A comprehensive analysis of adult patients with secondary hemophagocytic lymphohistiocytosis : a prospective cohort study
Secondary hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal condition with various underlying disorders in adult patients and is diagnosed based on the HLH-2004 criteria, which were established based on experience in pediatric patients. However, few studies have prospectively evaluated the treatment outcomes and diagnostic performance of HLH criteria in adult patients with secondary HLH. Thus, we performed a single-center, prospective cohort study of adult patients with suspected HLH, and we analyzed treatment outcomes of patients enrolled between 2017 and 2019 as an interim analysis ( ClinicalTrials.gov Identifier: NCT03117010). Of the 73 patients with suspected HLH, 70 patients completed the evaluation for ≥ 7 of the HLH-2004 criteria, and 55 patients were diagnosed with HLH (55/73, 75%). Although serum ferritin and fever had a sensitivity of more than 90%, both had exceptionally low specificity, whereas soluble CD25 had a sensitivity of more than 90% and specificity of 80%. Forty patients with malignancy-associated HLH had B cell (n = 19) or T- or NK-cell (n = 21) lymphoid malignancy, whereas 15 patients had non-malignant disorders. Non-malignancy-associated HLH had greater than 90% 1-year overall survival (OS) after diagnosis of HLH, whereas that for malignancy-associated HLH was less than 40%. In conclusion, our study showed promising treatment outcomes for patients enrolled in our prospective cohort study, and prospectively demonstrated the diagnostic performance of the HLH-2004 criteria in adult patients with suspected HLH. Given that lymphoma was the most common cause of HLH in adults, thorough evaluation for lymphoma should be performed in adults with suspected HLH.
| Medienart: |
E-Artikel |
|---|
| Erscheinungsjahr: |
2020 |
|---|---|
| Erschienen: |
2020 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:99 |
|---|---|
| Enthalten in: |
Annals of hematology - 99(2020), 9 vom: 21. Sept., Seite 2095-2104 |
| Sprache: |
Englisch |
|---|
| Beteiligte Personen: |
Yoon, Sang Eun [VerfasserIn] |
|---|
| Links: |
|---|
| Anmerkungen: |
Date Completed 24.08.2020 Date Revised 24.08.2020 published: Print-Electronic ClinicalTrials.gov: NCT03117010 Citation Status MEDLINE |
|---|
| doi: |
10.1007/s00277-020-04083-6 |
|---|
| funding: |
|
|---|---|
| Förderinstitution / Projekttitel: |
|
| PPN (Katalog-ID): |
NLM310230373 |
|---|
| LEADER | 01000naa a22002652 4500 | ||
|---|---|---|---|
| 001 | NLM310230373 | ||
| 003 | DE-627 | ||
| 005 | 20231225135638.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 231225s2020 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.1007/s00277-020-04083-6 |2 doi | |
| 028 | 5 | 2 | |a pubmed24n1034.xml |
| 035 | |a (DE-627)NLM310230373 | ||
| 035 | |a (NLM)32440790 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
| 041 | |a eng | ||
| 100 | 1 | |a Yoon, Sang Eun |e verfasserin |4 aut | |
| 245 | 1 | 2 | |a A comprehensive analysis of adult patients with secondary hemophagocytic lymphohistiocytosis |b a prospective cohort study |
| 264 | 1 | |c 2020 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a ƒaComputermedien |b c |2 rdamedia | ||
| 338 | |a ƒa Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Date Completed 24.08.2020 | ||
| 500 | |a Date Revised 24.08.2020 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a ClinicalTrials.gov: NCT03117010 | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Secondary hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal condition with various underlying disorders in adult patients and is diagnosed based on the HLH-2004 criteria, which were established based on experience in pediatric patients. However, few studies have prospectively evaluated the treatment outcomes and diagnostic performance of HLH criteria in adult patients with secondary HLH. Thus, we performed a single-center, prospective cohort study of adult patients with suspected HLH, and we analyzed treatment outcomes of patients enrolled between 2017 and 2019 as an interim analysis ( ClinicalTrials.gov Identifier: NCT03117010). Of the 73 patients with suspected HLH, 70 patients completed the evaluation for ≥ 7 of the HLH-2004 criteria, and 55 patients were diagnosed with HLH (55/73, 75%). Although serum ferritin and fever had a sensitivity of more than 90%, both had exceptionally low specificity, whereas soluble CD25 had a sensitivity of more than 90% and specificity of 80%. Forty patients with malignancy-associated HLH had B cell (n = 19) or T- or NK-cell (n = 21) lymphoid malignancy, whereas 15 patients had non-malignant disorders. Non-malignancy-associated HLH had greater than 90% 1-year overall survival (OS) after diagnosis of HLH, whereas that for malignancy-associated HLH was less than 40%. In conclusion, our study showed promising treatment outcomes for patients enrolled in our prospective cohort study, and prospectively demonstrated the diagnostic performance of the HLH-2004 criteria in adult patients with suspected HLH. Given that lymphoma was the most common cause of HLH in adults, thorough evaluation for lymphoma should be performed in adults with suspected HLH | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Adult | |
| 650 | 4 | |a H-score | |
| 650 | 4 | |a HLH-2004 | |
| 650 | 4 | |a Hemophagocytic lymphohistiocytosis | |
| 650 | 4 | |a Lymphoma | |
| 650 | 7 | |a Antineoplastic Agents, Hormonal |2 NLM | |
| 650 | 7 | |a Vincristine |2 NLM | |
| 650 | 7 | |a 5J49Q6B70F |2 NLM | |
| 650 | 7 | |a Dexamethasone |2 NLM | |
| 650 | 7 | |a 7S5I7G3JQL |2 NLM | |
| 650 | 7 | |a Doxorubicin |2 NLM | |
| 650 | 7 | |a 80168379AG |2 NLM | |
| 650 | 7 | |a Cyclophosphamide |2 NLM | |
| 650 | 7 | |a 8N3DW7272P |2 NLM | |
| 650 | 7 | |a Prednisone |2 NLM | |
| 650 | 7 | |a VB0R961HZT |2 NLM | |
| 700 | 1 | |a Eun, Yeonghee |e verfasserin |4 aut | |
| 700 | 1 | |a Huh, Kyungmin |e verfasserin |4 aut | |
| 700 | 1 | |a Chung, Chi Ryang |e verfasserin |4 aut | |
| 700 | 1 | |a Yoo, In Young |e verfasserin |4 aut | |
| 700 | 1 | |a Cho, Junhun |e verfasserin |4 aut | |
| 700 | 1 | |a Cho, Duck |e verfasserin |4 aut | |
| 700 | 1 | |a Ko, Young Hyeh |e verfasserin |4 aut | |
| 700 | 1 | |a Park, Silvia |e verfasserin |4 aut | |
| 700 | 1 | |a Kim, Won Seog |e verfasserin |4 aut | |
| 700 | 1 | |a Kim, Seok Jin |e verfasserin |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t Annals of hematology |d 1991 |g 99(2020), 9 vom: 21. Sept., Seite 2095-2104 |w (DE-627)NLM012918768 |x 1432-0584 |7 nnns |
| 773 | 1 | 8 | |g volume:99 |g year:2020 |g number:9 |g day:21 |g month:09 |g pages:2095-2104 |
| 856 | 4 | 0 | |u http://dx.doi.org/10.1007/s00277-020-04083-6 |3 Volltext |
| 912 | |a GBV_USEFLAG_A | ||
| 912 | |a GBV_NLM | ||
| 951 | |a AR | ||
| 952 | |d 99 |j 2020 |e 9 |b 21 |c 09 |h 2095-2104 | ||