Assistant Professor Penn State Health, United States
Introduction: Hemophagocytic lymphohistiocytosis (HLH) is an inflammatory syndrome triggered by malignancies, autoimmune conditions, or infection. In critical care, HLH is often mimicked by other more common syndromes that cause multi-organ dysfunction, such as sepsis or cardiovascular dysfunction. Diagnosis is essential as the management of HLH is profoundly different. In this case, we present a patient with angioimmunoblastic T cell lymphoma (AITL) who developed profound shock due to HLH and had an impressive clinical response to its treatment.
Description: A 42 year old woman with AITL with previous treatment of CHOEP and ICE chemotherapy presented with dyspnea, hives, tongue, and facial edema. Initially she was treated with antibiotics, corticosteroids, and epinephrine. She then declined in her respiratory status and developed stridor. Ultimately she was intubated and sent to the MICU for further care. After intubation, she developed profound vasoplegic shock which required escalating vasopressor support. Given the degree of shock and prior malignancy, the consideration of HLH contributing to her current clinical state ensued. Markers for HLH were elevated, including ferritin (4740ng/ml) and IL-2 (128,535 pg/mL). After initiation of etoposide and dexamethasone, she had not only improvement in facial edema and lung compliance, but vasopressor support. She was weaned from vasopressor support and extubated after 10 days of mechanical ventilation.
Discussion: AITL is a less common form of non-Hodgkins lymphoma and there is little literature about the associations of HLH. Of these cases, HLH has been seen both at the time of diagnosis and after treatment has been started. Challenges associated with the diagnosis include similarities of clinical syndromes of relapsed lymphoma, septic shock, and HLH. As there was rapid clinical deterioration despite the use of antibiotics, HLH had to be strongly considered.. There was a profound clinical response to etoposide, likely resulting in the deactivation of circulating inflammatory cytokines which lead to profound vasoplegic shock. HLH continues to be a challenging condition to diagnose and treat with high rates of mortality, but having a high index of suspicion in those with AITL can lead to earlier diagnosis and management with profound clinical response.
