Difference between revisions of "Cold agglutinin disease"
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[[#top|Back to Top]] | [[#top|Back to Top]] | ||
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− | {{#lst: | + | {{#lst:Editorial board transclusions|heme}} |
+ | *''We have moved [[How I Treat]] articles to a dedicated page.'' | ||
{| class="wikitable" style="float:right; margin-right: 5px;" | {| class="wikitable" style="float:right; margin-right: 5px;" | ||
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− | |<div style="background-color: #fee0d1; border: 1px solid #808000; padding: 5px; {{border-radius|16px}}" align="right"><font size="4"><b>{{#ask: [[-Has subobject::{{FULLPAGENAME}}]] | + | |<div style="background-color: #fee0d1; border: 1px solid #808000; padding: 5px; {{border-radius|16px}}" align="right"><font size="4"><b>{{#ask: [[-Has subobject::{{FULLPAGENAME}}]] |?Regimen |limit=10000|format=sum}} [[Tutorial#Regimens|regimens]] on this page</b></font></div> |
− | <div style="background-color: #deebf6; border: 1px solid #808000; padding: 5px; {{border-radius|16px}}"><font size="4"><b>{{#ask: [[-Has subobject::{{FULLPAGENAME}}]] | + | <div style="background-color: #deebf6; border: 1px solid #808000; padding: 5px; {{border-radius|16px}}"><font size="4"><b>{{#ask: [[-Has subobject::{{FULLPAGENAME}}]] |?Variant |limit=10000|format=sum}} [[Tutorial#Variants|variants]] on this page</b></font></div> |
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{{TOC limit|limit=3}} | {{TOC limit|limit=3}} | ||
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=All lines of therapy= | =All lines of therapy= | ||
==Bendamustine & Rituximab (BR) {{#subobject:0fde6d|Regimen=1}}== | ==Bendamustine & Rituximab (BR) {{#subobject:0fde6d|Regimen=1}}== | ||
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<div class="toccolours" style="background-color:#eeeeee"> | <div class="toccolours" style="background-color:#eeeeee"> | ||
===Regimen {{#subobject:47595c|Variant=1}}=== | ===Regimen {{#subobject:47595c|Variant=1}}=== | ||
− | {| class="wikitable" style="width: | + | {| class="wikitable sortable" style="width: 60%; text-align:center;" |
− | ! style="width: | + | !style="width: 33%"|Study |
− | ! style="width: | + | !style="width: 33%"|Dates of enrollment |
+ | !style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
|- | |- | ||
− | |[ | + | |[https://doi.org/10.1182/blood-2017-04-778175 Berentsen et al. 2017 (CAD5)] |
+ | |2012-10-01 to 2015-12-31 | ||
| style="background-color:#91cf61" |Phase 2 | | style="background-color:#91cf61" |Phase 2 | ||
|- | |- | ||
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</div></div> | </div></div> | ||
===References=== | ===References=== | ||
− | #'''CAD5:''' Berentsen S, Randen U, Oksman M, Birgens H, Tvedt THA, Dalgaard J, Galteland E, Haukås E, Brudevold R, Sørbø JH, Næss IA, Malecka A, Tjønnfjord GE. Bendamustine plus rituximab for chronic cold agglutinin disease: results of a Nordic prospective multicenter trial. Blood. 2017 Jul 27;130(4):537-541. Epub 2017 May 22. [ | + | #'''CAD5:''' Berentsen S, Randen U, Oksman M, Birgens H, Tvedt THA, Dalgaard J, Galteland E, Haukås E, Brudevold R, Sørbø JH, Næss IA, Malecka A, Tjønnfjord GE. Bendamustine plus rituximab for chronic cold agglutinin disease: results of a Nordic prospective multicenter trial. Blood. 2017 Jul 27;130(4):537-541. Epub 2017 May 22. [https://doi.org/10.1182/blood-2017-04-778175 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/28533306/ PubMed] [https://clinicaltrials.gov/study/NCT02689986 NCT02689986] |
