Hemophagocytic lymphohistiocytosis (HLH) is a rare and often misdiagnosed syndrome during pregnancy, characterized by nonspecific clinical manifestations that contribute to high maternal mortality rates. We present the case of a 31-year-old primigravida who initially presented with fever and was diagnosed with pneumonia, but later succumbed to HLH associated with T-cell lymphoma. This report underscores the diagnostic complexities of HLH, particularly in identifying its association with malignancies, and emphasizes the need for early recognition and intervention to mitigate its devastating outcomes.
Introduction:
Hemophagocytic lymphohistiocytosis (HLH) is a severe syndrome characterized by overactivation of histiocytes and lymphocytes, typically seen in infancy but can occur in adults secondary to various triggers, including infections and malignancies. Malignancy-associated HLH (M-HLH), particularly with hematologic neoplasms like T-cell lymphoma, presents diagnostic challenges due to overlapping clinical features with other conditions, leading to delays in treatment and poor outcomes.
Case Presentation:
A 31-year-old primigravida presented at 34 weeks gestation with fever, lethargy, and headache. Initially diagnosed with pneumonia, her condition deteriorated despite antibiotic therapy. Subsequent investigations revealed features consistent with HLH and T-cell lymphoma, confirmed posthumously. Despite efforts including chemotherapy, she succumbed to multiorgan failure shortly after delivery.
Discussion:
This case illustrates the diagnostic journey of HLH in pregnancy, highlighting the importance of clinical suspicion and advanced diagnostic modalities such as CT and MRI to differentiate HLH from other conditions. Challenges in timely diagnosis, exacerbated by nonspecific symptoms and overlapping laboratory findings, underscore the critical need for expanded diagnostic criteria and heightened awareness among healthcare providers.
Conclusion:
Early recognition and multidisciplinary management are crucial in mitigating the devastating effects of HLH during pregnancy, particularly when associated with malignancies like T-cell lymphoma. Enhanced diagnostic protocols and interdisciplinary collaboration are essential to improving outcomes and guiding effective treatment strategies in such complex clinical scenarios.
