T-cell based immunotherapy-associated cytokine release syndrome
Synonyms: T-cell based immunotherapy-associated CRS
- Type
- Particular clinical situation in a disease or syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare systemic condition affecting patients undergoing chimeric antigen receptor (CAR) T-cell therapy and characterized by a systemic inflammatory response due to massive activation of leukocytes with subsequent cytokine release. It can present with a variety of signs and symptoms ranging from mild, flu-like symptoms (such as fever, fatigue, headache, rash, arthralgia, and myalgia) to severe life-threatening manifestations including vascular leakage, disseminated intravascular coagulation, shock, and multiple organ failure. Respiratory manifestations are common and range from cough and tachypnea to acute respiratory distress syndrome (ARDS).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | T-cell based immunotherapy-associated cytokine release syndrome | T-cell based immunotherapy-associated CRS |
| fr | Syndrome de libération de cytokines lié à l'immunothérapie liée aux lymphocytes T | Syndrome de relargage cytokinique lié à l'immunothérapie liée aux lymphocytes T |
| de | Zytokin-Freisetzungssyndrom nach T-Zell-basierter Immuntherapie | T-Zell-basierte Immuntherapie-assoziiertes CRS |
| es | Síndrome de liberación de citocinas asociado a inmunoterapia basada en células T | SLC asociado a inmunoterapia basada en células T |
| it | Sindrome da rilascio di citochine associata all'immunoterapia basata sulle cellule T | CRS associata all'immunoterapia basata sulle cellule T |
| nl | 'Cytokine release'-syndroom door immuuntherapie met T-cellen | CRS door immuuntherapie met T-cellen |
| pt | Síndrome de libertação de citoquinas associada a terapia com células T CAR | |
| pl | Zespół uwalniania cytokin związany z terapią limfocytami T CAR | = en |
| cs | Syndrom uvolnění cytokinů spojený s CAR T-buněčnou terapií | = en |
| uk | Синдром вивільнення цитокінів, асоційований з терапією Т-клітинною терапією CAR | CRS, асоційована з CAR Т-клітинною терапією · Синдром вивільнення цитокінів, асоційований з терапією Т-клітинними рецепторами до химерних антигенів |
| zh | CAR T细胞治疗相关的细胞因子释放综合征 | 嵌合抗原受体-T细胞治疗相关的细胞因子释放综合征 |
| tr | CAR T hücre terapisi-ilişkili sitokin salınım sendromu | CAR T hücresi terapisi ile ilişkili CRS · Kimerik antijen reseptörü-T hücre terapisi ile ilişkili sitokin salınım sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D89.8 | NTBT | attributed | validated |
| ICD-11 | 4A4Y | NTBT | attributed | validated |
| UMLS | C5680162 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:182222Rare systemic disease
rare systemic and rheumatological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
31 annotations · validated 2021-06-03| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0011117 | Abnormal circulating interleukin concentration | |
| HP:0011112 | Abnormality of serum cytokine level | |
| HP:0012378 | Fatigue | |
| HP:0001945 | Fever | |
| HP:0012649 | Increased inflammatory response | |
| Frequent (79-30%) | ||
| HP:0002615 | Hypotension | |
| HP:0030783 | Increased circulating interleukin 6 concentration | |
| HP:0030356 | Increased serum interferon-gamma level | |
| HP:0003326 | Myalgia | |
| HP:0004396 | Poor appetite | |
| HP:0001649 | Tachycardia | |
| Occasional (29-5%) | ||
| HP:0011675 | Arrhythmia | |
| HP:0030005 | Capillary leak | |
| HP:0001289 | Confusion | |
| HP:0011037 | Decreased urine output | |
| HP:0002014 | Diarrhea | |
| HP:0003259 | Elevated circulating creatinine concentration | |
| HP:0002910 | Elevated circulating hepatic transaminase concentration | |
| HP:0012722 | Heart block | |
| HP:0002904 | Hyperbilirubinemia | |
| HP:0012418 | Hypoxemia | |
| HP:0002018 | Nausea | |
| HP:0002202 | Pleural effusion | |
| HP:0100598 | Pulmonary edema | |
| HP:0012664 | Reduced left ventricular ejection fraction | |
| HP:0002789 | Tachypnea | |
| HP:0002013 | Vomiting | |
| Very rare (<4-1%) | ||
| HP:0001919 | Acute kidney injury | |
| HP:0005521 | Disseminated intravascular coagulation | |
| HP:0002878 | Respiratory failure | |
| HP:0000988 | Skin rash | |
Run the differential on the 5 obligate / very frequent terms
Annotation source: 30753567[PMID]_30181581[PMID]_28925994[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/542323
- This record as JSON: /data/d/542323.json