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Lei et al. HLH presenting as ACS

5
DNA copies to 1.3 × 10 copies/mL. Teramura et al. [20] particularly T-cell lymphoma, is most consistently
reported that the median EBV genome copy number associated with worse prognosis, [1,8,31] followed by older
3
at diagnosis was 3.0 × 10 (range: undetectable to 5.5 age [11,22,32] and abnormal karyotype. [21,26] Other factors
7
× 10 ) copies/mL in EBV associated HLH, in contrast found to correlate with poor prognosis include high
1
to 6.6 × 10 (range: undetectable to 1.0 × 10 ) copies/mL EBV viral load (≥ 1,000 copies/mL), [27] organ failure at
3
[1]
in infectious mononucleosis. EBER negativity may be admission, [11] hyperferritinemia, [31] hypoalbuminemia,
explained by the relatively early stage of the clinical male, splenomegaly and thrombocytopenia. [22] TCR
course when the liver biopsy was performed, or simply clonality does not appear to be of prognostic value. [26,27]
technical difficulties encountered in some cases. [21] In
addition, the liver biopsy specimen of our patient had Financial support and sponsorship
previously been frozen for intraoperative consultation. None.
The freeze-and-thaw process may have damaged the
EBV RNA to cause a falsely negative test. Conflicts of interest
There are no conflicts of interest.
Since our patient had no prior history of immunodeficiency,
it is intriguing what initiated the defect of her cellular Patient consent
immunity. According to two independent large cohort
studies, [8,22] concomitant hematologic malignancy and A Notice of Privacy Practices signed by the patient
active infection were found in 2.9-3.7% of adult HLH for approval of use of patient information for research
cases. Given the predominant T-lymphocytic infiltrate purposes is available for review upon request.
on the liver biopsy, a T-cell lymphoma is high in our Ethics approval
differential diagnoses. However, the possibility of an
underlying T-cell lymphoma is difficult to confirm or Case report is automatically waived and does not
exclude in our case due to the fulminant clinical course. require further approval by Institutional Review Board
Flow cytometry and cytogenetics might have aided in at Loma Linda University Medical Center.
the diagnosis if the patient had been able to tolerate
additional biopsies, particularly a bone marrow study. REFERENCES
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