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 7 publications

(2 citation statements)

References 29 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…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

View full text Add to dashboard Cite

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.

show abstract

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

View full text Add to dashboard Cite

show abstract

“…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

View full text Add to dashboard Cite

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.

show abstract

Infection and Reactive Changes

2024

Bone Marrow Pathology

View full text Add to dashboard Cite

Hemophagocytic lymphohistiocytosis in the setting of HELLP Syndrome

Cited by 7 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

Infection and Reactive Changes

Contact Info

Product

Resources

About