User:Mr. Ibrahem/Hemophagocytic lymphohistiocytosis
| Hemophagocytic lymphohistiocytosis | |
|---|---|
| Other names | Haemophagocytic lymphohistiocytosis (British spelling), hemophagocytic or haemophagocytic syndrome,[1] familial hemophagocytic reticulosis[2] |
 | |
| Micrograph showing red blood cells within macrophages. H&E stain. | |
| Specialty | Hematology |
| Symptoms | Fever, bleeding problems, liver problems, large spleen, low blood cells[3] |
| Complications | Acute respiratory distress syndrome (ARDS), myocarditis, liver failure, encephalitis[4] |
| Usual onset | Early childhood, 50s[4] |
| Types | Primary (genetic), secondary[4] |
| Causes | Genetic mutations, cancer, infection, autoimmune problems[3] |
| Diagnostic method | Based on symptoms, blood tests, medical imaging, tissue biopsy[4] |
| Differential diagnosis | Sepsis, Kawasaki disease, toxic shock syndrome[4] |
| Treatment | Etoposide, immunosuppressants, stem cell transplantation[3] |
| Frequency | Rare[5] |
| Deaths | 22% 5-year survival (children)[4] |
Hemophagocytic lymphohistiocytosis (HLH) is a blood disorder in which the body produces high levels of cytokines resulting in organ damage.[3] Symptoms include fever, bleeding problems, liver problems, a large spleen, and low blood cells.[3] Complications can include acute respiratory distress syndrome (ARDS), myocarditis, liver failure, and encephalitis.[4]
It most commonly occurs due to inherited genetic mutations (25%) that alter regulation of the immune system or secondary to cancer, infection, or autoimmune problems.[3][5] The underlying mechanism involves activation of cytotoxic T lymphocytes, natural killer (NK) cells, and macrophages.[3] Diagnosis is based on symptoms, blood tests, medical imaging, and possibly tissue biopsy.[4] It is a type of cytokine storm.[6]
Treatment is with etoposide, immunosuppressants, such as corticosteroids, and stem cell transplantation.[3][7] Blood transfusion or platelet transfusion may be required.[5] In children, despite treatment, the 5-year survival rate is about 22%, while the risk of death in adults is about 40%.[4]
Hemophagocytic lymphohistiocytosis is rare, with the genetic form affecting about 1.5 per million people a year.[4] Other forms occur in about one in 2,000 people in the intensive care unit.[4] Onset is typically either in early childhood or around the age of 50.[4] Among children both sexes are affected with similar frequency.[4] The condition was first described in 1952 by Farquhar and Claireaux.[2]
References
[edit]- ^ Fisman, David N. (2000). "Hemophagocytic syndromes and infection". Emerging Infect. Dis. 6 (6): 601–8. doi:10.3201/eid0606.000608. PMC 2640913. PMID 11076718.
- ^ a b Rosado, Flavia G. N.; Kim, Annette S. (June 2013). "Hemophagocytic Lymphohistiocytosis: An Update on Diagnosis and Pathogenesis". American Journal of Clinical Pathology. 139 (6): 713–727. doi:10.1309/AJCP4ZDKJ4ICOUAT.
- ^ a b c d e f g h Al-Samkari, H; Berliner, N (24 January 2018). "Hemophagocytic Lymphohistiocytosis". Annual review of pathology. 13: 27–49. doi:10.1146/annurev-pathol-020117-043625. PMID 28934563.
- ^ a b c d e f g h i j k l m Konkol, S; Rai, M (January 2022). "Lymphohistiocytosis". StatPearls. PMID 32491708.
- ^ a b c "Hemophagocytic Lymphohistiocytosis". NORD (National Organization for Rare Disorders). Archived from the original on 10 May 2022. Retrieved 9 December 2022.
- ^ Kliegman, Robert M.; III, Joseph W. St Geme (1 April 2019). Nelson Textbook of Pediatrics E-Book. Elsevier Health Sciences. p. 2709. ISBN 978-0-323-56888-3. Archived from the original on 9 December 2022. Retrieved 9 December 2022.
- ^ Skinner, J; Yankey, B; Shelton, BK (2019). "Hemophagocytic Lymphohistiocytosis". AACN advanced critical care. 30 (2): 151–164. doi:10.4037/aacnacc2019463. PMID 31151946.