Clinical and laboratory outcomes following total or partial splenectomy in patients with hereditary spherocytosis

Tripodi, Serena Ilaria; Shamberger, Robert C.; Heeney, Matthew M.; Tubman, Venée N.

doi:10.1080/08880018.2019.1637983

Cited by 4 publications

(7 citation statements)

References 19 publications

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“…By monitoring the extent of anemia and obtaining the population-wide distribution of RBC quality, it would be possible to predict the extent to which a partial or complete splenectomy improves physiological outcomes. Such information may provide insights into the hitherto unexplained variability of HS patient responses to splenectomy (54).…”

Section: Discussionmentioning

confidence: 99%

Single-cell O ₂ exchange imaging shows that cytoplasmic diffusion is a dominant barrier to efficient gas transport in red blood cells

Richardson

Hulı́ková

Proven

et al. 2020

Proc. Natl. Acad. Sci. U.S.A.

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Disorders of oxygen transport are commonly attributed to inadequate carrying capacity (anemia) but may also relate to inefficient gas exchange by red blood cells (RBCs), a process that is poorly characterized yet assumed to be rapid. Without direct measurements of gas exchange at the single-cell level, the barriers to O2 transport and their relationship with hematological disorders remain ill defined. We developed a method to track the flow of O2 in individual RBCs by combining ultrarapid solution switching (to manipulate gas tension) with single-cell O2 saturation fluorescence microscopy. O2 unloading from RBCs was considerably slower than previously estimated in acellular hemoglobin solutions, indicating the presence of diffusional barriers in intact cells. Rate-limiting diffusion across cytoplasm was demonstrated by osmotically induced changes to hemoglobin concentration (i.e., diffusive tortuosity) and cell size (i.e., diffusion pathlength) and by comparing wild-type cells with hemoglobin H (HbH) thalassemia (shorter pathlength and reduced tortuosity) and hereditary spherocytosis (HS; expanded pathlength). Analysis of the distribution of O2 unloading rates in HS RBCs identified a subpopulation of spherocytes with greatly impaired gas exchange. Tortuosity imposed by hemoglobin was verified by demonstrating restricted diffusivity of CO2, an acidic gas, from the dissipative spread of photolytically uncaged H+ ions across cytoplasm. Our findings indicate that cytoplasmic diffusion, determined by pathlength and tortuosity, is a major barrier to efficient gas handling by RBCs. Consequently, changes in RBC shape and hemoglobin concentration, which are common manifestations of hematological disorders, can have hitherto unrecognized and clinically significant implications on gas exchange.

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Section: Discussionmentioning

confidence: 99%

Single-cell O ₂ exchange imaging shows that cytoplasmic diffusion is a dominant barrier to efficient gas transport in red blood cells

Richardson

Hulı́ková

Proven

et al. 2020

Proc. Natl. Acad. Sci. U.S.A.

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“…Most existing studies are limited by use of single institution reports, absence of standardized data, or limited follow-up. 11,12 To address this research gap, our consortium developed a multisite, web-based prospective patient registry based on use of standardized data definitions for children with CHA requiring surgery. We have previously demonstrated excellent hematologic and clinical outcomes in children with HS after total and partial splenectomy over one year of follow-up, with clinical outcomes improved in children with sickle cell disease.…”

Section: Discussionmentioning

confidence: 99%

“…[3][4][5][6][7] However, the long-term outcomes of TS and PS remain poorly defined, as most reports are from single institutions, retrospective, and limited by several biases. [8][9][10][11][12] For many clinical practices, there is great value using data from clinical and administrative datasets to predict expected health outcomes. 5,8 However, the use of common datasets for the study of less frequent conditions is difficult, as these data sets often do not record outcomes considered most important by clinicians and families.…”

Section: Introductionmentioning

confidence: 99%

“…There is continued interest in partial splenectomy (PS) due to risks of overwhelming postsplenectomy sepsis, venous thromboembolism, and pulmonary hypertension from TS 3–7 . However, the long‐term outcomes of TS and PS remain poorly defined, as most reports are from single institutions, retrospective, and limited by several biases 8–12 …”

Section: Introductionmentioning

confidence: 99%

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Long‐term hematologic and clinical outcomes of splenectomy in children with hereditary spherocytosis and sickle cell disease

