Hemophagocytic lymphohistiocytosis in the setting of HELLP Syndrome

Sarkissian, Sarmen; Khan, Yasir; Farrell, Daniel; Constable, David; Brém, Elizabeth

doi:10.1002/ccr3.1828

Cited by 6 publications

(2 citation statements)

References 29 publications

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“…In previous reports, four cases were misdiagnosed with HELLP syndrome instead of sHLH, and emergency cesarean section was performed. However, as their clinical condition did not improve even after delivery, the patients were rediagnosed with sHLH later [ 24 , 33 , 39 , 40 ]. Therefore, it is important to consider sHLH when the patient shows no improvement even after pregnancy termination.…”

Section: Discussionmentioning

confidence: 99%

Chronic active Epstein–Barr virus-associated secondary hemophagocytic lymphohistiocytosis in pregnancy: a case report

Takahashi

Makino

Iizuka

et al. 2021

BMC Pregnancy Childbirth

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Background Secondary hemophagocytic lymphohistiocytosis (sHLH) is a rare and fatal disease characterized by uncontrolled immune cell activation that can lead to a cytokine storm. Unfortunately, this condition can occur even during pregnancy, threatening both maternal and fetal lives. Case presentation A 23-year-old nulliparous woman at 26 weeks of gestation presented with continuous fever, coughing, and sore throat. Upon arrival at our hospital, her temperature was >38°C and laboratory findings indicated cytopenia (neutrophil count, 779/μL; hemoglobin level, 10.2 g/dL; platelet count, 29,000/μL), elevated ferritin level (1,308 ng/mL), and elevated soluble interleukin-2 receptor level (11,200 U/mL). Computed tomography showed marked splenomegaly. Bone marrow examination revealed hemophagocytosis, and blood examination showed a plasma Epstein–Barr virus (EBV) DNA level of 8.9 × 105 copies/μg. The monoclonal proliferation of EBV-infected T cells was confirmed by Southern blotting, and the patient was diagnosed with chronic active EBV-associated sHLH and T-cell lymphoproliferative disease. Immediately after admission, the patient’s condition suddenly deteriorated. She developed shock and disseminated intravascular coagulation, requiring endotracheal intubation along with methylprednisolone pulse and etoposide therapy. Although the patient recovered, she delivered a stillborn baby. After delivery, she was treated with reduced-dose dexamethasone, etoposide, ifosfamide, and carboplatin (DeVIC) and steroid (dexamethasone), methotrexate, ifosfamide, L-asparaginase, and etoposide (SMILE) chemotherapies. Five months after diagnosis, she received human leukocyte antigen-haploidentical allogeneic bone marrow transplantation from her sister. She remains in remission for 5 months from the time of transplantation to the present. Conclusions sHLH, which may cause maternal and fetal death, should be carefully considered in critically ill pregnant women, particularly those presenting with continuous fever and cytopenia.

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Section: Discussionmentioning

confidence: 99%

Chronic active Epstein–Barr virus-associated secondary hemophagocytic lymphohistiocytosis in pregnancy: a case report

Takahashi

Makino

Iizuka

et al. 2021

BMC Pregnancy Childbirth

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“…Our patient was diagnosed with a secondary HLH induced by pregnancy after negative TORCH and Epstein-Barr results, negative antibodies, and negative screening for malignancy (negative tumor markers and images). According to the literature, HLH related to pregnancy may be a common cause this disease [16][17][18].…”

Section: Discussionmentioning

confidence: 99%

Pregnancy-Induced Hemophagocytic Lymphohistiocytosis: A Case Report

Sánchez-Ato

Cuestas-Quiroz

Agurto-Saldaña

et al. 2019

J Obstet Gynecol India

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We present a 23-year-old pregnant woman gravida 3, para 1 27 weeks of gestation complaining of intermittent fever, hyporexia, and vomiting since one week. She had history of malar rash that increased with sun exposure since adolescence. Physical examination revealed fever, pulse of 99 beats per min, blood pressure of 80/50 mmHg, and breathing frequency of 19/min. She was pale, a maculopapular skin rash over both cheeks was observed. There was no lymphadenopathy or joint pain. The liver was palpable 2 cm below the right costal margin and tender; the spleen was not palpable. The uterus was gravid, normal tone, fetal heart rate was 158 beats per min, fetal movement was present, and uterine height was 23 cm. She was awake, oriented, and responsive. Laboratory results showed a hemoglobin of 11.1 g/dL, leukocytes of 6220/mm 3 , a platelet count of 240,000/mm 3 , and C protein reactive of 13.3 mg/L, urinary sediment showed leukocyturia without nitrites.

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Dexamethasone

2019

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A 30-year-old woman developed gastritis following treatment with dexamethasone for haemophagocytic lymphohistiocytosis (HLH). The woman was hospitalised with uterine contractions at 35 weeks and two days of gestation. Five days prior to the admission, she had presented with presumed communityacquired pneumonia and had started receiving treatment with azithromycin. Her co-morbidity included herpes simplex virus-1 (HSV-1) infection. Upon admission, clinical investigation led to a presumptive diagnosis of acute fatty liver of pregnancy (AFLP). Consequently, she underwent emergent caesarean section and delivered a healthy male infant. Upon subsequent investigation, her diagnosis was changed from AFLP to HELLP (haemolysis, elevated liver enzymes, low platelets) syndrome. Post-delivery, she was treated with ampicillin, gentamicin and clindamycin due to suspicion of chorioamnionitis. Subsequent investigation revealed HLH. Consequently, she started receiving treatment with HLH-94 protocol consisting of dexamethasone [route and dosage not stated] and etoposide. She was also diagnosed with concurrent Epstein-Barr virus (EBV) and Cytomegalovirus (CMV) infection and started receiving treatment with ganciclovir. Later, she was discharged with outpatient etoposide (week 6 of HLH-94 protocol). However, after three days, she was hospitalised again with EBV viraemia. The HLH-94 protocol was restarted. She was also treated with concurrent rituximab. Subsequently, she developed gastrointestinal bleeding due to corticosteroidinduced gastritis [duration of treatment to reaction onset not clearly stated]. She was also diagnosed with concurrent supraventricular tachycardia, thrombocytopenia and hypoxic respiratory distress. Therefore, she was admitted to the ICU. She was also diagnosed with concurrent neutropenia, multidrug resistant bacteraemia, sepsis, septic shock and acute respiratory distress syndrome. She was treated with unspecified granulocyte colony stimulating factor, granulocytemacrophage colony stimulating factor, antibiotics, vasopressors and mechanical intubation. However, she passed on day 24 of second admission due to ventricular fibrillation arrest [reaction outcome not stated].

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Hemophagocytic lymphohistiocytosis in the setting of HELLP Syndrome

Cited by 6 publications

References 29 publications

Chronic active Epstein–Barr virus-associated secondary hemophagocytic lymphohistiocytosis in pregnancy: a case report

Chronic active Epstein–Barr virus-associated secondary hemophagocytic lymphohistiocytosis in pregnancy: a case report

Pregnancy-Induced Hemophagocytic Lymphohistiocytosis: A Case Report

Dexamethasone

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