Case Presentation
A 45-year-old man was transferred to our hospital for the evaluation of
renal dysfunction and anasarca. After the first dose of the COVID-19
mRNA vaccine (Moderna), he developed fever and macrohematuria for 3 to 4
days and developed edema and abdominal distension from the day after the
first COVID-19 mRNA vaccination. Six days after vaccination, his symptom
was persisted and he was detected renal dysfunction (eGFR 39.2
mL/min/1.73 m2, serum Creatinine 1.59 mg/dL), elevated
C-reactive protein value (22.4 mg/dL), and proteinuria.
On admission to our hospital, the patient was 171.0 cm tall and weighed
80.0 kg (weight gain of 7 kg in 1 week), with a blood pressure of 142/73
mmHg, pulse rate of 60 /min, and body temperature of 37.3 degree. He
complained of epigastric pain, dyspnea, and bilateral pitting edema of
the lower extremities. Oxygen saturation was 92% in room air.
Electrocardiography was normal, and echocardiogram revealed normal left
ventricular systolic function. Laboratory data revealed elevated levels
of C-reactive protein (22.6 mg/dL) and renal dysfunction (eGFR 49
ml/min/1.73 m2). The platelet count was 9.1 × 10³ /μL
(Table 1). The PCR test results for COVID-19 were negative.
Bilateral pleural effusions and a slight accumulation of ascites were
detected on computed tomography (CT) (Figure 1). There was no abnormal
uptake on gallium scintigraphy. Levels of IL-6 and VEGF in pleural
effusion and ascites were significantly elevated (Table 2).
Bone marrow biopsy showed hyperplastic and increased megakaryocytes, and
reticular fiber hyperplasia was partially observed by silver staining.
We performed renal biopsy for the definitive diagnosis of renal
injuries. Light microscopy showed diffuse hypercellularity with
thrombotic microangiopathy (TMA) lesions of the glomeruli. There was
diffuse and global endothelial cell enlargement due to cytoplasmic
swelling, with a large number of inflammatory cells (Figure 2a). The
partial dissolution of the mesangial matrix (mesangiolysis) is also
shown (Figure 2b). Endothelial
cell swelling occluding the capillary lumen with loss of fenestrations
and expansion of the subendothelial space was observed by electron
microscopy (Figure 2d,2e). Immunoperoxidase staining for CD34 and CD68
was positive (Figure 2f,2g). Immunofluorescence analysis revealed
negative staining for IgG, IgA, IgM, C3, and C1q.
Overall, this patient fulfilled three major categories
(thrombocytopenia, anasarca, and systemic inflammation) and three minor
categories (renal insufficiency, organomegaly, and myelofibrosis). Thus,
the patient was diagnosed with TAFRO syndrome. After second high-dose
steroid pulse therapy with 500 mg methylprednisolone for 3 days,
followed by prednisolone 40 mg/day, anasarca, systematic inflammation
and renal injuries were improved (C-reactive protein <0.1
mg/dL, eGFR 67 ml/min/1.73 m2). The platelet count
increased to the normal range on the 31st day of admission. Eleven
months after discharge, the patient had never relapsed under PSL
treatment (5 mg/day).