Age: 55.0
Sex: F
Vaccine: COVID19
Symptoms: Computerised tomogram thorax, Cytomegalovirus test, Double stranded DNA antibody, Eosinophil count, Epstein-Barr virus antibody
Clinical narrative: RosaiDorfmanDestombes disease; Pain and swelling at the site of injection; Pain and swelling at the site of injection; COVID-19 Infection; This literature-non-study case was reported in a literature article and describes the occurrence of ROSAI-DORFMAN SYNDROME (RosaiDorfmanDestombes disease) in a 55-year-old female patient who received mRNA-1273 (Moderna COVID-19 Vaccine) for COVID-19 prophylaxis. The occurrence of additional non-serious events is detailed below.    The patient's past medical history included Infectious mononucleosis (At 24 years of age). Previously administered products included for Drug use for unknown indication: cefaclor. Past adverse reactions to the above products included Itchy rash with cefaclor. Concurrent medical conditions included Non-smoker.    On an unknown date, the patient received dose of mRNA-1273 (Moderna COVID-19 Vaccine) (unknown route) 1 dosage form. On an unknown date, the patient experienced ROSAI-DORFMAN SYNDROME (RosaiDorfmanDestombes disease) (seriousness criterion medically significant) and COVID-19 (COVID-19 Infection). an unknown date, the patient experienced VACCINATION SITE PAIN (Pain and swelling at the site of injection) and VACCINATION SITE SWELLING (Pain and swelling at the site of injection). The patient was treated with METHYLPREDNISOLONE at a dose of 24 milligram once a day and METHYLPREDNISOLONE at a dose of 8 milligram once a day. At the time of the report, ROSAI-DORFMAN SYNDROME (RosaiDorfmanDestombes disease), VACCINATION SITE PAIN (Pain and swelling at the site of injection), VACCINATION SITE SWELLING (Pain and swelling at the site of injection) and COVID-19 (COVID-19 Infection) had resolved.      DIAGNOSTIC RESULTS (normal ranges are provided in parenthesis if available): On an unknown date, Antinuclear antibody: (Positive) ANA titer was 1 to 320, and an ANA pattern of homogeneous. On an unknown date, Biopsy lymph gland: consistent with polytypic plasmacytosis, consistent with the areas of fibrosis rich in S100-positive cells that show evidence of emperipolesis consistent with RDD (Rosai-Dorfman-Destombes disease) and Showed Rosai-Dorfman disease with a marked widely spaced Reactive follicles with a prominent paracortex expansion, lymphocytic infiltrate in the paracortex against the background of abundant plasma cells, immunoblasts, and histiocytes. Numerous histocytes with vacuolated nuclei and abundant cytoplasm engulfing lymphocytes and other cells in a process termed emperipolesis (histiocytes with an abundant cytoplasm that have engulfed lymphocytes and other cells). Admixed paracortical T-cells. Positive in atypical lymphocytes showing Hodgkin's lymphoma unlikely.. On an unknown date, Biopsy skin: skin biopsy of a rash about a month later since presentation in the left mid-back that showed a perivascular cell infiltrate with eosinophils consistent with a drug reaction. On an unknown date, Blood iron: Within normal limits (a month later since contracting COVID- 19). On an unknown date, Blood test: consistent with bi-cytopenia (a month later). On an unknown date, Chest X-ray: within normal limits. On an unknown date, Complement factor C3: 49 mg/dl. On an unknown date, Complement factor C4: 4 mg/dl decreased. On an unknown date, Computerised tomogram neck: showed extensive bilateral levels of 14 lymphadenopathy, the largest of which was the left base of neck level IV, which was approximately 3 × 2.7 × 2.0 cm. On an unknown date, Computerised tomogram thorax: contrast showed bilateral axillary lymphadenopathy. The largest one on the right axilla was 2.6×1.2 cm. The largest one on the left axilla was 2.7 ×1.3 cm.. On an unknown date, Cytomegalovirus test: (Positive) Cytomegalovirus (CMV) IgM and IgG antibodies. On an unknown date, Double stranded DNA antibody: Negative. On an unknown date, Eosinophil count: 0.7 10^9/L. On an unknown date, Epstein-Barr virus antibody: Positive and Negative. On an unknown date, Flow cytometry: polyclonal B-cells, and T cells demonstrated no pan-T-cell antigen loss. On an unknown date, Haemoglobin: 10.9 gram per litre, (a month later since contracting COVID- 19) 9.6 gram per litre and 9 gram per litre. On an unknown date, Heart rate: 102 beats/min except for tachycardia. On an unknown date, Hepatitis A antibody: (Positive) Hep A IgG. On an unknown date, Hepatitis B virus test: Negative. On an unknown date, Hepatitis C virus test: Negative. On an unknown date, Histology: also consistent with the areas of fibrosis rich in S100-positive cells that show evidence of emperipolesis consistent with RDD.. On an unknown date, Lymphocyte count: 1.79 10^9/L, (a month later since contracting COVID- 19)  0.40 10^9/L and 0.340 10^9/L. On an unknown date, Mean cell volume: 71 femtolitre and 75 femtolitre. On an unknown date, Metabolic function test: within normal limits. On an unknown date, Monocyte count: 0.22 10^9/L. On an unknown date, Neutrophil count: 0.54 10^9/L, (a month later since contracting COVID-19) 1.16 10^9/L and 1.66 10^9/L. On an unknown date, Physical examination: showed a patchy