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Table 1 Characteristics and outcomes of nine patients with hemophagocytic syndrome who received tocilizumab. M, male; F, female; CAPS, catastrophic antiphospholipid syndrome; TMA, thrombotic microangiopathy; PVB19, parvovirus B19; LGL, large granular lymphocyte leukemia; HLH, hemophagocytic lymphohistiocytosis; DXM, dexamethasone; CYC, cyclophosphamide; IVIg, intravenous immunoglobulins; AIHA, autoimmune hemolytic anemia; SCT, stem cell transplantation; MMF, mycofenolate mofetil; Cst, corticosteroids; CsA, ciclosporin-A; CR, complete response; IS, immunosuppressive regimen; MV, mechanical ventilation; RRT, renal replacement therapy; VD, vasopressive drugs; OSL, organ support limitations; mHLH2009, modified 2009 HLH criteria

From: IL6-R blocking with tocilizumab in critically ill patients with hemophagocytic syndrome

 AgeGenderCause of HLHUnderlying immunodeficiencyOn-going IS at the onsetH-score/mHLH2009Other HLH therapyOrgan supportsHLH responseRelapseOutcomes
159MMultiple autoimmune disordersa, TMACstCst248 (99.3%)/7DXM, CYCMV, RRT, VDCRNoAlive
243MSepticemiaAllogenic SCTCst220 (96.3%)/5NoMV, RRT, VDCRNoDeath (septic shock; OSL)
323FIdiopathicHeart transplantationTacrolimus, MMF, Cst, IVIg210 (93%)/5NoRRT, VDCRNoAlive
460MInfections (varicella zoster virus, parvovirus B19, HSV-2), septicemiaHeart transplantationTacrolimus, MMF, Cst188 (78%)/5EtoposideMV, RRT, VDNoneDeath (septic shock, aspergillosis, refractory HLH)
552MParvovirus B19 and CAPSNoNo208 (92.5%)/5IVIg, DXMMV, RRT, VDCRNoAlive
653MIdiopathicLiver transplantationTacrolimus, MMF, Cst18 (79%)/5NoMV, RRTCRNoAlive
766FOverlap syndrome, TMACstCst, rituximab186 (75.8%)DXMMV, RRT, VDCRYes (etoposide)Death (gut ischemia; OSL)
857FRefractory AIHAT-LGL, B cell lymphomaDxm, CsA,188 (78%)/4CYC, DXMMV, RRT, VDCRNoDeath (septic shock, refractory AIHA)
925FS. hominis bacteriemia, HSV-1Kidney and liver transplantationTacrolimus, MMF, Cst218 (95.8%)/6No_CRNoAlive
  1. aPatient 1 was described in reference [3]. He was first hospitalized for thrombotic microangiopathy associated with autoimmunity and symptoms of rheumatoid arthritis, anti-synthetase syndrome, systemic lupus erythematosus, cryoglobulinemia, and Sjogren syndrome