A 52-year-old Black female with a history of sickle cell disease is transferred to The Johns Hopkins Hospital with fever and chills.

A 52-year-old Black female with sickle cell disease for which she had required biweekly blood transfusions for the past 30 years presented to an outside hospital with a two-week history of fever and chills. She was admitted and subsequent blood cultures grew methicillin resistant Staphylococcus aureus (MRSA). Broad spectrum antibiotics were started and her Port-a-cath, which was felt to be the source of the infection, was removed. Subsequent transesophageal echocardiogram revealed an aortic valve vegetation and large right atrial thrombus. She was transferred to The Johns Hopkins Hospital for consideration of aortic valve replacement and thrombectomy.

Sickle cell disease, transfusion dependant with development of anti-C, anti- V, and, anti-K1 antibodies

Chronic Renal Insufficiency

Hydroxyurea 1500mg qd
Lasix 40 mg bid
Folate 1 mg qd
Lisinopril 5 mg qd

She smoked as a young woman for <10 years, she is a social drinker, lives at home with husband and three children. No history of illicit drug use.

Father died of colon cancer in his 70's, mother had rheumatoid arthritis

NKDA

General: alert, obese Black female in mild distress
Vitals: 120/76, HR 108, RR 24, Temp 101.8
HEENT: icteric sclerae, PERRL, EOMI, oropharynx unremarkable
Cardiac: grade 3 holosystolic murmur heard best at right sternal border
Chest: decreased breath sounds in right upper lobe, crackles present at bases bilaterally
Abdomen: Hepatomegaly with the liver palpable 5-7 cm below the right costal margin, bowel sounds present, soft, non-tender. No masses
Neurologic: CNs intact, motor/sensory intact and symmetric throughout
Skin: No evidence of peripheral embolic phenomena

WBC 17,971 M/CU
          81% Polymorphonuclear cells
          12% Lymphocytes
          6% Bands
          1% Monocytes
Hemoglobin 7.0 G/DL
Hematocrit 21%
PLT 96, 000 M/CU
MCV 86 FL
RDW 23.7%
Reticulocyte Count 2.0%
NA 142 mEQ/L
K 4.9 mEQ/L
CL 103 mEQ/L
BUN 111 MG/DL
Creatinine 2.1 MG/DL
Albumin 1.7 G/DL
Total Bilirubin 11.5 MG/DL
Direct Bilirubin 8.2 MG/DL
Aspartate Aminotransaminase 84 U/L
Alanine Aminotransaminase 30 IU/L
Alk Phos 247 IU/L
Ferritin 11, 000 ng/ml

Review of outside TEE- laminar, non-mobile right atrial thrombus felt to be chronic from port-a-cath, chronic aortic stenosis with calcification unchanged from previous study, no definitive evidence of vegetation.
Head CT- no acute changes, remote lacunar infarct in left anterior putamen, thickened calvarium c/w sickle cell disease
Axial CT of the lower chest without contrast-There is cardiomegaly and calcification of the mitral valve. There is consolidation in the right lower lobe compatible with pneumonia. There is a smaller infiltrate in the left lower lobe. (Image 1).
Axial CT of the upper abdomen without contrast-The liver is hyperdense. The inferior vena cava and the hepatic veins are prominent. There are rim calcifications within the spleen (Image 2).

The patient was admitted and broad spectrum antibiotics were continued for possible sepsis. Her bilirubin continued to climb after admission. No blood transfusions were administered after admission. Additionally her renal function deteriorated and she eventually became anuric. Blood cultures were negative on antibiotics. By day 5 of hospitalization, her bilirubin was 38 mg/dl, BUN 135, creatinine 4.4 mg/dl. After consultation with the medical team, the family declined hemodialysis and opted for comfort care only. On hospital day 6 the patient expired. After informed consent, a post mortem examination was performed.

What factor(s) predisposed this woman to sepsis?

What is the most likely etiology of the patient's hepatic and renal dysfunction?