Difference between revisions of "Hemophagocytic lymphohistiocytosis"
m (Text replacement - "<span style="background:#EEEE00; padding:3px 6px 3px 6px; border-color:black; border-width:2px; border-style:solid;">Non-randomized</span>" to "style="background-color:#EEEE00"|Non-randomized") |
m (Text replacement - "http://www.ncbi.nlm" to "https://www.ncbi.nlm") |
||
Line 53: | Line 53: | ||
===References=== | ===References=== | ||
− | # Henter JI, Aricò M, Egeler RM, Elinder G, Favara BE, Filipovich AH, Gadner H, Imashuku S, Janka-Schaub G, Komp D, Ladisch S, Webb D. HLH-94: a treatment protocol for hemophagocytic lymphohistiocytosis. HLH study Group of the Histiocyte Society. Med Pediatr Oncol. 1997 May;28(5):342-7. [http://onlinelibrary.wiley.com/doi/10.1002/(SICI)1096-911X(199705)28:5%3C342::AID-MPO3%3E3.0.CO;2-H/abstract;jsessionid=231D9433B983F64065E73251160C4EB5.f04t04 link to original article] '''does not contain protocol''' [ | + | # Henter JI, Aricò M, Egeler RM, Elinder G, Favara BE, Filipovich AH, Gadner H, Imashuku S, Janka-Schaub G, Komp D, Ladisch S, Webb D. HLH-94: a treatment protocol for hemophagocytic lymphohistiocytosis. HLH study Group of the Histiocyte Society. Med Pediatr Oncol. 1997 May;28(5):342-7. [http://onlinelibrary.wiley.com/doi/10.1002/(SICI)1096-911X(199705)28:5%3C342::AID-MPO3%3E3.0.CO;2-H/abstract;jsessionid=231D9433B983F64065E73251160C4EB5.f04t04 link to original article] '''does not contain protocol''' [https://www.ncbi.nlm.nih.gov/pubmed/9121398 PubMed] |
− | ## '''Update:''' Henter JI, Samuelsson-Horne A, Aricò M, Egeler RM, Elinder G, Filipovich AH, Gadner H, Imashuku S, Komp D, Ladisch S, Webb D, Janka G; Histocyte Society. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood. 2002 Oct 1;100(7):2367-73. [http://www.bloodjournal.org/content/100/7/2367 link to original article] '''contains verified protocol''' [ | + | ## '''Update:''' Henter JI, Samuelsson-Horne A, Aricò M, Egeler RM, Elinder G, Filipovich AH, Gadner H, Imashuku S, Komp D, Ladisch S, Webb D, Janka G; Histocyte Society. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood. 2002 Oct 1;100(7):2367-73. [http://www.bloodjournal.org/content/100/7/2367 link to original article] '''contains verified protocol''' [https://www.ncbi.nlm.nih.gov/pubmed/12239144 PubMed] |
− | ## '''Update:''' Trottestam H, Horne A, Aricò M, Egeler RM, Filipovich AH, Gadner H, Imashuku S, Ladisch S, Webb D, Janka G, Henter JI; Histiocyte Society. Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol. Blood. 2011 Oct 27;118(17):4577-84. Epub 2011 Sep 6. [http://www.bloodjournal.org/content/118/17/4577.long link to original article] '''contains verified protocol''' [ | + | ## '''Update:''' Trottestam H, Horne A, Aricò M, Egeler RM, Filipovich AH, Gadner H, Imashuku S, Ladisch S, Webb D, Janka G, Henter JI; Histiocyte Society. Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol. Blood. 2011 Oct 27;118(17):4577-84. Epub 2011 Sep 6. [http://www.bloodjournal.org/content/118/17/4577.long link to original article] '''contains verified protocol''' [https://www.ncbi.nlm.nih.gov/pubmed/21900192 PubMed] |
=Relapsed/Refractory= | =Relapsed/Refractory= | ||
Line 73: | Line 73: | ||
===References=== | ===References=== | ||
− | # Wang Y, Huang W, Hu L, Cen X, Li L, Wang J, Shen J, Wei N, Wang Z. Multicenter study of combination DEP regimen as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis. Blood. 2015 Nov 5;126(19):2186-92. Epub 2015 Aug 19. [http://www.bloodjournal.org/content/126/19/2186.long link to original article] [ | + | # Wang Y, Huang W, Hu L, Cen X, Li L, Wang J, Shen J, Wei N, Wang Z. Multicenter study of combination DEP regimen as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis. Blood. 2015 Nov 5;126(19):2186-92. Epub 2015 Aug 19. [http://www.bloodjournal.org/content/126/19/2186.long link to original article] [https://www.ncbi.nlm.nih.gov/pubmed/26289641 PubMed] |
Revision as of 03:28, 2 December 2016
Use of this site is subject to you reading and agreeing with the terms set forth in the disclaimer.
