Case Reports in Obstetrics and Gynecology

Case Report

Hemophagocytic Lymphohistiocytosis in Pregnancy: A Case Series and Review of the Current Literature

Table 1

Hemophagocytic lymphohistiocytosis during pregnancy.
(a)
CaseAge (yrs)Cause/Associated DiagnosesGestation at Presentation (wks)Hepatomegaly/Splenomegaly/LymphadenopathyWBC (K/UL)Hgb (g/μL)Plt (K/UL)ALT/AST (U/L)
Arewa and Ajadi 201131HIV-1 and malaria21-/-/-4.251256/4
Chien 200928Unknown23NA8.95.911NA/92
Chmait 200024EBV with necrotizing lymphadenitis29+/+/+2.6923408/371
Dunn 201241Still’s disease19NANeutropenia9.83432695/1258
Gill 199430Unknown17+/+/NA3.18.719-/241
Gonzalez 200827Parvovirus B19NANANANANANA
Hanaoka 200733B-cell non-Hodgkin lymphoma21+/+/NA5.89.5104110/170
Hannebicque-Montaigne 201229SLE21NANANANANA
Kim 201329SLE12+/+/NA3.86.994NL/56
Klein 201439EBV30NAPancytopeniaPancytopeniaPancytopeniaElevated
Komaru 201336Primary Sjogren’s syndrome38d postpartumNANANANANA
Mayama 201428Parvovirus B1920-/-/-0.64.283NL
Mihara 199932EBV16NANANANANA
Nakabayashi 199930Unknown21NA/-/-2.911.17.7-/87
Perard 200728SLE22NA/-/-3.59.280-/45
Samra 201536Unknown16+/+/-1.39.9125NA
Shukla 201323Unknown10+/+/-1.96.318NL
Teng 200928AIHA23+/+/NA8.94.910900018/92
Tsuda 199530Parvovirus B199d postpartumNANANANANA
Tumian 201535CMV38-/-/-15.07.184389/341
Yamaguchi 2005NAVZVNANANANANANA
Yamanaka 199523VZVNANANANANANA
Yoshida 200933SLEAfter deliveryNANANANANA
Case 128SLE19-/+/+3.110.36058/141
Case 237Unknown24-/-/-NL12.082437/1118
Neonate demised 3-4 days after birth due to respiratory distress.
NA: data not available; NL: normal; HIV-1: human immunodeficiency virus 1; EBV: Epstein-Barr virus; SLE: systemic lupus erythematosus; AIHA: autoimmune hemolytic anemia; CMV: cytomegalovirus; VZV: varicella zoster virus; WBC: white blood cell count; Hgb: hemoglobin; Plt: platelet count; ALT: alanine transaminase; ALT: aspartate transaminase; Bx: biopsy; BM: bone marrow; Abx: antibiotics; IVIg: intravenous immunoglobulin; Tx: treatment; HAART: highly active antiretroviral therapy; AT III: antithrombin III; R-CHOP: rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone chemotherapy regimen; PVSCT: peripheral blood stem cell transplant; CSA: cyclosporine A; G-CSF: granulocyte colony stimulating factor; PTL: preterm labor; HELLP: hemolysis elevated liver enzymes and low platelet count syndrome; Oligo: oligohydramnios; UA: umbilical artery; IUGR: intrauterine growth restriction; TAB: therapeutic abortion; IUFD: intrauterine fetal demise; PROM: premature rupture of membranes; ICH: intracerebral hemorrhage; SAB: spontaneous abortion; C/S: cesarean section; VD: vaginal delivery.
References: Arewa and Ajadi 2011 [3]; Chein 2009 [4]; Chmait 2000 [5]; Dunn 2012 [6]; Gill 1994 [7]; Gonzalez 2008 [8]; Hanaoka 2007 [9]; Hannebicque-Montaigne 2012 [10]; Kim 2013 [11]; Klein 2014 [3]; Komaru 2013 [12]; Mayama 2014 [13]; Mihara 1999 [14]; Nakabayashi 1999 [15]; Perard 2007 [16]; Samra 2015 [17]; Shukla 2013 [18]; Teng 2009 [19]; Tsuda 1995 [20]; Tumian 2015 [21]; Yamaguchi 2005 [22]; Yamanaka 1995 [23]; Yoshida 2009 [24].
(b)