==Eculizumab monotherapy {{#subobject:08ef07|Regimen=1}}== | ==Eculizumab monotherapy {{#subobject:08ef07|Regimen=1}}== | ||
<div class="toccolours" style="background-color:#eeeeee"> | <div class="toccolours" style="background-color:#eeeeee"> | ||
===Regimen {{#subobject:95a0a9|Variant=1}}=== | ===Regimen {{#subobject:95a0a9|Variant=1}}=== | ||
− | {| class="wikitable" style="width: | + | {| class="wikitable sortable" style="width: 60%; text-align:center;" |
− | ! style="width: | + | !style="width: 33%"|Study |
− | ! style="width: | + | !style="width: 33%"|Dates of enrollment |
+ | !style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
|- | |- | ||
|[https://www.ncbi.nlm.nih.gov/pmc/articles/pmc6177646/ Röth et al. 2018 (DECADE)] | |[https://www.ncbi.nlm.nih.gov/pmc/articles/pmc6177646/ Röth et al. 2018 (DECADE)] | ||
− | | style="background-color:#ffffbe" |Phase 2, | + | |2011-01-06 to 2014-01-14 |
+ | | style="background-color:#ffffbe" |Phase 2, fewer than 20 pts | ||
|- | |- | ||
|} | |} | ||
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====Immunosuppressive therapy==== | ====Immunosuppressive therapy==== | ||
*[[Eculizumab (Soliris)]] as follows: | *[[Eculizumab (Soliris)]] as follows: | ||
− | ** | + | **Cycles 1 to 4: 600 mg IV once on day 1 |
− | **Cycles | + | **Cycles 5 to 15: 900 mg IV once on day 1 |
====Supportive therapy==== | ====Supportive therapy==== | ||
*Patients were vaccinated against ''Neisseria meningitidis'' or received "appropriate antibiotics" if vaccination occurred within 14 days of the first dose | *Patients were vaccinated against ''Neisseria meningitidis'' or received "appropriate antibiotics" if vaccination occurred within 14 days of the first dose | ||
− | ''' | + | '''7-day cycle for 4 cycles, then 14-day cycle for 11 cycles (26 weeks total)''' |
</div></div> | </div></div> | ||
===References=== | ===References=== | ||
− | <!-- # '''Abstract:''' Alexander Röth, MD, Martin Bommer, MD, Andreas Hüttmann, MD, Dörte Herich-Terhürne, Nils Kuklik, PhD, Lenz Veronika, Hubert Schrezenmeier, MD and Ulrich Dührsen, MD. Complement Inhibition with Eculizumab in Patients with Cold Agglutinin Disease (CAD): Results from a Prospective Phase II Trial (DECADE Trial). ASH Annual Meeting 2015 Abstract 274 | + | <!-- # '''Abstract:''' Alexander Röth, MD, Martin Bommer, MD, Andreas Hüttmann, MD, Dörte Herich-Terhürne, Nils Kuklik, PhD, Lenz Veronika, Hubert Schrezenmeier, MD and Ulrich Dührsen, MD. Complement Inhibition with Eculizumab in Patients with Cold Agglutinin Disease (CAD): Results from a Prospective Phase II Trial (DECADE Trial). ASH Annual Meeting 2015 Abstract 274 --> |