erythematous plaque-like rash on the face and around the nose, the perioral area, and the chin, extending to involve the upper chest and the arms. A patchy erythematous plaque-like rash was also observed in the back and the upper extremities. On an unknown date, Platelet count: 296000 per cubic millimetre. On an unknown date, Polymerase chain reaction: showed a clonal rearrangement for Ig and a polyclonal pattern for TCR gamma (test performed for Ig and T-cell receptor (TCR) gamma gene rearrangement). On an unknown date, Red blood cell count: Consistent with Anemia and 5.04 10^9/L. On an unknown date, Red blood cell sedimentation rate: 46 millimetre and Elevated. On an unknown date, Red cell distribution width: 15.8 %. On an unknown date, SARS-CoV-2 test: (Positive) (Approximately 15 days later, after the biopsy). On an unknown date, Scan lymph nodes: showed diffuse adenopathy of the bilateral cervical area (levels I to V) more in the left than the right with a maximum size of 2 × 2 cm, palpable lymph nodes in bilateral axillae with a maximum size of 3 × 2 cm more in the left than in the right, and lymph nodes palpable in the bilateral inguinal area. Lymph nodes were soft in consistency and mildly tender. The rest of the examination was within normal limits. On an unknown date, Total complement activity test: less than 10 units/ml. On an unknown date, Transaminases: mildly elevated to alanine transaminase/aspartate transaminase (ALT/AST) of 49/59 units/L (a month later since contracting COVID- 19). On an unknown date, Treponema test: Negative. On an unknown date, Vital signs measurement: Within normal limits. On an unknown date, Weight decreased: over the last 3 months 4 kg. On an unknown date, White blood cell count: Consistent with Lymphopenia, 2.5 10^9/L, (a month later since contracting COVID- 19) 1.8 10^9/L and 2.3 10^9/L.     For mRNA-1273 (Moderna COVID-19 Vaccine) (Unknown), the reporter did not provide any causality assessments.   Concomitant medications were not provided.Patient has no known medical history who initially presented to the office with chief complaints of painless swelling in the left side of the neck and a generalized rash persisting for past 1 month. Never abused alcohol and never used illicit drugs. No recent over the counter drug or herbal supplement were used. The patient was healthy until COVID-19 vaccine booster vaccination. Prior vaccination history was uneventful. Approximately 1 week later, patient developed swelling on left side of the neck and 2 weeks later, developed diffuse urticarial rash.Approximately 15 days later, after the biopsy, patient developed fever and mild cough and tested positive for SARS-COV-19 however, symptoms resolved completely within a few days.Approximately 3 months later, after COVID-19 infection, patient presented again with a painless rash on her face (in the nose and around the mouth and chin), extending to the upper chest area. Also reported worsening of her cervical lymphadenopathy and progressively worsening of lymphadenopathy in the neck, the axilla, and the groin and developed a rash that progressed to involve the extremities and the back. Described constitutional symptoms including fatigue and a weight loss of 4 kg over the last 3 months. She developed rash involved the extremities and back. Patient declined experiencing fever, chills, or significant night sweats. QuantiFERON TB was indeterminate. Histoplasma antibodies and Toxoplasmosis IgG were negative. There was no mediastinal or hilar lymphadenopathy and no pulmonary nodule or mass. Patient had microcytic anemia and leucopenia. Skin rash improved and lymphadenopathy clinically improved by 80%. Fatigue and exercise tolerance were improved. After 2 months of steroids, symptoms were completely resolved. Author reported biopsy confirmed case of RDD after receiving the COVID-19 mRNA vaccine and contracting a COVID-19 infection.  Company comment: This literature-non-study case concerns a female patient aged 55 years with no significant medical history or recent use of new drugs, who experienced the unexpected serious (medically significant) event of Rosai-Dorfman syndrome along with non-serious event of Covid-19 after booster dose of mRNA-1273 vaccination.  About 4 days after vaccination, patient had left arm pain and swelling at the site of injection, a week later she developed swelling on the left side of the neck, followed by a diffuse urticarial rash after 2 weeks. Skin biopsy of the rash, done about a month later was consistent with a drug reaction. Blood tests done showed WBC count of 2.5 × 109 /L, Hb-10.9 g/L, ALC- 1.79 ×109 /L, ANC- 0.54 × 109 /L, metabolic panel was within normal limits. Two weeks after the biopsy, she developed fever, mild cough and was tested positive for SARS-COV-19; however, her symptoms resolved completely within a few days. Repeat blood work done a month later after covid infection was also consistent with bi-cytopenia with Hb-9.6 g/L and a WBC count- 1.8 × 109 /L with ANC of 1.16 and ALC of 0.40 × 109 /L, liver transaminases were mildly elevated, with normal iron studies. About 3 months after covid infection, she presented again with a painless rash on her face, extending to the upper chest area, extremities