CaseFerritin (ng/mL)Triglyceride (mg/dL)Bone Marrow BxAbxSteroidsIVIgEtoposideOther TxComplicationsGestation (wks), Delivery MethodMaternal/Fetal Survival
Arewa and Ajadi 2011NANA+    Amodiaquine, HAART Term, C/SNA/Yes
Chien 20094517.5386+++  Percutaneous nephrostomy tubesPTL30, C/SYes/No
Chmait 2000NANA++ + Acyclovir, AT IIIHELLP, oligo, intermittent absent UA diastolic flow30, C/SNo/Yes
Dunn 20123745358+++   IUGR30, C/SYes/Yes
Gill 1994NANA++ +   Term, NAYes/Yes
Gonzalez 2008NANANA++  AT IIIPROM36, NAYes/Yes
Hanaoka 2007587.6258+++  R-CHOP, PBSCTOligo29, C/SYes/Yes
Hannebicque-Montaigne 2012NANANA      NANA
Kim 20132890NA- BM, + spleen+++ CSA, splenectomyTAB14, NAYes/No
Klein 2014ElevatedNA- BM, + jejunal + +CSA, rituximab 31, C/SNo/Yes
Komaru 2013NANANA +    NAYes/Yes
Mayama 20141269.2NA+ +    37, VDYes/Yes
Mihara 1999NANANA ++ Acyclovir, gabexate mesilate 35, NAYes/Yes
Nakabayashi 19997240NA++ + AT IIIPreeclampsia, IUGR, Oligo29, NAYes/Yes
Perard 200715000970++++  PROM, Eclampsia, PTL, ICH30, NAYes/Yes
Samra 20154000110-++    NA, VDYes/Yes
Shukla 2013>2200588+ +   SABNAYes/No
Teng 20098926386+ +    29, C/SYes/No
Tsuda 1995NANANA      NANA
Tumian 2015NANA++++ Plasma exchange, CSA, ganciclovir 38, C/SNA
Yamaguchi 2005865.8258++++ Acyclovir, CSA 37, C/SYes/Yes
Yamanaka 1995NANANA+++ AT III, mesilate G-CSFPreeclampsia36, NAYes/Yes
Yoshida 2009NANANA +    NAYes/Yes
Case 14573269+++++CSA, azathioprine, hydroxychloroquineAbruption, IUFD, stroke21, VDNo/No
Case 28110821- BM, + liver + +  37, VDYes/Yes
Neonate demised 3-4 days after birth due to respiratory distress.
NA: data not available; NL: normal; HIV-1: human immunodeficiency virus 1; EBV: Epstein-Barr virus; SLE: systemic lupus erythematosus; AIHA: autoimmune hemolytic anemia; CMV: cytomegalovirus; VZV: varicella zoster virus; WBC: white blood cell count; Hgb: hemoglobin; Plt: platelet count; ALT: alanine transaminase; ALT: aspartate transaminase; Bx: biopsy; BM: bone marrow; Abx: antibiotics; IVIg: intravenous immunoglobulin; Tx: treatment; HAART: highly active antiretroviral therapy; AT III: antithrombin III; R-CHOP: rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone chemotherapy regimen; PVSCT: peripheral blood stem cell transplant; CSA: cyclosporine A; G-CSF: granulocyte colony stimulating factor; PTL: preterm labor; HELLP: hemolysis elevated liver enzymes and low platelet count syndrome; Oligo: oligohydramnios; UA: umbilical artery; IUGR: intrauterine growth restriction; TAB: therapeutic abortion; IUFD: intrauterine fetal demise; PROM: premature rupture of membranes; ICH: intracerebral hemorrhage; SAB: spontaneous abortion; C/S: cesarean section; VD: vaginal delivery.
References: Arewa and Ajadi 2011 [3]; Chein 2009 [4]; Chmait 2000 [5]; Dunn 2012 [6]; Gill 1994 [7]; Gonzalez 2008 [8]; Hanaoka 2007 [9]; Hannebicque-Montaigne 2012 [10]; Kim 2013 [11]; Klein 2014 [3]; Komaru 2013 [12]; Mayama 2014 [13]; Mihara 1999 [14]; Nakabayashi 1999 [15]; Perard 2007 [16]; Samra 2015 [17]; Shukla 2013 [18]; Teng 2009 [19]; Tsuda 1995 [20]; Tumian 2015 [21]; Yamaguchi 2005 [22]; Yamanaka 1995 [23]; Yoshida 2009 [24].