Table 2.

Reported cases of sHLH in pregnancy.

Reported cases of pregnancy-associated sHLH
Case number	Authors	Age (years)	Obstetric history	Gestational age (weeks)	Aetiology	Clinical presentation and laboratory findings	Treatment (except antibiotics)	Maternal outcome	Mode of delivery and fetal outcome
1	Gill et al. 16	30	–	18	–	Fever, hepatomegaly, pancytopenia	IVIg	Remission	40/40 VD – survived
2	Tsuda et al. 17	–	–	–	Parvovirus B19	–	–	–	–
3	Yamanaka et al. 18	–	–	36	–	–	Steroids, IVIg, AT III	Remission	Survived
4	Ishida et al. 19	31	–	–	Hyperemesis	–	Steroids, IVIg	Death	22/40 – IU death
5	Mihara et al. 20	32	–	16	EBV	Fever, pancytopenia, elevated ferritin, LDH, sIL-2R	Steroids, aciclovir  IVIg, gabexate mesilate	Failure Remission	35/40 VD – survived
6	Nakabayashi et al. 21	30	–	21	Pre-eclampsia	Fever, pancytopenia, elevated ferritin, LDH, LFTs	IVIg AT III	Failure Remission	29/40 CS – survived
7	Chmait et al. 22	24	G2 P1	29	EBV	Fever, pancytopenia, elevated LFTs	IVIg, aciclovir, CS	Death	30/40 CS – survived
8	Yamaguchi et al. 23	–	–	–	HSV-2	Fever, pancytopenia, elevated ferritin, TG, sIL-2R	Steroids CsA	Failure Remission	40/40 VD – survived
9	Hanaoka et al. 24	33	G1 P0	21	B-cell lymphoma	Fever, hepatosplenomegaly, pancytopenia, elevated ferritin, TG, LDH, sIL-2R	R-CHOP, HSCT	Remission	28/40 CS – survived
10	Perard et al. 25	28	G4	22	SLE	Fever, pancytopenia, elevated ferritin, TG	Steroids IVIg	Failure Remission	30/40 VD – survived
11	Gonzalez et al. 26	27	–	22	Parvovirus B19	–	Steroids, AT III	Remission	36/40 – survived
12	Chien et al. 27	28	G1 P0	23	–	Fever, bicytopenia, elevated TG	CS delivery	Remission	28/40 CS – survived
13	Teng et al. 28	28	G1 P0	23	AIHA	Fever, bicytopenia, elevated ferritin, TG, LDH, sIL-2R	Steroids TOP	Failure Remission	29/40 CS – death
14	Yoshida et al 29	33	–	PP	SLE	–	Steroids	Remission	Survived
15	Arewa and Ajadi 30	31	G1 P0	21	HIV and malaria	Fever, bicytopenia, jaundice	Antimalarials, HAART, VD	Remission	40/40 VD – survived
16	Dunn et al. 31	41	–	19	AOSD	Fever, rash, headache, anaemia, elevated ferritin, TG, LDH, LFTs	Steroids, CS	Remission	30/40 CS – survived (twins)
17	Hannebicque-Montaigne et al. 32	21	–	29	SLE	Fever, pancytopenia, elevated ferritin, TG	Steroids, IVIg	Remission	38/40 VD – survived
18	Kim et al. 33	29	–	12	SLE	Fever, pancytopenia, elevated ferritin, TG, LDH	CsA, Splenectomy	Remission	14/40 TOP – death
19	Komaru et al. 34	36	G1 P0	40 + 38d	Sjogren’s	Splenomegaly, bicytopenia, jaundice, elevated ferritin, bilirubin, LDH, sIL-2R	Steroids	Remission	40/40 VD – survived
20	Shukla et al. 35	23	G1 P0	10	–	Fever, hepatosplenomegaly, pancytopenia, elevated ferritin, TG, bilirubin	Steroids EPL	Failure Remission	10/40 EPL – death
21	Goulding et al. 36	27	G1 P0	23	HSV-2	Fever, pancytopenia, elevated ferritin	Steroids, aciclovir, Valaciclovir	Remission	24/40 CS – death
