DOI: http://dx.doi.org/10.18203/2349-3933.ijam20204063

Analysis of patients of hemophagocytic lymphohistiocytosis secondary to infections

Arundhati G. Diwan, Supriya S. Barsode, Amit R. Nisal, Ruturaj S. Deshpande, Mayur D. Patil, Neeraj S. Shettar, Vishal C. Lali, Gargee M. Pore, Bhabatosh K. Saha

Abstract


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.

Keywords


Hemophagocytic lymphohistiocytosis, Macrophages, Tropical fever, Haematological malignancies

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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.