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A recently developed method to quantitate prothrombin in plasma uses the carinactivase-1 (CA-1) method. The present study was designed to establish the reference value by the CA-1 method in the neonatal period and to explore the effect of gestational age, birth weight, concurrent diseases, and vitamin K administration on the prothrombin levels. We enrolled 78 consecutive neonates. The gestational ages ranged from 28 to 41 weeks, and the birth weight ranged from 850 to 3750 g. Twenty-nine infants had concurrent diseases, and the others (n = 49) were healthy. A 300 microL blood sample was drawn into a plastic syringe containing 60 U freeze-dried buffered heparin. Prothrombin levels did not differ between appropriate-for-date (AFD) and light-for-date (LFD) babies (p = 0.090) or between groups with and without concurrent diseases (p = 0.210). In healthy AFD babies, prothrombin levels correlated with gestational age (r = 0.465, p = 0.003) and birth weight (r = 0.458, p = 0.003). In healthy low-birth-weight infants (n = 14) and those with concurrent diseases (n = 17), the changes after vitamin K administration were not significant. The CA-1 method is of clinical use in monitoring coagulation during the early neonatal period.

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Catastrophic antiphospholipid syndrome (CAPS) is a severe variant of antiphospholipid syndrome associated with multiorgan thrombosis in a short term. We present the case of a 14-year-old immunocompetent girl who developed renal, intestinal, and pulmonary infarction; thrombocytopenia; and hemolytic anemia within 1 week. She was diagnosed with thrombotic microangiopathy. Hence, plasma exchange and corticosteroid therapy were initiated, which improved thrombocytopenia. However, the patient’s platelet count decreased. Her general condition gradually worsened with eventual death. An autopsy revealed multiple infarctions in the kidneys bilaterally, jejunum, ileum, and pulmonary parenchyma. Microthrombi were not detected. Massive hemophagocytosis was observed in the splenic pulp, lymph nodes, and bone marrow. Several Epstein–Barr viruses (EBVs)-encoded small ribonucleic acid (RNA)-positive lymphocytes were also found in the bone marrow. The presence of antibodies to both viral capsid antigen-immunoglobulin G and EBV nuclear antigen indicated past infection. Antiphospholipid antibody was positive after her death. The patient was finally diagnosed with CAPS and EBV-associated hemophagocytosis, possibly due to EBV reactivation. Establishing a clinical diagnosis of CAPS was relatively difficult because two different causes of thrombocytopenia, CAPS and hemophagocytosis, led to a difficulty in understanding this case’s pathogenesis.