and the back, worsening of her lymphadenopathy, cervical, in the neck, axilla, and groin, along with constitutional symptoms of fatigue and weight loss. Vitals were normal, patchy erythematous plaque-like rash, diffuse adenopathy of the bilateral cervical area/ axilla/inguinal area were noted. Blood tests showed WBC count of 2.3 × 109 /L, ANC of 1.66 × 109 /L, ALC of 0.340 × 109 /L, Hb-9g/L, ESR- 46 mm. Chest x-ray -normal limits. Cytomegalovirus IgM and IgG antibodies were positive, EpsteinBarr virus IgG was positive, Hep A IgG was positive. Complement C3 /C4 were decreased. ANA was positive. CT neck done 1 month later showed extensive bilateral levels of 14 lymphadenopathy, Chest CT showed bilateral axillary lymphadenopathy. Supraclavicular lymph node biopsy done showed atypical interfollicular B-cell proliferation associated with RosaiDorfman disease and a marked polyclonal plasmacytosis. Polymerase chain reaction performed for Ig and T-cell receptor (TCR) gamma gene rearrangement showed a clonal rearrangement for Ig and a polyclonal pattern for TCR gamma. Treatment was initiated with methylprednisolone at a dose of 24 mg a day, tapered down over 3 weeks to 8 mg daily. Patient showed clinical improvements, skin rash improved, lymphadenopathy clinically improved by 80%, fatigue and exercise tolerance improved. She continued on a slow taper of steroids over the next 3 weeks. After 2 months of taking steroids, patient reported a complete resolution of her symptoms. Author states that the detection of clonal B-cell IgH gene rearrangements in the patient's lymph node may be related to the patient's exposure to viruses or COVID-19 vaccination. The benefit-risk relationship of mRNA-1273 is not affected by this report.   Most recent FOLLOW-UP information incorporated above includes: On 12-Jan-2023: Follow up received by safety on 12-Jan-2023 has Email with FTA and article propagated contains significant information: citation details were added, medical history was added, suspect anatomical site was added, event onset details were added, laboratory tests were added, treatment details were added, event outcome was updated, narrative was updated.; Sender's Comments: This literature-non-study case concerns a female patient aged 55 years with no significant medical history or recent use of new drugs, who experienced the unexpected serious (medically significant) event of Rosai-Dorfman syndrome along with non-serious event of Covid-19 after booster dose of mRNA-1273 vaccination.  About 4 days after vaccination, patient had left arm pain and swelling at the site of injection, a week later she developed swelling on the left side of the neck, followed by a diffuse urticarial rash after 2 weeks. Skin biopsy of the rash, done about a month later was consistent with a drug reaction. Blood tests done showed WBC count of 2.5 × 109 /L, Hb-10.9 g/L, ALC- 1.79 ×109 /L, ANC- 0.54 × 109 /L, metabolic panel was within normal limits. Two weeks after the biopsy, she developed fever, mild cough and was tested positive for SARS-COV-19; however, her symptoms resolved completely within a few days. Repeat blood work done a month later after covid infection was also consistent with bi-cytopenia with Hb-9.6 g/L and a WBC count- 1.8 × 109 /L with ANC of 1.16 and ALC of 0.40 × 109 /L, liver transaminases were mildly elevated, with normal iron studies. About 3 months after covid infection, she presented again with a painless rash on her face, extending to the upper chest area, extremities and the back, worsening of her lymphadenopathy, cervical, in the neck, axilla, and groin, along with constitutional symptoms of fatigue and weight loss. Vitals were normal, patchy erythematous plaque-like rash, diffuse adenopathy of the bilateral cervical area/ axilla/inguinal area were noted. Blood tests showed WBC count of 2.3 × 109 /L, ANC of 1.66 × 109 /L, ALC of 0.340 × 109 /L, Hb-9g/L, ESR- 46 mm. Chest x-ray -normal limits. Cytomegalovirus IgM and IgG antibodies were positive, EpsteinBarr virus IgG was positive, Hep A IgG was positive. Complement C3 /C4 were decreased. ANA was positive. CT neck done 1 month later showed extensive bilateral levels of 14 lymphadenopathy, Chest CT showed bilateral axillary lymphadenopathy. Supraclavicular lymph node biopsy done showed atypical interfollicular B-cell proliferation associated with RosaiDorfman disease and a marked polyclonal plasmacytosis. Polymerase chain reaction performed for Ig and T-cell receptor (TCR) gamma gene rearrangement showed a clonal rearrangement for Ig and a polyclonal pattern for TCR gamma. Treatment was initiated with methylprednisolone at a dose of 24 mg a day, tapered down over 3 weeks to 8 mg daily. Patient showed clinical improvements, skin rash improved, lymphadenopathy clinically improved by 80%, fatigue and exercise tolerance improved. She continued on a slow taper of steroids over the next 3 weeks. After 2 months of taking steroids, patient reported a complete resolution of her symptoms. Author states that the detection of clonal B-cell IgH gene rearrangements in the patient's lymph node may be related to the patient's exposure to viruses or COVID-19 vaccination. The benefit-risk relationship of mRNA-1273 is not affected by this report.