Analysis of patients of hemophagocytic lymphohistiocytosis secondary to infections
DOI:
https://doi.org/10.18203/2349-3933.ijam20204063Keywords:
Hemophagocytic lymphohistiocytosis, Macrophages, Tropical fever, Haematological malignanciesAbstract
Background: Hemophagocytic lymphohistiocytosis is characterized by an unremitting activation of CD8+ T lymphocytes and macrophages that leads to organ damage.
Methods: 40 patients diagnosed of having HLH secondary to infections admitted in the last 5 years in a tertiary hospital in Western Maharashtra, were studied retrospectively. The data was collected from the indoor patient records and files, detailed clinical profile and all relevant investigations were noted.
Results: 40 cases of diagnosed HLH were studied. Age group of 30-40 years was involved more (25%), followed by age group of 61-70 years. The condition was more common in males (64%). Dengue was more common having more chance of secondary HLH. Early diagnosis and treatment was effective in 90% cases.
Conclusions: HLH was seen to occur more in tropical fevers. The mortality rate was more in haematological malignancies. Early diagnosis and rapidly initiated treatment had a positive effect in decreasing the mortality rate of the condition.References
Otrock ZK, Eby CS. Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis. Am J Hematol. 2014;90:220-4.
Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis. Eur J Pediatr. 2007;166:95-109.
Nikiforow S, Berliner N. The unique aspects of presentation and diagnosis of hemophagocytic lymphohistiocytosis in adults hematology. Am Soc Hematol Educ Program. 2015;2015:183-9.
Schram AM, Berliner N. How I treat hemophagocytic lymphohistiocytosis in the adult patient. Blood. 2015;125:2908-14.
Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48:124-31.
Zainab A, Khan A, Tariq U, Muhammad SS. Clinical features and diagnosis of hemophagocytic lymphohistiocytosis. 2018;10(4):e2545.
Lizamarie BR, Ellen KR. A case series of adult secondary hemophagocytic lymphohistiocytosis treated at weill cornell medical college. Blood. 2016;128:4874.
Otrock ZK, Eby CS. Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis. Am J Hematol. 2014;90:220-4.
George MR. Hemophagocytic lymphohistiocytosis: review of etiologies and management. J Blood Med. 2014;5:69-86.
Li J, Wang Q, Zheng W, Ma J, Zhang W, Wang W, Tian X. Hemophagocytic lymphohistiocytosis: clinical analysis of 103 adult patients. Medicine. 2014;93:100-5.
Henter JI, Samuelsson-Horne A, Arico M, Egeler RM, Elinder G, Filipovich AH, et al. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood. 2002;100(7):2367-73.
Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL. How I treat hemophagocytic lymphohistiocytosis. Blood. 2011;118(15):4041-52.
