A case of complete atrioventricular block in secondary hemophagocytic syndrome/hemophagocytic lymphohistiocytosis recovered by plasma exchange and cytokine absorbing therapy with AN69ST continuous hemodiafiltration
Open Access
- 6 May 2020
- journal article
- case report
- Published by Taylor & Francis Ltd in Immunological Medicine
- Vol. 43 (4), 171-178
- https://doi.org/10.1080/25785826.2020.1761145
Abstract
We report a case of incipient systemic lupus erythematosus (SLE) that rapidly progressed to complete atrioventricular block (cAVB). A 20-year-old man was admitted with facial erythema, painless oral aphtha, polyarthritis, and myalgia of each extremity. On admission, he developed first-degree atrioventricular block, pericarditis, pleuritis, renal failure, hemophagocytic lymphohistiocytosis, neurophychiatric SLE (left cerebellar infarction), and Staphylococcus aureus bacteremia. He was subsequently diagnosed with SLE based on several positive findings on immunological tests (including positive for antinuclear antibody). Despite immediate glucocorticoid pulse therapy and plasma exchange (PE) along with antibiotic, he developed cAVB that required temporary pacing on day 2. Because it was thought that hypercytokinemia exacerbated pericarditis, which progressed to myocarditis and cAVB, we decided to PE and cytokine-adsorbing therapy with AN69ST-continuous hemodiafiltration (CHDF). Other than renal failure, his organ dysfunctions improved with the multidisciplinary therapy. CAVB improved and temporary pacing was no longer required on day 11. Even a first-degree atrioventricular block can rapidly progress to cAVB; therefore, strict attention to electrocardiogram is necessary in severe SLE cases. When presenting with organ dysfunctions caused by hypercytokinemia such as severe SLE cases or SLE with severe infection cases, use of the combination of PE and AN69ST-CHDF might be beneficial.Keywords
This publication has 30 references indexed in Scilit:
- Hemophagocytic LymphohistiocytosisAnnual review of pathology, 2018
- Successful treatment of fatal macrophage activation syndrome and haemophagocytic lymphohistiocytosis by combination therapy including continuous haemodiafiltration with a cytokine-adsorbing haemofilter (AN69ST) in a patient with systemic lupus erythematosusModern Rheumatology Case Reports, 2018
- Infection in systemic lupus erythematosus, similarities, and differences with lupus flareThe Korean Journal of Internal Medicine, 2017
- Continuous Hemodiafiltration with a Cytokine-Adsorbing Hemofilter in Patients with Septic Shock: A Preliminary ReportBlood Purification, 2014
- Continuous Hemodiafiltration with a Cytokine-Adsorbing Hemofilter for SepsisBlood Purification, 2012
- Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosusArthritis & Rheumatism, 2012
- Ruptured Sinus of Valsalva and Complete Atrioventricular Block Complicating Fulminant Course of Infective Endocarditis: A Case Report and Literature ReviewThe Kaohsiung Journal of Medical Sciences, 2006
- Cardiac involvement in systemic lupus erythematosusLupus, 2005
- A Prospective Multicenter Study of Staphylococcus aureus BacteremiaMedicine, 2003
- High‐grade atrioventricular heart block in an adult with systemic lupus erythematosus: The association of nuclear RNP (U1 RNP) antibodies, a case report, and review of the literatureArthritis & Rheumatism, 1989