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. 2022 Jan 2;48(3):610–620. doi: 10.1111/jog.15133

TABLE 4.

Characteristics of patients previously reported to have HLH during pregnancy

Case Age (years) Associated diagnoses Treatment and outcome Period of gestation (weeks) Complications Gestation (weeks), delivery method Indication Maternal/fetal survival
Chmait et al. 7 24 Necrotizing lymphadenitis IVIG postpartum day 6 29 SGA 30, CS Condition worsened, breech presentation No/yes
Teng et al. 3 28 AIHA Steroids failed, remission after CS 23 SGA 29, CS Fetal distress Yes/no
Dunn et al. 12 41 Still's disease Remission with corticosteroids 19 SGA 30, CS IUGR Yes/yes
Pérard et al. 13 28 SLE Failed with corticosteroids/IVIG, remission after delivery and third IVIG dose 22 Eclampsia, cerebral hemorrhage 30, VD PPROM Yes
Nakabayashi et al. 14 30 Unknown Failed with IVIG, remission with antithrombin concentrate 21 Preeclampsia, SGA 29, CS Preeclampsia, IUGR, Fetal distress Yes/yes
Mihara et al. 15 32 EBV Failed with corticosteroids, remission with IVIG acyclovir, methylprednisolone 16 35, VD Yes/yes
Hanaoka et al. 16 33 B‐cell lymphoma Failed with corticosteroids, remission with R‐CHOP postpartum day 8 21 28, CS Fetal distress Yes/yes
Chien et al. 17 28 Unknown Failed with corticosteroids, remission after CS 23 SGA 30, CS Fetal distress Yes/no
Klein et al. 18 39 EBV Failed with steroids, CsA, etoposide, rituximab 30 31, CS Twins, gastrointestinal bleeding No/yes
Goulding and Barnden 19 27 HSV Remission with steroids, acyclovir 23 + 5 24, CS PPROM and chorioamnionitis Yes/no
Mayama et al. 20 28 Parvovirus B19 Remission with steroids 20 37, VD Yes /yes
Tumian and Wong 21 35 CMV Failed with steroids, IVIG, CsA, acyclovir, plasma exchange 38 38, CS Fetal distress, previous CS No/yes
Samra et al. 22 36 Unknown Remission with steroids 16 Term, VD Yes/yes
Giard et al. 23 35 KF lymphadenitis Failed with steroids and etoposide 20 22, spontaneous abortion No/no
Fernández et al. 24 20 Tuberculosis Failed with steroids IVIG, etoposide, CsA. Remission after anti‐tuberculosis treatment 24 PPROM 29, CS Breech presentation, PPROM Yes/yes
Takada et al. 25 35 SLE Remission with steroids 11 35, VD Yes/yes
Rousselin et al. 26 44 Raynaud syndrome Remission with steroids 30 SGA 38, VD IUGR oligohydramnios Yes/yes
Yildiz et al. 27 36 Unknown Remission with steroids 29 31 + 6, CS Fetal distress Yes/yes
He et al. 6 27 NK/T‐cell lymphoma Failed with steroids and etoposide 30 30 + 4, CS Fetal distress No/yes
Sarkissian et al. 28 30 HSV‐1, CMV, EBV Failed with steroids and etoposide 35 + 2 HELLP 35 + 2, CS HELLP No/yes
Song et al. 29 26 Infection (Staphylococcus epidermidis) Failed with corticosteroids, IVIG. Remission with etoposide 31 31, VD Yes/yes
Song et al. 29 36 Unknown Remission with corticosteroids, etoposide 14 Spontaneous miscarriage No/no
Song et al. 29 30 Angioimmunoblastic T‐cell lymphoma Failed with steroids and etoposide. Remission after ECHOP, allo‐HSCT 34 34, VD Yes/yes
Song et al. 29 30 Unknown Failed with corticosteroids/delivery, remission with HLH‐04 regimen, DEP regimen 30 35, CS Yes/yes
Song et al. 29 27 EBV Failed with steroids, remission with etoposide 19 NA Yes/yes
Song et al. 29 29 Unknown

Failed with corticosteroids/delivery,

remission with etoposide

 30 30, CS Transverse position Yes/yes
Song et al. 29 24 Still's disease Remission with corticosteroids, fludarabine 10 16, Induced abortion Yes/no
Song et al. 29 24 Unknown Failed with corticosteroids and cyclosporine, remission after abortion 17 19, Induced abortion Yes/no
Song et al. 29 26 Tuberculosis Failed with corticosteroid, remission after anti‐tuberculosis treatment 28 28, VD Yes/yes
Song et al. 29 20 SLE Remission with corticosteroids and cyclosporine. 10 Spontaneous miscarriage Yes/no
Song et al. 29 24 Unknown Remission with corticosteroids 36 36, VD Yes/yes
Song et al. 29 29 Unknown Failed with corticosteroids/delivery 28 28, VD No/yes
Song et al. 29 25 EBV Failed with corticosteroids/delivery 24 24, Delivered No/no
Parrott et al. 30 28 SLE Failed with steroids IVIG and etoposide 18 SGA 21 + 4, Spontaneously delivered IUGR No/no
Parrott et al. 30 37 CMV Remission with steroids, etoposide, acyclovir, HLH‐94 24 SGA 37, VD IUGR Yes/yes
Cheng et al. 4 29 Unknown Failed with steroids, remission with etoposide, corticosteroid IVIG after CS 26 + 2 27 + 2, CS Condition worsened Yes/yes
Nasser et al. 31 36 HSV 2 Remission with steroids, acyclovir 31 31, CS Yes/no
Shukla et al. 5 23 Unknown Failed with steroids, remission after abortion 10 Spontaneous abortion Yes/no
Kerley et al. 32 33 Unknown Failed with steroids. BMT, CR 22 22, VD (induced labor) Yes/no
Yamaguchi et al. 33 NA HSV 2 Failed with steroids, remission with cyclosporine, acyclovir Midgestation 37, CS Breech presentation

Abbreviations: AIHA, autoimmune hemolytic anemia; allo‐HSCT, allogenic hematopoietic stem cell transplant; BMT bone marrow transplantation; CMV, cytomegalovirus; CR, complete remission; CS, cesarean section; CsA, cyclosporine A; DEP, doxorubicin‐etoposide‐methylprednisolone; EBV, Epstein–Barr virus; ECHOP, etoposide/cyclophosphamide/doxorubicin/vincristine/prednisone; HELLP, hemolysis, elevated liver enzymes, low platelet count; HLH, hemophagocytic lymphohistiocytosis; HSV, herpes simplex virus; IUGR, intrauterine growth retardation; IVIG, intravenous immunoglobulin; KF, Kikuchi‐Fujimoto; NA, information not available; NK, natural killer; PPROM, premature rupture of the membranes; R‐CHOP, rituximab/cyclophosphamide/doxorubicin/vincristine/prednisone; SGA, small for gestational age; SLE, systemic lupus erythematosus; VD, vaginal delivery.