− | #'''DECADE:''' Röth A, Bommer M, Hüttmann A, Herich-Terhürne D, Kuklik N, Rekowski J, Lenz V, Schrezenmeier H, Dührsen U. Eculizumab in cold agglutinin disease (DECADE): an open-label, prospective, bicentric, nonrandomized phase 2 trial. Blood Adv. 2018 Oct 9;2(19):2543-2549. [https://doi.org/10.1182/bloodadvances.2018024190 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/pmc6177646/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/30291112/ PubMed] [https://clinicaltrials.gov/ | + | #'''DECADE:''' Röth A, Bommer M, Hüttmann A, Herich-Terhürne D, Kuklik N, Rekowski J, Lenz V, Schrezenmeier H, Dührsen U. Eculizumab in cold agglutinin disease (DECADE): an open-label, prospective, bicentric, nonrandomized phase 2 trial. Blood Adv. 2018 Oct 9;2(19):2543-2549. [https://doi.org/10.1182/bloodadvances.2018024190 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/pmc6177646/ link to PMC article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/30291112/ PubMed] [https://clinicaltrials.gov/study/NCT01303952 NCT01303952] |
+ | |||
==Fludarabine & Rituximab (FR) {{#subobject:96c221|Regimen=1}}== | ==Fludarabine & Rituximab (FR) {{#subobject:96c221|Regimen=1}}== | ||
FR: '''<u>F</u>'''ludarabine & '''<u>R</u>'''ituximab | FR: '''<u>F</u>'''ludarabine & '''<u>R</u>'''ituximab | ||
<div class="toccolours" style="background-color:#eeeeee"> | <div class="toccolours" style="background-color:#eeeeee"> | ||
===Regimen {{#subobject:645885|Variant=1}}=== | ===Regimen {{#subobject:645885|Variant=1}}=== | ||
− | {| class="wikitable" style="width: | + | {| class="wikitable sortable" style="width: 80%; text-align:center;" |
− | ! style="width: | + | !style="width: 25%"|Study |
− | ! style="width: | + | !style="width: 25%"|Dates of enrollment |
− | ! style="width: | + | !style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] |
+ | !style="width: 25%"|[[Levels_of_Evidence#Efficacy|Efficacy]] | ||
|- | |- | ||
− | |[ | + | |[https://doi.org/10.1182/blood-2010-06-288647 Berentsen et al. 2010] |
+ | |2005-02-01 to 2009-10-31 | ||
| style="background-color:#91cf61" |Phase 2 | | style="background-color:#91cf61" |Phase 2 | ||
| style="background-color:#e0ecf4" |ORR: 76% | | style="background-color:#e0ecf4" |ORR: 76% | ||
Line 83: | Line 88: | ||
</div></div> | </div></div> | ||
===References=== | ===References=== | ||
− | #Berentsen S, Randen U, Vågan AM, Hjorth-Hansen H, Vik A, Dalgaard J, Jacobsen EM, Thoresen AS, Beiske K, Tjønnfjord GE. High response rate and durable remissions following fludarabine and rituximab combination therapy for chronic cold agglutinin disease. Blood. 2010 Oct 28;116(17):3180-4. Epub 2010 Jul 15. [ | + | #Berentsen S, Randen U, Vågan AM, Hjorth-Hansen H, Vik A, Dalgaard J, Jacobsen EM, Thoresen AS, Beiske K, Tjønnfjord GE. High response rate and durable remissions following fludarabine and rituximab combination therapy for chronic cold agglutinin disease. Blood. 2010 Oct 28;116(17):3180-4. Epub 2010 Jul 15. [https://doi.org/10.1182/blood-2010-06-288647 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/20634373/ PubMed] [https://clinicaltrials.gov/study/NCT00373594 NCT00373594] |
==Rituximab monotherapy {{#subobject:65c6f1|Regimen=1}}== | ==Rituximab monotherapy {{#subobject:65c6f1|Regimen=1}}== | ||
<div class="toccolours" style="background-color:#eeeeee"> | <div class="toccolours" style="background-color:#eeeeee"> | ||
===Regimen {{#subobject:372fe5|Variant=1}}=== | ===Regimen {{#subobject:372fe5|Variant=1}}=== | ||
− | {| class="wikitable" style="width: | + | {| class="wikitable sortable" style="width: 80%; text-align:center;" |
− | ! style="width: | + | !style="width: 25%"|Study |
− | ! style="width: | + | !style="width: 25%"|Dates of enrollment |
− | ! style="width: | + | !style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] |
+ | !style="width: 25%"|[[Levels_of_Evidence#Efficacy|Efficacy]] | ||
