Difference between revisions of "HLH-2004 for untreated hemophagocytic lymphohistiocytosis (HLH)"

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<includeonly>Source: [[HLH-2004 for untreated hemophagocytic lymphohistiocytosis (HLH)]]
 
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{| class="wikitable" style="float:right; margin-left: 5px;"
 
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|[[#top|back to top]]
 
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|}</includeonly>
 
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<noinclude>
 
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==Regimen in use at==
 
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{{Special:WhatLinksHere/{{PAGENAME}}}}
 
  
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[[Category:Chemotherapy regimens]]
 
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[[Category:Hemophagocytic lymphohistiocytosis regimens]]
 
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[[Category:Disease-specific regimens]]
 
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</noinclude>
 
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===Regimen===
 
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{| border="1" style="text-align:center;" !align="left"
 
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|'''Study'''
 
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|[[Levels_of_Evidence#Evidence|'''Evidence''']]
 
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|-
 
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|[https://www.researchgate.net/profile/Gritta_Janka2/publication/6851976_HLH-2004_Diagnostic_and_therapeutic_guidelines_for_hemophagocytic_lymphohistiocytosis/links/5421309f0cf203f155c61206/HLH-2004-Diagnostic-and-therapeutic-guidelines-for-hemophagocytic-lymphohistiocytosis.pdf Henter et al. 2007 (HLH-2004)]
 
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|style="background-color:#EEEE00"|Non-randomized
 
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|}
 
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====Initial therapy====
 
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*Start allogeneic stem cell transplant (SCT) donor search
 
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*[[Dexamethasone (Decadron)]] in a tapering schedule as follows:
 
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**Weeks 1 & 2: dexamethasone 10 mg/m<sup>2</sup> (route not specified) once per day
 
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**Weeks 3 & 4: dexamethasone 5 mg/m<sup>2</sup> (route not specified) once per day
 
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**Weeks 5 & 6: dexamethasone 2.5 mg/m<sup>2</sup> (route not specified) once per day
 
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**Week 7: dexamethasone 1.25 mg/m<sup>2</sup> (route not specified) once per day
 
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**Week 8: "taper then discontinue"
 
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*[[Etoposide (Vepesid)]] as follows:
 
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**Weeks 1 & 2: etoposide 150 mg/m<sup>2</sup> IV twice per week
 
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**Weeks 3 to 8: etoposide 150 mg/m<sup>2</sup> IV once per week
 
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*[[Cyclosporine modified (Neoral)|Cyclosporine A]] 3 mg/kg/dose by mouth twice per day (total dose per day is 6 mg/kg), if normal kidney function. Then, titrate doses with goal of levels around 200 mcg/L (monoclonal, trough level). It was not clear from the paper whether this is modified or non-modified cyclosporine.
 
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*Intrathecal therapy to "start only if progressive neurological symptoms or if an abnormal CSF has not improved," maximum of 4 doses, as follows:
 
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**Weeks 3 to 6: [[Methotrexate (MTX)]] (dosed by age as listed below) IT once per week. Methotrexate doses by age:
 
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***less than 1 year old: 6 mg each dose
 
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***1 to 2 years old: 8 mg each dose
 
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***2 to 3 years old: 10 mg each dose
 
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***greater than 3 years old: 12 mg each dose
 
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**Weeks 3 to 6: [[Prednisolone (Millipred)|Prednisolone]] IT once per week. Prednisolone doses by age:
 
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***less than 1 year old: 4 mg each dose
 
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***1 to 2 years old: 6 mg each dose
 
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***2 to 3 years old: 8 mg each dose
 
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***greater than 3 years old: 10 mg each dose
 
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====Supportive medications====
 
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*Prophylactic [[Trimethoprim/Sulfamethoxazole_(Bactrim_DS)|cotrimoxazole]] (5 mg/kg of trimethoprim equivalent), three times weekly, start on week 1 and treat onwards
 
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*An oral [[:Category:Antifungals|antimycotic]] from weeks 1 to 9
 
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*[[IvIG]] 0.5 g/kg IV once every 4 weeks
 
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*"Gastroprotection suggested" for weeks 1 to 9
 
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'''8-week course; patients with resolved, non-familial, non-genetically verified disease stopped therapy. Patients with familial, genetically verified, persistent, or reactivation/relapsed disease continued to continuation therapy, until an allogeneic HCT could be performed, as follows:'''
 
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====Continuation therapy====
 
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''Starts during week 9.''
 
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*[[Dexamethasone (Decadron)]] 10 mg/m<sup>2</sup> (route not specified) once per day for three days, every two weeks (on even weeks)
 
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*[[Etoposide (Vepesid)]] 150 mg/m<sup>2</sup> IV once every two weeks (on odd weeks)
 
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*[[Cyclosporine modified (Neoral)|Cyclosporine A]] by mouth twice per day, aiming for trough blood levels of 200 mcg/L (not clear from the paper whether this is modified or non-modified)
 
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*Proceed with allogeneic stem cell transplant (SCT) as soon as an acceptable donor is found, such as:
 
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**HLA-idential record donor
 
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**Matched unrelated donor
 
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**Mismatched unrelated donor
 
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**Family haploidentical donor
 
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'''Used continuously, with allogeneic stem cell transplant done when possible'''
 
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===References===
 
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# Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, Ladisch S, McClain K, Webb D, Winiarski J, Janka G. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007 Feb;48(2):124-31. [https://www.researchgate.net/profile/Gritta_Janka2/publication/6851976_HLH-2004_Diagnostic_and_therapeutic_guidelines_for_hemophagocytic_lymphohistiocytosis/links/5421309f0cf203f155c61206/HLH-2004-Diagnostic-and-therapeutic-guidelines-for-hemophagocytic-lymphohistiocytosis.pdf link to full article at ResearchGate] [http://onlinelibrary.wiley.com/doi/10.1002/pbc.21039/abstract;jsessionid=4E561DF344D9A0163C24F27F34D53ADF.f02t02 link to original article] '''contains verified protocol''' [https://www.researchgate.net/file.PostFileLoader.html?id=557ab7b36307d92f338b457a&assetKey=AS%3A273796236283904%401442289517969 link to study protocol] [https://www.ncbi.nlm.nih.gov/pubmed/16937360 PubMed]
 

Latest revision as of 02:45, 28 February 2023

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