Table 2.
Details on patients, their underlying diseases, treatment protocols, HLH-specific treatment, possible triggers, peak levels of inflammatory markers and outcome of the HLH
| Pat. nr. | Year of onset | Age at onset of HLH | Sex | Underlying disease | Treatment protocol | State at diagnosis of HLH | HLH-specific therapy | Outcome of HLH | Preceding treatment with LAmB | Preceding parenteral nutrition | Concurrent infections | Peak CRP level (mg/l) | Peak PCT level (ng/ml) |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 1995 | 3.9a | m | Pre-B-ALL | ALL REZ BFM 90 | Maintenance therapy | IVIG | Died (1 day after diagnosis) | n.a. | n.a. | InflA | n.a. | n.a. |
| 2 | 1997 | 17.1a | f | AML FAB M2 | AML BFM 93 | Maintenance therapy | IVIG, Dxm, VP-16 | Died (47 days after diagnosis) | No | no | EBV, paranasal aspergillosis | 104.7 | n.a. |
| 3 | 1997 | 1.5a | m | Medulloblastoma | HIT SKK 92 | Tumour progression under treatment | Dxm., VP-16 | Died (2 days after diagnosis) | n.a. | Yes | CMV | n.a. | n.a. |
| 4 | 2001 | 17.1a | m | Mediastinal germ cell tumour | HLH therapy | HLH as initial presentation | IVIG, VP-16, Dxm, Daclizumab, CS-A | Died (23 days after diagnosis) | No | No | – | 16.0 | n.a. |
| 5 | 2002 | 15.5a | m | Anaplastic large T cell lymphoma | ALCLl | HLH as initial presentation | G-CSF, Dxm, VP-16 | Remission | No | No | PVB19 | 25.0 | n.a. |
| 6 | 2002 | 11.0a | f | C-ALL, second relapse | ALL REZ BFM Pilot 02; BMT | Day + 68 after MUD BMT, acute GvHD | VP-16, Dxm, Daclizumab |
Remission | Yes | Yes | EBV, HHV6, pulmonary aspergillosis | n.a. | n.a. |
| 7 | 2005 | 10.7a | f | AML, FAB M2 | AML BFM 2004 | Day + 24 after allogeneic HLA identical BMT, acute GvHD | Dxm | Remission | No | Yes | – | < 5.0 | n.a. |
| 8 | 2006 | 12.4a | f | Ewing sarcoma | EURO Ewing 99 | Sixth VIDE | Dxm, infliximab | Remission | Yes | Yes | – | 128 | n.a. |
| 9 | 2007 | 14.8a | f | AML FAB M2 (refractory) | RIC MUD alloPSCT | Day + 2 after RIC PSCT | Infliximab, daclizumab, methylprednisolone | Remission | Yes | Yes | EBV | 236 | n.a. |
| 10 | 2008 | 16.8a | f | AML FAB M1 | AML BFM 2004 | 1 + 2: maintenance therapy | 1: Dxm, infliximab; 2: Dxm, rituximab, IVIG, infliximab | Died in second episode (132 days after first diagnosis) | Yes | No | 1: ADV, BKV, HHV6, PVB19; 2: EBV | 264 | n.a. |
| 11 | 2008 | 18.0 | f | Mb. Krabbe | Second HaploPSCT | Day + 22 after 2nd haploSCT (graft rejection after the 1st SCT) | Dxm, infliximab | Remission | No | No | Cerebral toxoplasmosis | 157.0 | 0.47 |
| 12 | 2008 | 7.5a | f | Secondary AML after treatment of embryonal rhabdomyosarcoma | CWS 2002, MUD SCT | Day + 44 after MUD SCT | Dxm, IVIG | Remission | Yes | Yes | BKV, HHV-6, PVB19 | 42.4 | n.a. |
| 13 | 2009 | 2.4a | f | AML FAB M6 refractory to treatment | Allogeneic peripheral SCT | Conditioning for allogeneic SCT | Dxm, infliximab | Remission | Yes | Yes | HHV6, RSV | 220.4 | 13.59 |
| 14 | 2010 | 10.9a | f | AML FAB M5 | AML BFM 2004 (HR) | 1: AI-2CDA consolidation, 2–4: maintenance treatment | Dxm | Remission after fourth episode | Yes | Yes | 1: BKV; 2:-4: CMV | 90.2 | n.a. |
| 15 | 2011 | 1.3a | f | AML FAB M5a | AML BFM 2004 | 1: end of ADxE induction 2: re-induction of HAM | Dxm | Remission after second episode | Yes | Yes | 1: EBV (weak: HHV 6, HHV7 and HHV8) | 94.4 | 15.66 |
| 16 | 2011 | 17.4a | m | Osteosarcoma (relapse) | HaploSCT | Day + 8 after HaploSCT, graft rejection | Dxm | Remission | Yes | Yes | – | 110.3 | 3.22 |
| 17 | 2012 | 7.3a | f | AML FAB M0/I | AML BFM 2004 | End of ADxE induction | Dxm | Remission | n.a. | n.a. | – | 317.5 | 12.4 |
| 18 | 2012 | 2.2a | m | AML FAB M5 | AML BFM 2004 | HAM protocol | Dxm | Remission | n.a. | Yes | HHV6, HSV1 | 53.1 | 13.57 |
| 19 | 2013 | 3.1a | m | T-ALL | AEIOP BFM ALL 2009 | 1: protocol IB-ASP 2: protocol III | Dxm | Remission after second episode | n.a. | n.a. | 1: BKV; 2: CMV | 37.4 | 1.8 |
| 20 | 2013 | 1.3a | m | Infant ALL | DLI/stem cell boost after 2nd HaploSCT | Day + 120 after haploSCT, day +12 after stem cell boost, acute GvHD | Dxm, sirolimus | Remission | Yes | Yes | JCV | 190.3 | 1.08 |
| 21 | 2013 | 4.3a | m | T-ALL | AEIOP BFM ALL 2009 | Protocol IB | Dxm, IVIG | Remission | No | Yes | ADV, BKV, HHV6 | 90.2 | n.a. |
| 22 | 2014 | 7.1a | m | Relapse of C-ALL | ALL REZ BFM | Day + 225 after alloBMT, chronic GvHD, colitis | Dxm | Remission | Yes | Yes | ADV, BKV, CMV | 74.4 | 0.42 |
m/f male/female, HR high risk, BFM Berlin-Frankfurt-Münster, MUD matched unrelated donor, BMT bone marrow transplantation, SCT stem cell transplantation, haplo haploidentical, RIC reduced intensity conditioning, VP-16 etoposid, ATG anti-thymocyte globulin, DLI donor lymphocyte infusion, CS-A cyclosporine A, Dxm dexamethasone, n.a. not available, ADV adenovirus, BKV BK virus, CMV cytomegalovirus, EBV Epstein-Barr virus, HHV human herpesvirus, HSV herpes simplex virus, InflA influenza A virus, JCV JC virus, PVB19 parvovirus B19, RSV respiratory syncytial virus
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