|- | |- | ||
− | |[ | + | |[https://doi.org/10.1182/blood-2003-10-3597 Berentsen et al. 2003] |
+ | |2001-10-01 to 2003-05-01 | ||
| style="background-color:#91cf61" |Phase 2 | | style="background-color:#91cf61" |Phase 2 | ||
| style="background-color:#9ebcda" |ORR: 54% | | style="background-color:#9ebcda" |ORR: 54% | ||
|- | |- | ||
|[https://doi.org/10.1080/10428190500286481 Schöllkopf et al. 2006] | |[https://doi.org/10.1080/10428190500286481 Schöllkopf et al. 2006] | ||
+ | |2002-10 to 2003-04 | ||
| style="background-color:#91cf61" |Phase 2 | | style="background-color:#91cf61" |Phase 2 | ||
| style="background-color:#8c96c6" |ORR: 45% | | style="background-color:#8c96c6" |ORR: 45% | ||
Line 108: | Line 116: | ||
</div></div> | </div></div> | ||
===References=== | ===References=== | ||
− | #Berentsen S, Ulvestad E, Gjertsen BT, Hjorth-Hansen H, Langholm R, Knutsen H, Ghanima W, Shammas FV, Tjønnfjord GE. Rituximab for primary chronic cold agglutinin disease: a prospective study of 37 courses of therapy in 27 patients. Blood. 2004 Apr 15;103(8):2925-8. Epub 2003 Dec 30. [ | + | #Berentsen S, Ulvestad E, Gjertsen BT, Hjorth-Hansen H, Langholm R, Knutsen H, Ghanima W, Shammas FV, Tjønnfjord GE. Rituximab for primary chronic cold agglutinin disease: a prospective study of 37 courses of therapy in 27 patients. Blood. 2004 Apr 15;103(8):2925-8. Epub 2003 Dec 30. [https://doi.org/10.1182/blood-2003-10-3597 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/15070665/ PubMed] |
#Schöllkopf C, Kjeldsen L, Bjerrum OW, Mourits-Andersen HT, Nielsen JL, Christensen BE, Jensen BA, Pedersen BB, Taaning EB, Klausen TW, Birgens H. Rituximab in chronic cold agglutinin disease: a prospective study of 20 patients. Leuk Lymphoma. 2006 Feb;47(2):253-60. [https://doi.org/10.1080/10428190500286481 link to original article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/16321854/ PubMed] | #Schöllkopf C, Kjeldsen L, Bjerrum OW, Mourits-Andersen HT, Nielsen JL, Christensen BE, Jensen BA, Pedersen BB, Taaning EB, Klausen TW, Birgens H. Rituximab in chronic cold agglutinin disease: a prospective study of 20 patients. Leuk Lymphoma. 2006 Feb;47(2):253-60. [https://doi.org/10.1080/10428190500286481 link to original article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/16321854/ PubMed] | ||
==Sutimlimab monotherapy== | ==Sutimlimab monotherapy== | ||
<div class="toccolours" style="background-color:#eeeeee"> | <div class="toccolours" style="background-color:#eeeeee"> | ||
===Regimen=== | ===Regimen=== | ||
− | {| class="wikitable" style="width: | + | {| class="wikitable sortable" style="width: 100%; text-align:center;" |
− | ! style="width: | + | !style="width: 20%"|Study |
− | ! style="width: | + | !style="width: 20%"|Dates of enrollment |
− | ! style="width: | + | !style="width: 20%"|[[Levels_of_Evidence#Evidence|Evidence]] |
+ | !style="width: 20%"|Comparator | ||
+ | !style="width: 20%"|[[Levels_of_Evidence#Comparative_efficacy|Comparative Efficacy]] | ||
|- | |- | ||
|[https://doi.org/10.1056/nejmoa2027760 Röth et al. 2021 (CARDINAL)] | |[https://doi.org/10.1056/nejmoa2027760 Röth et al. 2021 (CARDINAL)] | ||
− | | style="background-color:#91cf61" |Phase 2 | + | |NR |
+ | | style="background-color:#91cf61" |Phase 2 (RT) | ||
+ | | style="background-color:#d3d3d3" | | ||
