Development of a Hematology-General Medicine Hybrid Team to Improve Care of Patients With Sickle Cell Disease.

PROBLEM: Approximately 100,000 individuals in the United States have sickle cell disease (SCD). These individuals face multiple barriers to equitable care. At Brigham and Women's Hospital, existing health inequities for these patients were compounded by admitting, rounding, and team structures that assigned patients with SCD to multiple medicine teams with a hematologist attending, leading to delays in patient care and gaps in residents' hematology knowledge. APPROACH: A hematology-general medicine hybrid team was created in September 2021 to enhance trainee knowledge, skill, and confidence in managing hematology conditions and improve the quality of care delivered to individuals with SCD. This allowed for regionalization of patients with classical hematology conditions to specific hospital floors under the care of one team with a hematologist as the attending of record. OUTCOMES: From October 1, 2021, to January 11, 2022, the majority (745/824, 90%) of in-hospital days for patients with a primary hematology diagnosis were under the care of the hematology-general medicine hybrid team. Regionalization to the home floor of the hybrid team was achieved on 331 (40%) of these 824 hospital days, consistent with regionalization rates for other teams. From October 1, 2021, to September 30, 2022, there were 128 unique patients with SCD admitted over 511 encounters and cared for by approximately 78 residents and 12 medical students. Feedback from residents reported improved knowledge in the management of hematology conditions, especially SCD. NEXT STEPS: The authors are working on a comprehensive analysis of the hybrid team's impact on trainee skill and confidence in managing SCD. The authors believe that this model can be replicated at other institutions to optimize trainee education, consolidate care, and address implicit bias against patients with SCD, even with the hematology attending as a consultant instead of as the attending of record.

Medical consult: aHUS, TTP? How to distinguish and what to do.

Immune thrombotic thrombocytopenic purpura (iTTP) caused by an autoantibody-mediated deficiency of ADAMTS13 and atypical hemolytic syndrome (aHUS) caused by alternative complement dysregulation are the most common primary thrombotic microangiopathies (TMAs). The evaluation of a patient with TMA is a medical emergency since it is critical to quickly distinguish iTTP and aHUS from other causes of TMA. Untreated iTTP is rapidly fatal, and delays in initiating complement inhibition in aHUS increase the risk of irreversible renal failure. An ADAMTS13 activity level of less than 10% is diagnostic of iTTP in the appropriate clinical setting. In settings where rapid-turnaround ADAMTS13 testing is not available, clinical features and clinical prediction tools are useful to identify patients who should receive emergent plasma exchange. We present an evidence-based approach to the initial (first 24 hours) diagnosis and management of iTTP and review the clinical and laboratory features that can be used to identify patients with aHUS who will benefit from early C5 blockade. We also discuss the potential use of complement blockade to improve outcomes in selected patients with secondary TMA.

Development of Duffy Null-Specific Absolute Neutrophil Count Reference Ranges.

This study establishes a Duffy null phenotype­specific absolute neutrophil count reference range to optimize care and improve health equity.

Absolute neutrophil count by Duffy status among healthy Black and African American adults.

Many people of African ancestry have lower absolute neutrophil counts (ANCs) without increased risk for infection. This is associated with the Duffy-null phenotype (nonexpression of the Duffy antigen on red blood cells), which is commonly found in those of African descent. Currently, there are no studies that compare the ANC of individuals with Duffy-null phenotype to those with Duffy non-null phenotypes within a self-identified Black population. The aim of this study was to assess the impact of Duffy status on ANCs based on complete blood counts with differential and Duffy testing in a healthy population of self-identified Black individuals at a single primary care center. This study found that 66.7% (80 of 120) of Black individuals have the Duffy-null phenotype and that there is a significant difference in ANCs between Duffy-null and Duffy non-null individuals (median, 2820 cells per µL vs 5005 cells per µL; P < .001). Additionally, 19 of 80 (23.8%) Duffy-null individuals had an ANC of <2000 cells per µL compared with no (0) Duffy non-null individuals. The Duffy-null phenotype is clinically insignificant; however, inappropriate reference ranges can propagate systemic racism. Therefore, we advocate for the development of Duffy-null-specific ANC reference ranges as well as replacing the term benign ethnic neutropenia with Duffy-nullassociated neutrophil count.