Background Hemophagocytic lymphohistiocytosis (HLH) is a rare disease resulting from the overactivation of the immune system due to under regulation of cytotoxic lymphocytes, macrophages and natural killer (NK) cells. HLH is associated with malignancies, infections, autoimmune disorders and rarely AIDS and is rapidly fatal. Case presentation This case report identified a 53 year old male with acquired immunodeficiency syndrome (AIDS) who presented with neutropenic fever of unknown origin. He had two previous hospitalizations prior to the hospitalization diagnosing HLH. The first led to a diagnosis of drug fevers in the setting of treatment for thrombotic thrombocytopenic purpura and subsequent hospitalization led to empiric treatment of hospital acquired pneumonia after workup for intermittent fevers was negative. He was discharged but readmitted 10 days after for recurrence of neutropenic fevers. During this final hospitalization, he was found to have elevated liver enzymes, ferritin, triglycerides and soluble IL-2 receptor with persistent fevers, new splenomegaly and bicytopenia meeting the 2004 HLH criteria. Bone marrow biopsy confirmed the diagnosis of HLH as well as EBV associated large B-cell lymphoma. The patient improved on treatment with steroids, rituximab, tocilizumab, and chemotherapy but ultimately passed away due to refractory septic shock from multi-drug resistant Klebsiella pneumoniae. Conclusion This novel case highlights a patient diagnosed with HLH in the setting of several risk factors for the disease, including AIDS, B-cell lymphoma and EBV. Additionally, this case highlights the importance of early consideration of HLH in the setting of neutropenic fever without clear infectious etiology and search for malignancy associated reasons for HLH especially in immunocompromised patients.

Introduction: On January 1, 2021, the Centers for Medicare and Medicaid Services (CMS) implemented the Hospital Price Transparency Final Rule to promote price competition and improve hospital care affordability. Hospitals in the US are required to disclose, among other items, the cash prices and the payer-specific negotiated prices for CMS-specified, high-volume common services. We investigated the compliance rate and descriptive costs for breast cancer services in the central New Jersey region. Methods: We collected CMS-specified hospital services representing 4 unique Current Procedural Terminology (CPT)/diagnosis related group codes (screening mammography, US guided biopsy, mastectomy, partial lumpectomy). Cash prices and payer-specific negotiated prices for these services were obtained from Turquoise Health, a data service company that specializes in collecting pricing information from hospitals. We collected the median cash price, the proportion of hospitals for which the cash price was lower than its median commercial negotiated price, interquartile ranges (IQR) for cash prices across all services by practice type, and the correlation between cash price of service and neighborhood poverty level. Results: 106 hospitals in a 50-mile radius from central New Jersey were reviewed, representing 22 academic and 84 community clinics. Of these, only 4 hospitals disclosed both their cash price and commercially negotiated price for all services. Overall, there was a correlation for mammography cash price and neighborhood level of poverty (Rs -0.34, p = 0.026). No correlations were noted for the other services. Cash prices varied substantially across hospitals, as evidenced by large IQR for US-guided biopsy $ 1877.19 (1647.05 – 5388.2), mastectomy $6417.00 (4847.34 – 48166.69), and lumpectomy $3820.00 (3021.76 – 17041.84) in academic centers. When compared to community hospitals, academic institutions were more likely to set their cash prices below negotiated insurance prices. Discussion: Of the 106 hospitals investigated, only 4 disclosed both their cash price and commercially negotiated price. As evidenced by the negative correlation between the cash median cash price of screening mammography and neighborhood level of poverty, hospitals encourage entry into the health system. Unfortunately, downstream costs for diagnosis and treatment are unpredictable and present major challenges in preventing financial toxicity and assuring health equity. Because of its descriptive nature, this study was unable to identify factors or outcomes associated with the cash price variation. Uninsured or underinsured patients who choose to take the cash price offered by hospitals remain extremely vulnerable. Citation Format: Alexandra Noveihed, Naveena Lall, Qasim S. Hussaini, Roy Elias, Arjun Gupta, Ramy Sedhom. Cash, Commercial Negotiated Prices, and Correlations with Neighborhood Poverty Levels for Shoppable Breast Cancer Services [abstract]. In: Proceedings of the 2022 San Antonio Breast Cancer Symposium; 2022 Dec 6-10; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2023;83(5 Suppl):Abstract nr P6-08-10.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare disease resulting from the overactivation of the immune system due to under regulation of cytotoxic lymphocytes, macrophages and natural killer (NK) cells. HLH is associated with malignancies, infections, autoimmune disorders and rarely AIDS and is rapidly fatal. Case Presentation: This case report identified a 53 year old male with acquired immunodeficiency syndrome (AIDS) who presented with neutropenic fever of unknown origin. He had two previous hospitalizations prior to the hospitalization diagnosing HLH. The first led to a diagnosis of drug fevers in the setting of treatment for thrombotic thrombocytopenic purpura and subsequent hospitalization led to empiric treatment of hospital acquired pneumonia after workup for intermittent fevers was negative. He was discharged but readmitted ten days after for recurrence of neutropenic fevers. During this final hospitalization, he was found to have elevated liver enzymes, ferritin, triglycerides and soluble IL-2 receptor with persistent fevers, new splenomegaly and bicytopenia meeting the 2004 HLH criteria. Bone marrow biopsy confirmed the diagnosis of HLH as well as EBV associated large B-cell lymphoma. The patient improved on treatment with steroids, rituximab, tocilizumab, and chemotherapy but ultimately passed away due to refractory septic shock from multi-drug resistant Klebsiella pneumoniae. Conclusion: This novel case highlights a patient diagnosed with HLH in the setting of several risk factors for the disease, including AIDS, B-cell lymphoma and EBV. Additionally, this case highlights the importance of early consideration of HLH in the setting of neutropenic fever without clear infectious etiology and search for malignancy associated reasons for HLH especially in immunocompromised patients.