Table 2 Characteristics of patient’s cases reported in the literature with HLH during pregnancy
| Â | Maternal age (years) | Gestational age (weeks) | Associated disease | Clinical signs | Treatment | Outcome | Outcome |
|---|---|---|---|---|---|---|---|
Nakabayashi et al. (1999) [29] | ND | 21 | Preeclampsia | Fever, hepatosplenomegaly, cytopenias, hyperferritinemia | IVIG | Complete remission | survive |
Chmait et al. (2000) [30] | 24 | 29 | History of necrotizing lymphadenitis; EBV (discovered postmortem) | Fever, cytopenias, hyperferritinemia | Delivery | no response | Death |
Yamagushi et al. (2005) [8] | ND | 2nd trimester | HSV-2, genital herpes infection | Fever, skin lesions, pancytopenia, hypertriglycemia, hyperferritinemia | Corticosteroids; Cyclosporin A | Complete remission (failed corticosteroids, remission with Cyclosporin A) | survive |
Hanaoka et al. (2007) [5] | 33 | 23 | B-cell lymphoma | Fever, hepatosplenomegaly, cytopenias, hyperferritinemia, hypertriglyceridemia, DIC, elevated sCD25 | Emergent C-section (fetal distress); R-CHOP chemotherapy | Complete remission | survive |
Perard et al. (2007) [6] | 28 | 22 | Systemic lupus erythematosus | Fever, pancytopenia, hypertriglycemia, hyperferritinemia | Corticosteroids, IVIG 3 doses, Premature Delivery | No improvement with steroids; premature delivery; complete remission after third IVIG dose | survive |
Teng et al. (2009) [7] | 28 | 23 | Autoimmune hemolytic anemia | Fever, hepatosplenomegaly, Cytopenias, hyperferritinemia, hypertriglyceridemia | Corticosteroids Cesarean | Failed corticosteroids; complete remission after Cesarean | survive |
Yoshida et al. (2009) [9] | ND | Post-partum | Systemic lupus erythematosus | Fever, cytopenias, hyperferritinemia hypertriglyceridemia | Corticosteroids | Complete remission | survive |
Chien et al. (2009) [31] | 28 | 23 | Unclear | Fever, cytopenias, hyperferritinemia, hypertriglyceridemia | Cesarean delivery | Complete remission | survive |
Arewa et al. (2011) [11] | 31 | 21 | HIV | Fever, jaundice, abdominal pain, cytopenias | HAART Delivery at term | Complete remission | survive |
Hannebicque Montaigne et al. (2012) [32] | 21 | 29 | Systemic lupus erythematosus | Fever, pancytopenia, hyperferritinemia, hypertriglycemia | Corticosteroids IVIG | Complete remission | survive |
Dunn et al. (2012) [33] | 41 | 19 | Still’s disease | Fever, rash, cytopenias, hyperferritinemia, hypertriglyceridemia, elevated sCD25 | Corticosteroids | Complete remission | survive |
Shukla et al. (2013) [34] | 23 | 10 | Unclear | Fever, hepatosplenomegaly, cytopenia, hypertriglycemia, hyperferritinemia | Corticosteroids; spontaneous abortion | Failed steroids; complete remission after abortion | survive |
Mayama et al. (2014) [35] | 28 | 21 | Parvovirus B19 | Fever, cytopenias, hyperferritinemia, hypertriglyceridemia | Corticosteroids | Complete remission | survive |
Goulding et al. (2014) [10] | 27 | 23 | HSV-2 | Fever, cytopenias and hyperferritinemia | Corticosteroids, acyclovir | Complete remission | survive |
Klein et al. (2014) [36] | 39 | 30 | EBV | Fever, hepatosplenomegaly, cytopenias, hyperferritinemia | Corticosteroids, cyclosporine and etoposide in combination with Rituximab | no response | death |
Tumian et al. (2015) [12] | 35 | 38 | CMV (postmortem diagnosis) | fever, cytopenias, hyperferritinemia, hypertriglyceridemia, hepatitis | Corticosteroids IgIV, cyclosporine | no response | death |
Samra et al. (2015) [4] | 36 | 16 | Unclear | fever, hepatosplenomegaly, cytopenias, hyperferritinemia, hypertriglyceridemia, | Corticosteroids | Complete remission | survive |
Rousselin et al. (2015) [37] | 44 | 30 | Autoimmune disease | Fever, hepatomegaly cytopenias, hyperferritinemia, hypertriglycemia | glucocorticoides | Complete remission | survive |
Giard et al. (2016) [38] | 35 | 13 | Kikuchi Fujimoto lymphadenitis | fever, cytopenias, hyperferritinemia, hypertriglycemia | Corticosteroids Etoposide Abortion | response | death |
Ikeda et al. (2017) [39] | 32 | 11 | EBV | fever, cytopenias, hyperferritinemia, hypertriglyceridemia | Single dose dexamethasone Etoposide Cyclosporine | Complete remission | survive |
Robert et al. (2017) [40] | 33 | 22 | unclear | fever, hyperferritinemia | Corticosteroids, etoposide, BMT | Partial remission after etoposide, complete remission after BMT | survive |
Yildiz et al. (2017) [3] | 36 | 29 | unclear | fever, cytopenias, hyperferritinemia hypertriglyceridemia | Corticosteroids | Complete remission | survive |
He M et al. (2017) [41] | 27 | 30 | NK/T cells lymphoma | Fever, splenomegaly, cytopenias, hyperferritinemia | Corticosteroids and etoposide in combination with rituximab | no response | death |