22	Klein et al. 37	39	–	30	EBV	Pancytopaenia, elevated ferritin, sIL-2R	Steroids, CsA, etoposide, RTX	Death	31/40 CS – death (twins)
23	Mayama et al. 38	28	G2 P2	19	Parvovirus B19	Fever, pancytopenia, elevated ferritin, LDH	Steroids	Remission	37/40 VD – survived
24	Samra et al. 39	36	–	16	–	Fever, pancytopenia, elevated ferritin, TG	Steroids	Remission	40/40 VD – survived
25	Tumian and Wong. 40	35	G2 P1	38	CMV	Fever, anaemia, jaundice, elevated ferritin, TG, LDH, LFTs, sIL-2R	CS delivery, steroids, CsA	Death	38/40 CS
26	Giard et al. 41	35	G2 P1	13	–	Fever, pancytopenia, jaundice, elevated ferritin, TG, LFTs, sCD25	Steroids, etoposide	Death	22/40 TOP – death
27	Ota et al. 42	26	G1 P0	23	Liver abscess	Fever, thrombocytopaenia, elevated ferritin, TG, LDH, LFTs, sIL-2R	Nil	Death	23/40 – IU death
28	He et al. 43	27	G2 P0	30	NK/T-cell lymphoma	Fever, splenomegaly, pancytopenia, elevated ferritin, LDH, LFTs	Steroids, etoposide, RTX, CS delivery, splenectomy	Death	30/40 CS – survived
29	Ikeda et al. 44	32	–	11	EBV	Fever, bicytopenia, elevated ferritin	Steroids, etoposide, CsA	Remission	11/40 EPL – death
30	Kerley et al. 45	33	G1 P0	22	–	Fever, bicytopenia, elevated ferritin, LDH, LFTs	Steroids Delivery, etoposide, BMT	Failure Remission	22/40 VD – death
31	Rousselin et al. 46	44	G1 P0	30	–	Fever, hepatomegaly, pancytopenia, elevated ferritin, TG, LDH, LFTs	Steroids	Remission	38/40 VD – survived
32	Nasser et al. 47	36	G7 P2	31	HSV-2	Fever, splenomegaly, thrombocytopaenia, elevated ferritin, LDH, LFTs	Steroids, aciclovir, Delivery	Remission	CS – death
33	Cheng et al. 48	–	–	26	–	Fever, bicytopenia, elevated ferritin, TG	Steroids, etoposide	Remission	27/40 TOP – death
34	Parrott et al. 14	28	G2 P1	18	SLE	Fever, splenomegaly, pancytopenia, elevated ferritin, LFTs, sIL-2R, stroke	Steroids, etoposide, IVIg, CsA	Failure	21/40 VD – IU death
35	Parrott et al. 14	37	G4 P3	24	CMV	Fever, thrombocytopaenia, jaundice, elevated ferritin, TG, LFTs, bilirubin, LDH, sIL-2R	Steroids, etoposide	Remission	37/40 VD – survived
36	Yip et al. 49	23	G2 P1	22	–	Fever, pancytopenia, elevated ferritin, TG	Steroids, IVIg Anakinra	Failure Remission	31/40 CS – survived

AOSD: adult-onset Still’s disease; AT III: antithrombin III; BMT: bone marrow transplantation; CMV: cytomegalovirus; CS: caesarean section; CsA: ciclosporin A; d: days; EBV: Epstein-Barr virus; EPL: early pregnancy loss; G: gravida; HAART: highly active anti-retroviral therapy; HIV: human immunodeficiency virus; HSCT: haematopoietic stem cell transplantation; HSV: herpes simplex virus; Ig: immunoglobulin; IU: intrauterine; IVIg: intravenous immunoglobulin; LFTs: liver function tests; LDH: lactate dehydrogenase; NK: natural killer; P: para; PP: postpartum; R-CHOP: rituximab: cyclophosphamide: doxorubicin: vincristine: prednisolone; RTX: rituximab; sCD25: soluble CD25; sIL-2R: soluble interleukin-2 receptor; SLE: systemic lupus erythematosus; TOP: termination of pregnancy; TG: triglycerides; VD: vaginal delivery.