You are presented with a 61-year-old-man who has a 4-week history of jaundice,
light-colored stools, dark urine, pruritis, nausea, fatigue, and fever with temperatures
spiking to 38.5°C, and a 1-week history of facial and lip swelling. Six weeks previously
he was hospitalized for Covid-19 for which he received intravenous glucocorticoids
and broad spectrum antibiotics, the latter for presumed secondary bacterial pneumonia.
On examination he had normal vital signs, swelling of his lips, face, and eyelids,
conjunctival icterus, and an enlarged, tender liver without splenomegaly.
His hemoglobin was 12.9 g/dL, platelet count 261,000, and white blood cell count
4,600 with an absolute neutrophil count of 260. His total bilirubin level was 14.3
mg/dL with a direct fraction of 13.2 mg/dL. His ALT was 305 U, AST 156 U/dL and alkaline
phosphatase 241 U/L. His serum creatinine was 1.3 mg/dL. A PCR test for Covid-19 was
negative, and he had serum antibodies to Covid-19 nucleocapsid and spike antigens.
What is you diagnosis, what test(s) would you order to confirm your suspicions,
and what treatment, if any do you recommend?
DIAGNOSIS: Post-Covid19 hyperinflammatory syndrome with hemophagocytic lymphohistiocytosis
(HLH).
In a subgroup of patients with COVID19, despite viral clearance and sometimes
after an asymptomatic period, autoimmunity or SARS-CoV-2 antigenic mimicry of host
proteins (one theory) leads to immune hyperactivation involving both the innate and
adaptive immune systems. In turn, this may cause HLH which is characterized by cytopenias
and high levels of inflammatory markers (ESR, CRP, IL-6, TNF-alpha, etc.), very high
ferritin levels, low fibrinogen levels, and hemophagocytosis. The treatment is parenteral
and then oral administration of glucocorticoids to which this patient responded.
Figure depicting the clinical features of the hyperinflammatory syndrome (left image). Liver biopsy shows hemophagocytosis with macrophages (green arrows) engulfing red cells (yellow arrows). Cholestatic hepatitis is evidenced by hepatocytes with cholestasis (bile pigment, blue arrows), and dying hepatocytes (apoptosis, black arrows) (right image).
Reference: Braga Neto, M.B., Badley, A.D., Parikh, S.A., Graham, R.P., Kamath, P.S. Calm before the storm. N Engl J Med 2022. 386:5, 479-485.
Bonus Question: What is this creature's name and which one of his survival habits correlates with the practice of bedside medicine? ANSWER: This lemur is called an AYE AYE. It makes its living by percussing logs, branches, etc. in search of grubs and other insects. Medical students and physicians hone their percussion skills to become expert bedside diagnosticians.
The AYE AYE is the official mascot of the Advanced Physical Diagnosis SWAT Teams.