Haemophagocytic Lymphohistiocytosis During Pregnancy: a Case Presentation and Literature Review

Overview

Journal Case Rep Perinat Med

Publisher De Gruyter

Date 2025 Mar 5

PMID 40041215

Authors

Larissa Favero Vanraes

Veerle Beckers

Kim Van Berkel

Leonardo Gucciardo

Gilles Faron

Affiliations

Soon will be listed here.

Abstract

Objectives: Haemophagocytic lymphohistiocytosis (HLH) is a potentially fatal disorder of the immune system that typically occurs in the paediatric population. Diagnosing this rare disease in the adult population is challenging, particularly during pregnancy.

Case Presentation: We present a case of a gravid patient developing HLH at week 13 of gestation undergoing a medical termination of pregnancy at 27 weeks due to anhydramnios and associated stopped foetal growth.

Conclusions: Disease triggers could vary from a simple viral infection to the pregnancy as such causing the disorder. Treatment should benefit the mother and limit the foetal harm.

References

Dunn T, Cho M, Medeiros B, Logan A, Ungewickell A, Liedtke M . Hemophagocytic lymphohistiocytosis in pregnancy: a case report and review of treatment options. Hematology. 2012; 17(6):325-8. DOI: 10.1179/1607845412Y.0000000007. View

Chien C, Lee F, Luk H, Wu C . Anesthetic management for cesarean delivery in a parturient with exacerbated hemophagocytic syndrome. Int J Obstet Anesth. 2009; 18(4):413-6. DOI: 10.1016/j.ijoa.2009.02.016. View

Alsulyman O, Castro M, Zuckerman E, McGehee W, Goodwin T . Preeclampsia and liver infarction in early pregnancy associated with the antiphospholipid syndrome. Obstet Gynecol. 1996; 88(4 Pt 2):644-6. DOI: 10.1016/0029-7844(96)00098-1. View

Hayden A, Lin M, Park S, Pudek M, Schneider M, Jordan M . Soluble interleukin-2 receptor is a sensitive diagnostic test in adult HLH. Blood Adv. 2018; 1(26):2529-2534. PMC: 5728644. DOI: 10.1182/bloodadvances.2017012310. View

Pagel J, Beutel K, Lehmberg K, Koch F, Maul-Pavicic A, Rohlfs A . Distinct mutations in STXBP2 are associated with variable clinical presentations in patients with familial hemophagocytic lymphohistiocytosis type 5 (FHL5). Blood. 2012; 119(25):6016-24. DOI: 10.1182/blood-2011-12-398958. View

Yamaguchi K, Yamamoto A, Hisano M, Natori M, Murashima A . Herpes simplex virus 2-associated hemophagocytic lymphohistiocytosis in a pregnant patient. Obstet Gynecol. 2005; 105(5 Pt 2):1241-4. DOI: 10.1097/01.AOG.0000157757.54948.9b. View

Jordan M, Allen C, Weitzman S, Filipovich A, McClain K . How I treat hemophagocytic lymphohistiocytosis. Blood. 2011; 118(15):4041-52. PMC: 3204727. DOI: 10.1182/blood-2011-03-278127. View

Hannebicque-Montaigne K, Le Roch A, Launay D, Coulon C, Deruelle P, Langlois S . [Haemophagocytic syndrome in pregnancy: a case report]. Ann Fr Anesth Reanim. 2012; 31(3):239-42. DOI: 10.1016/j.annfar.2011.11.023. View

Hanaoka M, Tsukimori K, Hojo S, Abe Y, Mutou T, Muta K . B-cell lymphoma during pregnancy associated with hemophagocytic syndrome and placental involvement. Clin Lymphoma Myeloma. 2007; 7(7):486-90. DOI: 10.3816/clm.2007.n.033. View

10.

Kerley R, Kelly R, Cahill M, Kenny L . Haemophagocytic lymphohistiocytosis presenting as HELLP syndrome: a diagnostic and therapeutic challenge. BMJ Case Rep. 2017; 2017. PMC: 5534682. DOI: 10.1136/bcr-2017-219516. View

11.

Wang Y, Wang Z, Zhang J, Wei Q, Tang R, Qi J . Genetic features of late onset primary hemophagocytic lymphohistiocytosis in adolescence or adulthood. PLoS One. 2014; 9(9):e107386. PMC: 4169386. DOI: 10.1371/journal.pone.0107386. View

12.

Henter J, Horne A, Arico M, Egeler R, Filipovich A, Imashuku S . HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2006; 48(2):124-31. DOI: 10.1002/pbc.21039. View

13.

Teng C, Hwang G, Lee B, Wang R, Chou M . Pregnancy-induced hemophagocytic lymphohistiocytosis combined with autoimmune hemolytic anemia. J Chin Med Assoc. 2009; 72(3):156-9. DOI: 10.1016/S1726-4901(09)70043-7. View

14.

Nakabayashi M, Adachi T, IZUCHI S, Sugisaki A . Association of hypercytokinemia in the development of severe preeclampsia in a case of hemophagocytic syndrome. Semin Thromb Hemost. 2000; 25(5):467-71. DOI: 10.1055/s-2007-994952. View

15.

Yildiz H, Vandercam B, Thissen X, Komuta M, Lanthier N, Debieve F . Hepatitis during pregnancy: A case of hemophagocytic lymphohistiocytosis. Clin Res Hepatol Gastroenterol. 2017; 42(3):e49-e55. DOI: 10.1016/j.clinre.2017.10.007. View

16.

Rubin T, Zhang K, Gifford C, Lane A, Choo S, Bleesing J . Perforin and CD107a testing is superior to NK cell function testing for screening patients for genetic HLH. Blood. 2017; 129(22):2993-2999. PMC: 5766842. DOI: 10.1182/blood-2016-12-753830. View

17.

Hahn S, Hasler P, Vokalova L, Van Breda S, Than N, Hoesli I . Feto-Maternal Microchimerism: The Pre-eclampsia Conundrum. Front Immunol. 2019; 10:659. PMC: 6455070. DOI: 10.3389/fimmu.2019.00659. View

18.

Leslie K, Koil C, Rayburn W . Chemotherapeutic drugs in pregnancy. Obstet Gynecol Clin North Am. 2005; 32(4):627-40. DOI: 10.1016/j.ogc.2005.08.009. View

19.

Gholam C, Grigoriadou S, Gilmour K, Gaspar H . Familial haemophagocytic lymphohistiocytosis: advances in the genetic basis, diagnosis and management. Clin Exp Immunol. 2011; 163(3):271-83. PMC: 3048610. DOI: 10.1111/j.1365-2249.2010.04302.x. View

20.

Mayama M, Yoshihara M, Kokabu T, Oguchi H . Hemophagocytic lymphohistiocytosis associated with a parvovirus B19 infection during pregnancy. Obstet Gynecol. 2014; 124(2 Pt 2 Suppl 1):438-441. DOI: 10.1097/AOG.0000000000000385. View