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).