Primary Hemophagocytic Lymphohistocytosis in Adult Woman; A Case Report

International Journal of Medical Science

© 2017 by SSRG - IJMS Journal

Volume 4 Issue 4

Year of Publication : 2017

Authors : Mohammed Saad Alqahtani

10.14445/23939117/IJMS-V4I4P101

How to Cite?

Mohammed Saad Alqahtani, "Primary Hemophagocytic Lymphohistocytosis in Adult Woman; A Case Report," SSRG International Journal of Medical Science, vol. 4,  no. 4, pp. 1-3, 2017. Crossref, https://doi.org/10.14445/23939117/IJMS-V4I4P101

Abstract:

Hemophagocytic lymphohistiocytosis (HLH) is a frequently fatal immune dysregulation disorder. We are reporting a case of HLH in a 37-year old Pakistani female nurse who presented to the emergency department with fever and huge hepatosplenomegaly. Investigations showed cytopenias affecting WBCs and platelets, high ferritin, and positive hemophagocytosis in bone marrow biopsy. The primary designation of HLH was established after ruling out secondary causes including infections, malignancy, rheumatologic disorders, and organ transplants. The case was responding well to the standard immunochemotherapy for HLH without relapsing fevers or the need for bone marrow transplantation.

Keywords:

[1] Allen CE, McClain KL: Pathophysiology and epidemiology of hemophagocytic lymphohistiocytosis. Hematology Am Soc Hematol Educ Program 2015, 2015:177-182.
[2] Mehta RS, Smith RE: Hemophagocytic lymphohistiocytosis (HLH): a review of literature. Med Oncol 2013, 30(4):740.
[3] George MR: Hemophagocytic lymphohistiocytosis: review of etiologies and management. J Blood Med 2014, 5:69-86.
[4] Meeths M, Horne A, Sabel M, Bryceson YT, Henter JI: Incidence and clinical presentation of primary hemophagocytic lymphohistiocytosis in Sweden. Pediatr Blood Cancer 2015, 62(2):346-352.
[5] Niece JA, Rogers ZR, Ahmad N, Langevin AM, McClain KL: Hemophagocytic lymphohistiocytosis in Texas: observations on ethnicity and race. Pediatr Blood Cancer 2010, 54(3):424-428.
[6] Akenroye AT, Madan N, Mohammadi F, Leider J: Hemophagocytic Lymphohistiocytosis mimics many common conditions: case series and review of literature. Eur Ann Allergy Clin Immunol 2017, 49(1):31-41.
[7] Henter JI, Samuelsson-Horne A, Arico M, Egeler RM, Elinder G, Filipovich AH, Gadner H, Imashuku S, Komp D, Ladisch S et al: Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood 2002, 100(7):2367-2373.
[8] Clementi R, Emmi L, Maccario R, Liotta F, Moretta L, Danesino C, Arico M: Adult onset and atypical presentation of hemophagocytic lymphohistiocytosis in siblings carrying PRF1 mutations. Blood 2002, 100(6):2266-2267.
[9] Nagafuji K, Nonami A, Kumano T, Kikushige Y, Yoshimoto G, Takenaka K, Shimoda K, Ohga S, Yasukawa M, Horiuchi H et al: Perforin gene mutations in adult-onset hemophagocytic lymphohistiocytosis. Haematologica 2007, 92(7):978-981.
[10] Otrock ZK, Eby CS: Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis. Am J Hematol 2015, 90(3):220-224.
[11] Fu L, Wang J, Wei N, Wu L, Wang Y, Huang W, Zhang J, Liu J, Wang Z: Allogeneic hematopoietic stem-cell transplantation for adult and adolescent hemophagocytic lymphohistiocytosis: a single center analysis. Int J Hematol 2016, 104(5):628-635.
[12] Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, Ladisch S, McClain K, Webb D, Winiarski J et al: HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007, 48(2):124-131.
[13] Rajagopala S, Singh N: Diagnosing and treating hemophagocytic lymphohistiocytosis in the tropics: systematic review from the Indian subcontinent. Acta Med Acad 2012, 41(2):161-174.
[14] Abbaker AI, Dammas AS: Familial hemophagocytic lymphohistiocytosis in two Saudi siblings. Sudan J Paediatr 2015, 15(1):57-60.
[15] Elyamany G, Alzahrani A, Elfaraidi H, Alsuhaibani O, Othman N, Al Mussaed E, Alabbas F: Hemophagocytic Lymphohistiocytosis: Single-Center Series of 12 Cases from Saudi Arabia. Clin Med Insights Pediatr 2016, 10:21-26.
 

