Case presentation
A 36-year-old woman was referred to our hospital with chief complaints
of fever and night sweeting. The patient mentioned that she had pain in
the Interphalangeal joints from about 1.5 years ago, after receiving
COVID-19 vaccine. She was referred to different physicians and oral
corticosteroids and non-steroidal anti-inflammatory drugs (NSAIDs) were
prescribed for her. She didn’t have fever, night sweeting, pharyngitis
symptoms, or skin rash at that time. She denied photosensitivity, malar
rash, or Raynaud phenomenon. The patient had unwilling weight loss about
10 kilograms in less than 6 months.
On the first day of admission, the patient had pharyngitis, fever, red
macular rashes in the trunk without face involvement (figure1), pain in
knees and little small joints of the hands since two weeks before
referring to us and the fever responded to NSAIDs first, but after a few
days, the fever didn’t respond to NSAIDs. There was no pain in back,
pelvic, or chest. And also she didn’t have dyspnea.
She was referred to another hospital and physicians prescribed
antibiotic for her and infectious work-up was performed for her. She had
anemia in evaluations and packed cell was prescribed for her. In that
center, 2ME, wright, coombs wright, anti-nuclear antibodies (ANA),
anti-double-stranded DNA (anti-ds-DNA), anti-citrulinated peptide
antibody (anti-CCP), human leukocyte antigen (HLA) B-27 were checked and
all were negative. Also, blood culture was negative. All laboratory data
are seen in table 1. The patient was referred to our hospital for more
evaluations.
In our center, due to persistent fever, pharyngitis symptoms, cutaneous
rashes, digital arthralgia, and negative serology tests, Still’s disease
was diagnosed for the patient. Also, rheumatologists were suspicious to
Hemophagocytic Lymphohistiocytosis (HLH) and malignancy due to
decreasing trends of blood cells. So, hematology consult was request for
her. Hematologist requested bone marrow aspiration (BMA). The report of
BMA confirmed HLH disease because hemophagocytosis was observed in her
BMA specimen.
With this diagnosis, 4 milligrams of intravenous dexamethasone every 8
hours was prescribed for him and all blood parameters and blood ferritin
had improving trends.
We were suspicious to malignancies and we requested CT scan with
contrast. In CT scan we observed mediastinum, supraclavicular, and
axillary lymphadenopathy. There were no significant findings in the CT
scan. CT scans of other organs were normal.
The patient was discharged with a good condition, without any
complaints, and with oral drugs (tab Dexamethasone 0.5 mg/daily, tab
Cyclosporine 50mg/daily, tab Hydroxychloroquine 200 mg/daily).