Case description
Presenting clinical features
A 27-year-old previously healthy male patient (height 176 cm, and weight
79 kg) presented with fever, macrohematuria, and purpura in the lower
legs developed 4 days before admission, respectively. Written informed
consent was obtained, and approval of the ethics protocol was granted
from the Ethics Committee of Qianfoshan Hospital (2020111108). He denied
drug exposures and recent infectious illness. He had no abdominal pain
or diarrhea. Vital signs were normal and physical examination was
unremarkable except for petechiae. His mother died of anemia (details
unclear).
Laboratory findings revealed hemolytic anemia (hemoglobin level: 74 g/L;
hematocrit: 22.7%; reticulocyte count: 54×109/L; total bilirubin:
66 mg/L; indirect bilirubin: 51 mg/L; aspartate aminotransferase:
50 U/L; lactate dehydrogenase: 3489
U/L; and haptoglobin: undetectable), thrombocytopenia (platelet count:
9.0×109/L), and renal damage (Urinalysis disclosed a proteinuria score
of 2+, a red blood cell count of 8.4 per high-power field, a white blood
cell count of 4.4 per high-power field, and serum creatinine: normal).
Peripheral smear showed numerous schistocytes (1.2%). Prothrombin time,
partial thromboplastin time, and renal function test PT was
12.1 s, APTT was 37.9 s, fibrinogen was 5.25 g/L, factor Xa activity was
115%, and antithrombin III (AT III) activity was 92%, all within
normal limits. The laboratory tests showed a Direct antiglobulin test
(+), indicating peripheral cytopenias, particularly
autoimmune cytopenias (AIC) such as autoimmune thrombocytopenia.
Anti-SSA, Jo-52 (+). A bone marrow biopsy was also performed,
showing only erythroid hyperplasia without other abnormalities. A
diagnosis of ES was made given the evidence of immune-mediated hemolysis
with thrombocytopenia in the absence of a known etiology, we
administered methylprednisolone pulse therapy with the dose of 500 mg/d
for 3 consecutive days. At the following days, he had a drop in his Hgb
was from 15.2 g/dL to 7.4 g/dL, with an elevated LDH level soaring to
4136U/L.