References:

[1] Allen CE, McClain KL: Pathophysiology and epidemiology of hemophagocytic lymphohistiocytosis. Hematology Am Soc Hematol Educ Program 2015, 2015:177-182.
[2] Mehta RS, Smith RE: Hemophagocytic lymphohistiocytosis (HLH): a review of literature. Med Oncol 2013, 30(4):740.
[3] George MR: Hemophagocytic lymphohistiocytosis: review of etiologies and management. J Blood Med 2014, 5:69-86.
[4] Meeths M, Horne A, Sabel M, Bryceson YT, Henter JI: Incidence and clinical presentation of primary hemophagocytic lymphohistiocytosis in Sweden. Pediatr Blood Cancer 2015, 62(2):346-352.
[5] Niece JA, Rogers ZR, Ahmad N, Langevin AM, McClain KL: Hemophagocytic lymphohistiocytosis in Texas: observations on ethnicity and race. Pediatr Blood Cancer 2010, 54(3):424-428.
[6] Akenroye AT, Madan N, Mohammadi F, Leider J: Hemophagocytic Lymphohistiocytosis mimics many common conditions: case series and review of literature. Eur Ann Allergy Clin Immunol 2017, 49(1):31-41.
[7] Henter JI, Samuelsson-Horne A, Arico M, Egeler RM, Elinder G, Filipovich AH, Gadner H, Imashuku S, Komp D, Ladisch S et al: Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood 2002, 100(7):2367-2373.
[8] Clementi R, Emmi L, Maccario R, Liotta F, Moretta L, Danesino C, Arico M: Adult onset and atypical presentation of hemophagocytic lymphohistiocytosis in siblings carrying PRF1 mutations. Blood 2002, 100(6):2266-2267.
[9] Nagafuji K, Nonami A, Kumano T, Kikushige Y, Yoshimoto G, Takenaka K, Shimoda K, Ohga S, Yasukawa M, Horiuchi H et al: Perforin gene mutations in adult-onset hemophagocytic lymphohistiocytosis. Haematologica 2007, 92(7):978-981.
[10] Otrock ZK, Eby CS: Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis. Am J Hematol 2015, 90(3):220-224.
[11] Fu L, Wang J, Wei N, Wu L, Wang Y, Huang W, Zhang J, Liu J, Wang Z: Allogeneic hematopoietic stem-cell transplantation for adult and adolescent hemophagocytic lymphohistiocytosis: a single center analysis. Int J Hematol 2016, 104(5):628-635.
[12] Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, Ladisch S, McClain K, Webb D, Winiarski J et al: HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007, 48(2):124-131.
[13] Rajagopala S, Singh N: Diagnosing and treating hemophagocytic lymphohistiocytosis in the tropics: systematic review from the Indian subcontinent. Acta Med Acad 2012, 41(2):161-174.
[14] Abbaker AI, Dammas AS: Familial hemophagocytic lymphohistiocytosis in two Saudi siblings. Sudan J Paediatr 2015, 15(1):57-60.
[15] Elyamany G, Alzahrani A, Elfaraidi H, Alsuhaibani O, Othman N, Al Mussaed E, Alabbas F: Hemophagocytic Lymphohistiocytosis: Single-Center Series of 12 Cases from Saudi Arabia. Clin Med Insights Pediatr 2016, 10:21-26.