A Case of Evans' Syndrome after Multiple Injuries and Septic Complications

Sir, Evans’ syndrome (ES) is defined as the concomitant or sequential association of warm autoimmune hemolytic anemia with immune thrombocytopenia (ITP) and, less frequently, autoimmune neutropenia.[1] Differential diagnoses such as thrombotic microangiopathies, anemia due to bleeding complicating ITP, or specific conditions must be ruled out.[1] The association of ES with other diseases, such as hematological malignancies, autoimmune diseases, or infections, can interfere with its management and alter its prognosis. Due to the rarity of the disease, the treatment of ES mostly relies on corticosteroids, rituximab, splenectomy, and supportive therapy. ES is still associated with high mortality. We herein report a case of ES that developed after multiple injuries and septic complications, including fungal infection. The patient was a 57-year-old woman who experienced a traffic accident while driving a truck. The patient was injured in a head-on collision with a passenger car. On arrival, she was in a shock state with restlessness and had midfacial comminuted fractures, left distal femur fractures, bilateral patellar fractures, bilateral distal tibial and fibular fractures, left Lisfranc fractures, and right calcaneus fractures [Figure 1]. Results of blood test were shown in [Table 1]. She underwent tracheal intubation and emergency transfusion. She repeatedly underwent surgery for trauma. She developed septic shock twice due to bacterial cholangitis and fungal infection of the respiratory system and sinus, followed by complicated hemolytic anemia with severe thrombocytopenia [Figure 2]. The haptoglobin level was found to be <10 mg/dL, and haptoglobin was administered. ES was suspected, so steroid therapy with antibiotics was initiated in addition to platelet and red cell transfusion. ADAMTS13 was measured when the platelet count was <1.0 × 104/mm3 and was found to be 19% (normal range). The patient was negative for heparin-induced thrombocytopenia antibodies. After the administration of a steroid, the patient’s respiration and circulation stabilized. Platelet counts showed a gradual increase. At the same time, culture examinations detected Klebsiella oxytoca and Gemella morbillorum from an extremity wound culture, Klebsiella oxytoca from a blood culture, Candida albicans from a sputum culture, and Candida albicans and Candida glabrata from a nasal discharge culture. She survived and was transported to another hospital for rehabilitation.Table 1: Results of a biochemical analysis on arrivalFigure 1: Results of radiological studiesFigure 2: Time courseIn the present case, the patient developed ES after multiple traumas; surgical treatment of the traumatic injuries; and complications of bacterial cholangitis, fungal lower respiratory tract infection, and sinusitis. As the presence of autoimmune diseases could not be confirmed in this case, trauma, surgical invasion, transfusion, and/or infection may have contributed to the induction of ES. To the best of our knowledge, no previous reports have described ES induced by trauma or fungal infection.[1-3] These factors could induce various cytokines either themselves or in association with tissue damage, leading to a cytokine storm.[4] A cytokine storm results in the formation of excessive immune dysregulation, followed by the development of ES.[2,5] Since these complications can be fatal and may go unnoticed if the clinician does not recognize the coexistence of ES, the accumulation of similar cases will be necessary to clarify the clinical course. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Research quality and ethics statement The authors followed applicable EQUATOR Network (https://www.equator-network.org/) guidelines, notably the CARE guideline, during the conduct of this report. Financial support and sponsorship This work was supported in part by a Grant-in-Aid for Special Research in Subsidies for ordinary expenses of private schools from The Promotion and Mutual Aid Corporation for Private Schools of Japan. Conflicts of interest There are no conflicts of interest.