Is there a regimen missing from this list? Would you like to share a different dosage/schedule or an additional reference for a regimen? Have you noticed an error? Do you have an idea that will help the site grow to better meet your needs and the needs of many others? You are invited to contribute to the site.
4 regimens on this page
4 variants on this page
|
Untreated
HLH-94
back to top |
Regimen
Study | Evidence |
Henter et al. 1997 | Non-randomized |
Initial therapy
- Dexamethasone (Decadron) in a tapering schedule as follows:
- 10 mg/m2/day (single or split doses not specified) for weeks 1 & 2
- 5 mg/m2/day (single or split doses not specified) for weeks 3 & 4
- 2.5 mg/m2/day (single or split doses not specified) for weeks 5 & 6
- 1.25 mg/m2/day (single or split doses not specified) for week 7
- Taper off during week 8 (exact schedule not specified)
- Etoposide (Vepesid) 150 mg/m2 IV as follows:
- Twice per week for weeks 1 & 2
- Once per week for weeks 3 to 8
- Methotrexate (MTX) (dose not specified) IT once per week for weeks 3 to 6 in patients "with progressive neurological symptoms and/or persisting abnormal cerebrospinal fluid findings."
Supportive medications:
- Prophylactic cotrimoxazole (5 mg/kg of trimethoprim equivalent), three times weekly
- An oral antimycotic during initial dexamethasone phase
8-week course; patients without familial or persistent disease then stopped. Patients with familial, persistent, or relapsed disease were treated with up to one year of continuation therapy, until an allogeneic HCT could be performed, as follows:
Continuation therapy
- Dexamethasone (Decadron) 10 mg/m2/day for three days, every two weeks (even weeks)
- Etoposide (Vepesid) 150 mg/m2 IV once every two weeks (odd weeks)
- Cyclosporine A aiming for trough blood levels of 200 μg/L (not clear from the paper whether this is modified or non-modified)
Continuous for up to 52 weeks
References
- Henter JI, Aricò M, Egeler RM, Elinder G, Favara BE, Filipovich AH, Gadner H, Imashuku S, Janka-Schaub G, Komp D, Ladisch S, Webb D. HLH-94: a treatment protocol for hemophagocytic lymphohistiocytosis. HLH study Group of the Histiocyte Society. Med Pediatr Oncol. 1997 May;28(5):342-7. link to original article does not contain protocol PubMed
- Update: Henter JI, Samuelsson-Horne A, Aricò M, Egeler RM, Elinder G, Filipovich AH, Gadner H, Imashuku S, Komp D, Ladisch S, Webb D, Janka G; Histocyte Society. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood. 2002 Oct 1;100(7):2367-73. link to original article contains verified protocol PubMed
- Update: Trottestam H, Horne A, Aricò M, Egeler RM, Filipovich AH, Gadner H, Imashuku S, Ladisch S, Webb D, Janka G, Henter JI; Histiocyte Society. Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol. Blood. 2011 Oct 27;118(17):4577-84. Epub 2011 Sep 6. link to original article contains verified protocol PubMed
Relapsed/Refractory
DEP
back to top |
DEP: Doxorubicin, Etoposide, MethylPrednisolone
Regimen
To be completed
References
- Wang Y, Huang W, Hu L, Cen X, Li L, Wang J, Shen J, Wei N, Wang Z. Multicenter study of combination DEP regimen as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis. Blood. 2015 Nov 5;126(19):2186-92. Epub 2015 Aug 19. link to original article PubMed