| style="background-color:#9ebcda" |ORR: 54% | | style="background-color:#9ebcda" |ORR: 54% | ||
+ | |- | ||
+ | |[https://doi.org/10.1182/blood.2021014955 Röth et al. 2022 (CADENZA)] | ||
+ | |2018-03-06 to 2020-09-29 | ||
+ | | style="background-color:#1a9851" |Phase 3 (E-esc) | ||
+ | |Placebo | ||
+ | | style="background-color:#1a9850" |Superior composite primary endpoint | ||
|- | |- | ||
|} | |} | ||
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====Immunosuppressive therapy==== | ====Immunosuppressive therapy==== | ||
*[[Sutimlimab (Enjaymo)]] by the following weight-based criteria: | *[[Sutimlimab (Enjaymo)]] by the following weight-based criteria: | ||
− | ** | + | **Less than 75 kg: 6500 mg IV once on day 1 |
− | ** | + | **75 kg or more: 7500 mg IV once on day 1 |
− | '''7-day | + | '''7-day course, then 14-day cycle for 12 cycles (26 weeks)''' |
</div></div> | </div></div> | ||
+ | |||
===References=== | ===References=== | ||
− | #'''CARDINAL:''' Röth A, Barcellini W, D'Sa S, Miyakawa Y, Broome CM, Michel M, Kuter DJ, Jilma B, Tvedt THA, Fruebis J, Jiang X, Lin S, Reuter C, Morales-Arias J, Hobbs W, Berentsen S. Sutimlimab in Cold Agglutinin Disease. N Engl J Med. 2021 Apr 8;384(14):1323-1334. [https://doi.org/10.1056/nejmoa2027760 link to original article] [https://pubmed.ncbi.nlm.nih.gov/33826820/ PubMed] [https://clinicaltrials.gov/ | + | #'''CARDINAL:''' Röth A, Barcellini W, D'Sa S, Miyakawa Y, Broome CM, Michel M, Kuter DJ, Jilma B, Tvedt THA, Fruebis J, Jiang X, Lin S, Reuter C, Morales-Arias J, Hobbs W, Berentsen S. Sutimlimab in Cold Agglutinin Disease. N Engl J Med. 2021 Apr 8;384(14):1323-1334. [https://doi.org/10.1056/nejmoa2027760 link to original article] [https://pubmed.ncbi.nlm.nih.gov/33826820/ PubMed] [https://clinicaltrials.gov/study/NCT03347396 NCT03347396] |
− | #'''CADENZA:''' Röth A, Berentsen S, Barcellini W, D'Sa S, Jilma B, Michel M, Weitz IC, Yamaguchi M, Nishimura JI, Vos JMI, Storek M, Wong N, Patel P, Jiang X, Vagge DS, Wardęcki M, Shafer F, Lee M, Broome CM. Sutimlimab in patients with cold agglutinin disease: results of the randomized placebo-controlled phase 3 CADENZA trial. Blood. 2022 Sep 1;140(9):980-991. [https://doi.org/10.1182/blood.2021014955 link to original article] [https://pubmed.ncbi.nlm.nih.gov/35687757/ PubMed] [https://clinicaltrials.gov/ | + | #'''CADENZA:''' Röth A, Berentsen S, Barcellini W, D'Sa S, Jilma B, Michel M, Weitz IC, Yamaguchi M, Nishimura JI, Vos JMI, Storek M, Wong N, Patel P, Jiang X, Vagge DS, Wardęcki M, Shafer F, Lee M, Broome CM. Sutimlimab in patients with cold agglutinin disease: results of the randomized placebo-controlled phase 3 CADENZA trial. Blood. 2022 Sep 1;140(9):980-991. [https://doi.org/10.1182/blood.2021014955 link to original article] [https://pubmed.ncbi.nlm.nih.gov/35687757/ PubMed] [https://clinicaltrials.gov/study/NCT03347422 NCT03347422] |
− | + | ||
− | |||
[[Category:Cold agglutinin disease regimens]] | [[Category:Cold agglutinin disease regimens]] | ||
[[Category:Disease-specific pages]] | [[Category:Disease-specific pages]] | ||
[[Category:Complementopathies]] | [[Category:Complementopathies]] | ||
[[Category:Hemolytic process]] | [[Category:Hemolytic process]] |
Latest revision as of 18:19, 27 June 2024
Section editor | |
---|---|
Benjamin Tillman, MD Vanderbilt University Nashville, TN, USA |
- We have moved How I Treat articles to a dedicated page.
