Difference between revisions of "Hemophagocytic lymphohistiocytosis"
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=Guidelines= | =Guidelines= | ||
==Histiocyte Society== | ==Histiocyte Society== | ||
− | *'''2019:''' La Rosée P, Horne A, Hines M, von Bahr Greenwood T, Machowicz R, Berliner N, Birndt S, Gil-Herrera J, Girschikofsky M, Jordan MB, Kumar A, van Laar JA, Lachmann G, Nichols KE, Ramanan AV, Wang Y, Wang Z, Janka G, Henter JI. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood. 2019 Jun 6;133(23):2465-2477. Epub 2019 Apr 16. [http://www.bloodjournal.org/content/early/2019/04/16/blood.2018894618.long link to original article] [https:// | + | *'''2019:''' La Rosée P, Horne A, Hines M, von Bahr Greenwood T, Machowicz R, Berliner N, Birndt S, Gil-Herrera J, Girschikofsky M, Jordan MB, Kumar A, van Laar JA, Lachmann G, Nichols KE, Ramanan AV, Wang Y, Wang Z, Janka G, Henter JI. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood. 2019 Jun 6;133(23):2465-2477. Epub 2019 Apr 16. [http://www.bloodjournal.org/content/early/2019/04/16/blood.2018894618.long link to original article] [https://pubmed.ncbi.nlm.nih.gov/30992265 PubMed] |
=="How I treat"== | =="How I treat"== | ||
− | *'''2015:''' Schram AM, Berliner N. How I treat hemophagocytic lymphohistiocytosis in the adult patient. Blood. 2015 May 7;125(19):2908-14. Epub 2015 Mar 10. [http://www.bloodjournal.org/content/125/19/2908.long link to original article] [https:// | + | *'''2015:''' Schram AM, Berliner N. How I treat hemophagocytic lymphohistiocytosis in the adult patient. Blood. 2015 May 7;125(19):2908-14. Epub 2015 Mar 10. [http://www.bloodjournal.org/content/125/19/2908.long link to original article] [https://pubmed.ncbi.nlm.nih.gov/25758828 PubMed] |
=Untreated= | =Untreated= | ||
Line 61: | Line 61: | ||
===References=== | ===References=== | ||
− | # '''HLH-94:''' 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 study Group of the Histiocyte Society. HLH-94: a treatment protocol for hemophagocytic lymphohistiocytosis. Med Pediatr Oncol. 1997 May;28(5):342-7. [https://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:// | + | # '''HLH-94:''' 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 study Group of the Histiocyte Society. HLH-94: a treatment protocol for hemophagocytic lymphohistiocytosis. Med Pediatr Oncol. 1997 May;28(5):342-7. [https://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://pubmed.ncbi.nlm.nih.gov/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''' [https:// | + | ## '''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://pubmed.ncbi.nlm.nih.gov/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''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3208276/ link to PMC article] [https:// | + | ## '''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/pmc/articles/PMC3208276/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/21900192 PubMed] |
==HLH-2004 regimen {{#subobject:cd0245|Regimen=1}}== | ==HLH-2004 regimen {{#subobject:cd0245|Regimen=1}}== | ||
Line 107: | Line 107: | ||
===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] '''contains verified protocol''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4635114/ link to PMC article] [https:// | + | # 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] '''contains verified protocol''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4635114/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/26289641 PubMed] |
[[Category:Hemophagocytic lymphohistiocytosis regimens]] | [[Category:Hemophagocytic lymphohistiocytosis regimens]] | ||
[[Category:Disease-specific pages]] | [[Category:Disease-specific pages]] | ||
[[Category:Histiocytoses]] | [[Category:Histiocytoses]] |
Revision as of 01:44, 25 February 2020
Section editor | |
---|---|
Ronald S. Go, MD Mayo Clinic Rochester, MN |
4 regimens on this page
4 variants on this page
|
Guidelines
Histiocyte Society
- 2019: La Rosée P, Horne A, Hines M, von Bahr Greenwood T, Machowicz R, Berliner N, Birndt S, Gil-Herrera J, Girschikofsky M, Jordan MB, Kumar A, van Laar JA, Lachmann G, Nichols KE, Ramanan AV, Wang Y, Wang Z, Janka G, Henter JI. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood. 2019 Jun 6;133(23):2465-2477. Epub 2019 Apr 16. link to original article PubMed
"How I treat"
- 2015: Schram AM, Berliner N. How I treat hemophagocytic lymphohistiocytosis in the adult patient. Blood. 2015 May 7;125(19):2908-14. Epub 2015 Mar 10. link to original article PubMed
Untreated
HLH-94 regimen
back to top |
Regimen
Study | Evidence |
---|---|
Henter et al. 1997 (HLH-94) | Non-randomized |
Immunosuppressive therapy, initial
- Dexamethasone (Decadron) in a tapering schedule as follows:
- Weeks 1 & 2: 10 mg/m2/day (single or split doses not specified)
- Weeks 3 & 4: 5 mg/m2/day (single or split doses not specified)
- Weeks 5 & 6: 2.5 mg/m2/day (single or split doses not specified)
- Week 7: 1.25 mg/m2/day (single or split doses not specified)
- Taper off during week 8 (exact schedule not specified)
- Etoposide (Vepesid) as follows:
- Weeks 1 & 2: 150 mg/m2 IV twice per week
- Weeks 3 to 8: 150 mg/m2 IV once per week
- 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:
Immunosuppressive therapy, continuation
- Dexamethasone (Decadron) 10 mg/m2/day PO 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 mcg/L (not clear from the paper whether this is modified or non-modified)
Up to 52-week course
References
- HLH-94: 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 study Group of the Histiocyte Society. HLH-94: a treatment protocol for hemophagocytic lymphohistiocytosis. 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 link to PMC article PubMed
HLH-2004 regimen
Relapsed or refractory
DEP
back to top |
DEP: Doxil (Liposomal doxorubicin), Etoposide, MethylPrednisolone
Regimen
Study | Evidence | Efficacy |
---|---|---|
Wang et al. 2015a | Phase II | ORR: 76% |
Note: Liposomal doxorubicin could be repeated, although details are scant in the text.
Chemotherapy
- Pegylated liposomal doxorubicin (Doxil) 25 mg/m2 IV once on day 1
- Etoposide (Vepesid) 100 mg/m2 IV once per day on days 1, 8, 15, 22
- Methylprednisolone (Solumedrol) as follows:
- Days 1 to 3: 15 mg/kg/day
- Days 4 to 6: 2 mg/kg/day
- Days 7 to 10: 1 mg/kg/day
- Days 11 to 14: 0.75 mg/kg/day
- Days 15 to 21: 0.5 mg/kg/day
- Days 22 to 28: 0.4 mg/kg/day
- Days 29 to 35: 0.3 mg/kg/day
- Days 36 to 42: 0.2 mg/kg/day
- Days 43 to 49: 0.1 mg/kg/day
7-week course
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 contains verified protocol link to PMC article PubMed