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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

 

Age

Gender

Cause of HLH

Underlying immunodeficiency

On-going IS at the onset

H-score/mHLH2009

Other HLH therapy

Organ supports

HLH response

Relapse

Outcomes

1

59

M

Multiple autoimmune disordersa, TMA

Cst

Cst

248 (99.3%)/7

DXM, CYC

MV, RRT, VD

CR

No

Alive

2

43

M

Septicemia

Allogenic SCT

Cst

220 (96.3%)/5

No

MV, RRT, VD

CR

No

Death (septic shock; OSL)

3

23

F

Idiopathic

Heart transplantation

Tacrolimus, MMF, Cst, IVIg

210 (93%)/5

No

RRT, VD

CR

No

Alive

4

60

M

Infections (varicella zoster virus, parvovirus B19, HSV-2), septicemia

Heart transplantation

Tacrolimus, MMF, Cst

188 (78%)/5

Etoposide

MV, RRT, VD

None

Death (septic shock, aspergillosis, refractory HLH)

5

52

M

Parvovirus B19 and CAPS

No

No

208 (92.5%)/5

IVIg, DXM

MV, RRT, VD

CR

No

Alive

6

53

M

Idiopathic

Liver transplantation

Tacrolimus, MMF, Cst

18 (79%)/5

No

MV, RRT

CR

No

Alive

7

66

F

Overlap syndrome, TMA

Cst

Cst, rituximab

186 (75.8%)

DXM

MV, RRT, VD

CR

Yes (etoposide)

Death (gut ischemia; OSL)

8

57

F

Refractory AIHA

T-LGL, B cell lymphoma

Dxm, CsA,

188 (78%)/4

CYC, DXM

MV, RRT, VD

CR

No

Death (septic shock, refractory AIHA)

9

25

F

S. hominis bacteriemia, HSV-1

Kidney and liver transplantation

Tacrolimus, MMF, Cst

218 (95.8%)/6

No

_

CR

No

Alive

  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