4 regimens on this page
4 variants on this page
|
All lines of therapy
Bendamustine & Rituximab (BR)
BR: Bendamustine, Rituximab
Regimen
Study | Dates of enrollment | Evidence |
---|---|---|
Berentsen et al. 2017 (CAD5) | 2012-10-01 to 2015-12-31 | Phase 2 |
Chemotherapy
- Bendamustine 90 mg/m2 IV once per day on days 1 & 2
Targeted therapy
- Rituximab (Rituxan) 375 mg/m2 IV once on day 1
28-day cycle for 4 cycles
References
- CAD5: Berentsen S, Randen U, Oksman M, Birgens H, Tvedt THA, Dalgaard J, Galteland E, Haukås E, Brudevold R, Sørbø JH, Næss IA, Malecka A, Tjønnfjord GE. Bendamustine plus rituximab for chronic cold agglutinin disease: results of a Nordic prospective multicenter trial. Blood. 2017 Jul 27;130(4):537-541. Epub 2017 May 22. link to original article contains dosing details in manuscript PubMed NCT02689986
Eculizumab monotherapy
Regimen
Study | Dates of enrollment | Evidence |
---|---|---|
Röth et al. 2018 (DECADE) | 2011-01-06 to 2014-01-14 | Phase 2, fewer than 20 pts |
Note: Eligible patients had symptomatic cold agglutinin-mediated hemolysis with a serum lactate dehydrogenase (LDH) level greater than or equal to 2 x upper limit of normal and a cold agglutinin titer greater than 1:64 at 4°C.
Immunosuppressive therapy
- Eculizumab (Soliris) as follows:
- Cycles 1 to 4: 600 mg IV once on day 1
- Cycles 5 to 15: 900 mg IV once on day 1
Supportive therapy
- Patients were vaccinated against Neisseria meningitidis or received "appropriate antibiotics" if vaccination occurred within 14 days of the first dose
7-day cycle for 4 cycles, then 14-day cycle for 11 cycles (26 weeks total)
References
- DECADE: Röth A, Bommer M, Hüttmann A, Herich-Terhürne D, Kuklik N, Rekowski J, Lenz V, Schrezenmeier H, Dührsen U. Eculizumab in cold agglutinin disease (DECADE): an open-label, prospective, bicentric, nonrandomized phase 2 trial. Blood Adv. 2018 Oct 9;2(19):2543-2549. link to original article link to PMC article contains dosing details in manuscript PubMed NCT01303952
Fludarabine & Rituximab (FR)
FR: Fludarabine & Rituximab
Regimen
Study | Dates of enrollment | Evidence | Efficacy |
---|---|---|---|
Berentsen et al. 2010 | 2005-02-01 to 2009-10-31 | Phase 2 | ORR: 76% |
Note: Eligible patients had to be diagnosed with cold agglutinin disease and require therapy because of anemia, substantial cold-induced circulatory symptoms, or both.
