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Pulmonary hypertension in sickle cell disease (SCD) is a complex phenomenon resulting from multiple overlapping etiologies, including pulmonary vasoconstriction in the setting of chronic hemolytic anemia, diastolic dysfunction, and chronic thromboembolic disease. The presence of pulmonary hypertension of any cause in SCD confers a significant increase in mortality risk. Evidence to guide the management of patients with sickle cell disease and chronic thromboembolic pulmonary hypertension (CTEPH) is scant and largely the realm of case reports and small case series. Centered on a discussion of a complex young patient with hemoglobin hemoglobin SC who ultimately underwent treatment with pulmonary thromboendarterectomy, we review the available literature to guide management and discuss and overview of treatment of CTEPH in SCD, considering the unique considerations and challenges facing patients suffering from this multisystem disease. [ABSTRACT FROM AUTHOR]

Delayed hemolytic transfusion reaction (DHTR) and hyperhemolysis syndrome (HHS) are both complications of red blood cell transfusions in patients with sickle cell disease.Clinically, both present with hemolysis and can be difficult to differentiate. Hemoglobin electrophoresis may aid in the diagnosis. Herein we describe a case in which a patient with hemoglobin SC disease presented with features of severe hemolysis several days after initiation of red blood cell exchange. Increase in reticulocyte count and complete absence of hemoglobin A on electrophoresis during this event supported the diagnosis of severe DHTR, indicating a rapid and selective destruction of the transfused red blood cells. Ability to interpret the hemoglobin electrophoresis can help clinicians distinguish between these two severe transfusion complications in patients living with sickle cell disease. It is important to identify the presence or absence of concomitant HHS, as patients with HHS tend to have a worse prognosis and there is a higher rate of recurrence of HHS with subsequent transfusions. Accurate diagnosis can lead to prompt management and decrease morbidity and mortality. • Severe delayed hemolytic transfusion reaction is a potential complication of transfusion. • Rarely, this complication can progress into hyperhemolysis syndrome, especially in patients with sickle cell disease. • Hemoglobin electrophoresis may help differentiate delayed hemolytic transfusion reaction from hyperhemolytic syndrome. [ABSTRACT FROM AUTHOR]

Background: Venous thromboembolism (VTE) is a recognized complication of sickle cell disease (SCD), yet the optimal pharmacologic anticoagulant is unknown. Methods: A retrospective single-institution cohort study of patients with SCD complicated by first VTE from January 2009 through July 2017 was performed using ICD 9/10 codes. Data collected included the anticoagulant used, VTE recurrence, and incidence of bleeding. Results: 109 patients with VTE were identified. SCD genotypes included HbSS in 92 (84%), HbSC in 13 (12%), and HbS-β+ thalassemia in 4 (4%). After the initial VTE event, 32 patients received a vitamin K antagonist (VKA), 34 for low-molecular-weight heparin (LMWH), and 43 for direct oral anticoagulants (DOACs). 16 patients (15%) experienced a clinically significant bleeding event, including 9 on VKA, 5 on LMWH, and 2 on DOACs. At a median follow-up of 11.8 (range, 3.4–60) months, 33 patients had a recurrent VTE, including 10 on VKA, 10 on LMWH, and 13 on DOACs (p = 0.833). Bleeding incidence was least with the DOACs, which were associated with fewer bleeding events (OR 0.22), and greatest with VKA (OR 1.55) (p < 0.05). Conclusion: There was no difference between VTE recurrence and choice of anticoagulation in SCD patients with VTE. Bleeding events were lower for DOACs compared to VKA or LMWH. [ABSTRACT FROM AUTHOR]

A 25-year-old female with sickle cell anemia presented with respiratory failure due to acute chest syndrome (ACS). Given her severely compromised cardiopulmonary status, she was started on veno-venous extracorporeal membrane oxygenation (VV-ECMO). After 20 days, the patient’s respiratory status improved and she was successfully decannulated. Veno-venous extracorporeal membrane oxygenation can be utilized for severe ACS in adult patients with sickle cell disease. Prompt initiation of this modality may improve outcomes in adult patients with sickle cell disease complicated by life threatening ACS. [ABSTRACT FROM AUTHOR]

The article describes the case of a 28-year-old African American man with sickle cell disease (SCD) who developed septicemia with Vibrio (V.) vulnificus. Topics discussed include complication by recurrent vaso-occlusive pain crises as well as hepatic dysfunction and cirrhosis secondary to iron overload, association of V. vulnificus with shellfish-associated death in the U.S., and advise to hematologists caring for patients with SCD complicated by iron overload.

Objective: To highlight the risk of complications among women with sickle cell anemia (SCA) receiving fertility preservation treatment (FPT) before hematopoietic stem cell transplant (HSCT).Design: Single-center case series.Setting: Academic fertility center.Patient(s): Women aged 15-32 years with SCA undergoing FPT before HSCT.Intervention(s): Retrospective, systematic review.Main Outcome Measure(s): FPT modality, SCA complications during FPT.Result(s): Over an 8-year period (2009-2017), seven women with SCA ages 15-32 years (mean 28.5 years) underwent FPT with embryo cryopreservation (n = 1), oocyte cryopreservation (n = 4), and ovarian tissue cryopreservation (n = 2). The five women subjects who underwent oocyte or embryo cryopreservation were treated with an antagonist controlled ovarian hyperstimulation protocol and individualized gonadotropin dosing. The trigger medications included leuprolide acetate (n = 2), and human chorionic gonadotropin (n = 3). Most patients (n = 5) received a disease-modifying therapy for SCA (hydroxyurea or chronic transfusions) before FPT. Three patients experienced periprocedural SCA complications that included life-threatening respiratory failure, painful crisis requiring interruption of a stimulation cycle, and severe postharvest painful crisis.Conclusion(s): Women with SCA may choose to undergo diverse FPT strategies before HSCT and are at risk for serious SCA-related complications. Evidence-based strategies to mitigate SCA-related morbidity and to optimize fertility preservation outcomes are needed. [ABSTRACT FROM AUTHOR]