Table 2.
Reported cases of hemophagocytic lymphohistiocytosis during pregnancy and their characteristics.
| Publication | Underlying disease/associated infection | Gestational age (wks) | Pertinent labs | Presenting symptom or sign | Treatment | Outcome |
|---|---|---|---|---|---|---|
| Chmait et al.12 | History of necrotizing lymphadenitis; EBV (discovered postmortem) | 29 | Ferritin; NA; TG: NA; Hb: 9; WBC: 2600; Plt: 70,000; DIC: NA | Routine checkup: pancytopenia | Delivery at 30 weeks | Course complicated by DIC, Multi organ failure and death |
| Yamaguchi et al.1 | 3 HSV 2, genital herpes infection | Mid gestation | Ferritin: 865.8; TG: 180; Hb: 8; WBC: 2620; Plt: 123,000; DIC: - | High grade fever, cytopenia | Corticosteroids; Cyclosporin A | Failed corticosteroids (Remission with Cyclosporin A; Successful delivery) |
| Hanaoka et al.14 | B-cell lymphoma | 23 | Ferritin: 587.6; TG: 222; Hb: 9.5; WBC: 5810; Plt: 104,000; DIC: + | Pancytopenia, hepatosplenomegaly, high-grade fever at 23 wks gestation | Emergent C-section (fetal distress); R-CHOP chemotherapy | Remission; successful C-section |
| Perard et al.10 | Systemic lupus erythematosus | 22 | Ferritin: 15,000; TG: 9.7; Hb: 9.2; WBC: 3500; Plt: 80,000, DIC: - | High grade fevers | Corticosteroids; IVIG 3 doses | No improvement with steroids; premature delivery; successful remission after third IVIG dose (and/or delivery) |
| Chien et al.15 | Unclear etiology | 23 | Ferritin: 1.36; TG: 386; Hb: 7.4; WBC: 8900, Plt: 11,000; DIC: - | High grade fever, cytopenia | Cesarean delivery | Preterm labor; successful C-section delivery; complete remission |
| Teng et al.16 | Autoimmune hemolytic anemia at 23 weeks of gestation | 23 | Ferritin: 8926; TG: 386; Hb: 7.4; WBC: 8900; Plt: 109,000; DIC: - | High grade fever, cytopenia | Corticosteroids | Failed corticosteroids; remission post termination of pregnancy |
| Arewa et al.17 | HIV | 21 | Ferritin: NA; TG: NA; Hb: 6; WBC: 4200; Plt: 125,000; DIC: NA | Jaundice, fever, abdominal pain | HAART; delivery | Complete remission |
| Dunn et al.11 | Still's disease | 19 | Ferritin: 3745; TG: 358; Hb: 9.8; ANC: 400; Plt: 343,000;DIC: - | Rash, fever, and headache | High-dose corticosteroids | Stable blood counts; successful delivery |
| Shukla et al.18 | Unclear etiology | 10 | Ferritin: 2200; TG: 588; Hb: 6.3; WBC: 1880; Plt: 18,000; DIC: - | Moderate grade fever for 2 wks | Corticosteroids; spontaneous abortion | Failed steroids; remission after abortion |
| Mayama et al.19 | Parvovirus B19 | 21 | Ferritin: 1269.2; TG: NA; Hb: 4.2; WBC: 600; Plt: 83,000; DIC: - | Fever and pancytopenia | Corticosteroids | Remission with steroids |
| Our patient | Unclear etiology | Ferritin: 4000; TG: 110; Hb: 9.9; WBC: 1300; Plt:125,000; DIC:- | Fever and pancytopenia | Corticosteroids | Remission with steroids |
NA, Non-available; Hb, hemoglobin (g/dL); WBC, white blood cell count (/mL); plt, platelet count (mm3); TG, triglycerides (mg/dL); DIC, disseminated intravascular coagulopathy; ANC, absolute neutrophile count (/mL).