Chemotherapy
- Fludarabine (Fludara) 40 mg/m2 PO once per day on days 1 to 5
Targeted therapy
- Rituximab (Rituxan) 375 mg/m2 IV once on day 1
28-day cycle for 4 cycles
References
- Berentsen S, Randen U, Vågan AM, Hjorth-Hansen H, Vik A, Dalgaard J, Jacobsen EM, Thoresen AS, Beiske K, Tjønnfjord GE. High response rate and durable remissions following fludarabine and rituximab combination therapy for chronic cold agglutinin disease. Blood. 2010 Oct 28;116(17):3180-4. Epub 2010 Jul 15. link to original article contains dosing details in manuscript PubMed NCT00373594
Rituximab monotherapy
Regimen
Study | Dates of enrollment | Evidence | Efficacy |
---|---|---|---|
Berentsen et al. 2003 | 2001-10-01 to 2003-05-01 | Phase 2 | ORR: 54% |
Schöllkopf et al. 2006 | 2002-10 to 2003-04 | Phase 2 | ORR: 45% |
Note: Patients in Berentsen et al. 2003 were required to have cold agglutinin disease, as defined by the combination of chronic hemolysis and a cold agglutinin titer of 1:64 or higher, and a typical pattern for the direct antiglobulin test (DAT).
Immunosuppressive therapy
- Rituximab (Rituxan) 375 mg/m2 IV once per day on days 1, 8, 15, 22
28-day course
References
- Berentsen S, Ulvestad E, Gjertsen BT, Hjorth-Hansen H, Langholm R, Knutsen H, Ghanima W, Shammas FV, Tjønnfjord GE. Rituximab for primary chronic cold agglutinin disease: a prospective study of 37 courses of therapy in 27 patients. Blood. 2004 Apr 15;103(8):2925-8. Epub 2003 Dec 30. link to original article contains dosing details in manuscript PubMed
- Schöllkopf C, Kjeldsen L, Bjerrum OW, Mourits-Andersen HT, Nielsen JL, Christensen BE, Jensen BA, Pedersen BB, Taaning EB, Klausen TW, Birgens H. Rituximab in chronic cold agglutinin disease: a prospective study of 20 patients. Leuk Lymphoma. 2006 Feb;47(2):253-60. link to original article contains dosing details in abstract PubMed
Sutimlimab monotherapy
Regimen
Study | Dates of enrollment | Evidence | Comparator | Comparative Efficacy |
---|---|---|---|---|
Röth et al. 2021 (CARDINAL) | NR | Phase 2 (RT) | ORR: 54% | |
Röth et al. 2022 (CADENZA) | 2018-03-06 to 2020-09-29 | Phase 3 (E-esc) | Placebo | Superior composite primary endpoint |
Immunosuppressive therapy
- Sutimlimab (Enjaymo) by the following weight-based criteria:
- Less than 75 kg: 6500 mg IV once on day 1
- 75 kg or more: 7500 mg IV once on day 1
7-day course, then 14-day cycle for 12 cycles (26 weeks)
References
- CARDINAL: Röth A, Barcellini W, D'Sa S, Miyakawa Y, Broome CM, Michel M, Kuter DJ, Jilma B, Tvedt THA, Fruebis J, Jiang X, Lin S, Reuter C, Morales-Arias J, Hobbs W, Berentsen S. Sutimlimab in Cold Agglutinin Disease. N Engl J Med. 2021 Apr 8;384(14):1323-1334. link to original article PubMed NCT03347396
- CADENZA: Röth A, Berentsen S, Barcellini W, D'Sa S, Jilma B, Michel M, Weitz IC, Yamaguchi M, Nishimura JI, Vos JMI, Storek M, Wong N, Patel P, Jiang X, Vagge DS, Wardęcki M, Shafer F, Lee M, Broome CM. Sutimlimab in patients with cold agglutinin disease: results of the randomized placebo-controlled phase 3 CADENZA trial. Blood. 2022 Sep 1;140(9):980-991. link to original article PubMed NCT03347422