organisms. Prompt initiation of antibiotics in asplenic patients with symptoms suggestive of infection; along with vaccination; have shown to reduce mortality. Mesenteric vasculitis and infarction are life threatening lower gastrointestinal manifestations of SLE. These patients may develop necrotic bowel segments, sepsis or perforation of their intestine. Patients with SLE can also develop thrombosis causing an acute presentation of the disease; more commonly seen among patients with coexisting APS. Autoimmune conditions, particularly SLE and APS are known causes of hypercoagulable state along with other hematologic conditions. Young patients with pathology findings suggestive of ischemia and vascular thrombosis must undergo a hypercoagulable workup, as early detection of these conditions and initiation of anticoagulants may decrease morbidity and mortality among these patients. Additional information has been requested.