Natural killer/T-cell lymphoma and secondary haemophagocytic lymphohistiocytosis in pregnancy

Neistadt, Barbara; Carrubba, A.R.; Zaretksy, Michael V

doi:10.1136/bcr-2018-224832

Cited by 4 publications

(2 citation statements)

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“…1 Pregnancy-related HLH is exceedingly rare and only described in case studies. [2][3][4] An expert consensus panel of pediatricians, convened by the Histiocyte society in 1994, developed the current primary HLH treatment recommendations of dexamethasone and etoposide, commonly referred to as the HLH-94 protocol. 5 Treatment of secondary HLH is extrapolated from the HLH-94 protocol, though some clinicians opt for alternative immunomodulators to etoposide given its extensive toxicity profile.…”

Section: Introductionmentioning

confidence: 99%

“…Secondary HLH may be triggered by various infections, autoimmune diseases, rheumatologic disorders, hematologic malignancies, and immunodeficiencies 1 . Pregnancy‐related HLH is exceedingly rare and only described in case studies 2–4 . An expert consensus panel of pediatricians, convened by the Histiocyte society in 1994, developed the current primary HLH treatment recommendations of dexamethasone and etoposide, commonly referred to as the HLH‐94 protocol 5 .…”

Section: Introductionmentioning

confidence: 99%

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Postpartum fevers, a rare presentation of secondary hemophagocytic lymphohistiocytosis

Lee

Pham

Karanjawala

et al. 2023

Clinical Case Reports

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Postpartum fevers, a rare presentation of secondary hemophagocytic lymphohistiocytosis

Lee

Pham

Karanjawala

et al. 2023

Clinical Case Reports

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Lymphoma-associated hemophagocytic lymphohistiocytosis (LA-HLH): a scoping review unveils clinical and diagnostic patterns of a lymphoma subgroup with poor prognosis

Knauft,

Schenk,

Ernst

et al. 2024

Leukemia

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Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome driven by pathologic activation of cytotoxic T-lymphocytes and macrophages. Despite advances in diagnostics and management, adult patients with lymphoma-associated HLH (LA-HLH) harbor particularly poor prognosis and optimal treatment remains challenging. As systematic data on LA-HLH are scarce, we aimed to synthesize research evidence by thorough analysis of the published literature in PubMed (MEDLINE-database) within the context of a scoping review. Of 595 search results, 132 articles providing information on 542 patients were reviewed and analyzed. Median patient age was 60 years (range, 18–98) with male predominance (62.7%). B- and T-NHL were equally represented (45.6% and 45.2%), Hodgkin’s lymphoma was reported in 8.9% of the cases. The majority of patients (91.6%) presented in Ann-Arbor-Stages III and IV, and bone marrow infiltration was observed in a significant proportion of patients (61.5%). Soluble CD25 levels were markedly elevated (median 10,000 U/ml), with levels beyond 10,000 U/ml indicating unfavorable prognosis for 30-day and overall survival. 66.8% of the patients died after median 5.1 months. LA-HLH remains a clinical challenge requiring specialized management. Timely diagnosis and appropriate lymphoma-specific treatment are of utmost importance to enhance patient outcomes.

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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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Natural killer/T-cell lymphoma and secondary haemophagocytic lymphohistiocytosis in pregnancy

Cited by 4 publications

References 30 publications

Postpartum fevers, a rare presentation of secondary hemophagocytic lymphohistiocytosis

Postpartum fevers, a rare presentation of secondary hemophagocytic lymphohistiocytosis

Lymphoma-associated hemophagocytic lymphohistiocytosis (LA-HLH): a scoping review unveils clinical and diagnostic patterns of a lymphoma subgroup with poor prognosis

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

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