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.