Hall

Reiter

Englum

et al. 2020

Pediatric Blood & Cancer

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Background: Total splenectomy (TS) and partial splenectomy (PS) are used for children with congenital hemolytic anemia (CHA), although the long-term outcomes of these procedures are poorly defined. This report describes long-term outcomes of children with CHA requiring TS or PS. Procedure:We collected data from children ages 2-17 with hereditary spherocytosis (HS) or sickle cell disease (SCD) requiring TS or PS from 1996 to 2016 from 14 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a prospective, observational patient registry. We summarized hematologic outcomes, clinical outcomes, and adverse events to 5 years after surgery. Hematologic outcomes were compared using mixed effects modeling.Results: Over the study period, 110 children with HS and 97 children with SCD underwent TS or PS. From preoperatively compared to postoperatively, children with HS increased their mean hemoglobin level by 3.4 g/dL, decreased their mean reticulocyte percentage by 6.7%, and decreased their mean bilirubin by 2.4mg/dL. Hematologic improvements and improved clinical outcomes were sustained over 5 years of follow-up. For children with SCD, there was no change in hemoglobin after PS or TS following surgery, although all clinical outcomes were improved. Over 5 years, there was one child with HS and 5 children with SCD who developed post-splenectomy sepsis.Conclusions: For children with HS, there are excellent long-term hematologic and clinical outcomes following either PS or TS. Although hemoglobin levels do not change after TS or PS in SCD, the long-term clinical outcomes for children with SCD are favorable.

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“…A longitudinal study conducted by Bisharat et al found that approximately half to two-thirds of infections following splenectomy occurred within the first two years with an average of (20) reported that the time to infection is lower in patients with splenectomy due to hematologic problems (median interval of 20 months) compared to post-traumatic splenectomy (median interval of 50 months). Partially removing the spleen is thought to be associated with a lower risk of infection, but there are still some controversies (21,22). In our study, all cases had undergone total splenectomy.…”

Section: Discussionmentioning

confidence: 68%

Bacterial Etiology of Fever Episodes of Splenectomized Patients in Three Medical Centers in the City of Mashhad in Northeastern Iran

Arian

Moniri

Najafi

et al. 2022

Arch Clin Infect Dis

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Background: Many medical and surgical conditions may need to be treated with splenectomy. As this lymphoid tissue plays an important role in controlling various infections, and many life-threatening infections can occur in the absence of the spleen, any episode of fever should be taken seriously. Objectives: This study aims to assess the bacterial etiology of fever episodes in splenectomized patients in three medical centers in Mashhad, a city in northeastern Iran. Methods: Between 2006 and 2017, splenectomized patients in Imam Reza, Ghaem, and Dr. Sheikh hospitals were included in a cross-sectional study. Data collected included, age at splenectomy, hospitalization duration, indications for admission to the intensive care unit, vital signs at admission, bacterial species responsible for sepsis, times of hospitalization due to fever episodes, clinical signs and symptoms, antibiotic prophylaxis, and outcomes at six and one years. The data were analyzed using SPSS Statistics 20. Results: A total of 280 splenectomized patients were reviewed, and 23 of them had episodes of fever. The most common causes of splenectomy were spleen masses and idiopathic thrombocytopenic purpura (ITP), each accounting for 17.4% of cases. The mean age of the patients was 24.2 ± 1.6 years. 47.8% of the patients were male, and 52.2% were female. The median admission duration was seven days. A majority of admissions were due to intra-abdominal infections (26.7%), pneumonia (13.3%), and bacteremia (10.0%). There were 30 episodes of fever recorded, of which 2 (6.7%) resulted in death. Blood culture was positive in four cases (13.3%) for Streptococcus pneumoniae, Staphylococcus aureus, Citrobacter, and Brucella. A suitable antibiotic coverage was obtained in 13.3% of cases (i.e., ceftriaxone + vancomycin or fluoroquinolone + vancomycin), and a minimum suitable empiric coverage was obtained in 10.0% of cases, and no appropriate antibiotic coverage was obtained in 76.7% of cases. Conclusions: The present study highlights widespread inappropriate empiric therapy of fever episodes in splenectomized patients, as well as a lack of due attention to timely sample collection before antibiotic administration. Despite this, the isolated organisms were varied and included S. pneumoniae, S. aureus, coagulase negative staphylococci, P. aeruginosa, Brucella, and Citrobacter.

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Clinical and laboratory outcomes following total or partial splenectomy in patients with hereditary spherocytosis

Cited by 4 publications

References 19 publications

Single-cell O ₂ exchange imaging shows that cytoplasmic diffusion is a dominant barrier to efficient gas transport in red blood cells

Single-cell O ₂ exchange imaging shows that cytoplasmic diffusion is a dominant barrier to efficient gas transport in red blood cells

Long‐term hematologic and clinical outcomes of splenectomy in children with hereditary spherocytosis and sickle cell disease

Bacterial Etiology of Fever Episodes of Splenectomized Patients in Three Medical Centers in the City of Mashhad in Northeastern Iran

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Clinical and laboratory outcomes following total or partial splenectomy in patients with hereditary spherocytosis

Cited by 4 publications

References 19 publications

Single-cell O 2 exchange imaging shows that cytoplasmic diffusion is a dominant barrier to efficient gas transport in red blood cells

Single-cell O 2 exchange imaging shows that cytoplasmic diffusion is a dominant barrier to efficient gas transport in red blood cells

Long‐term hematologic and clinical outcomes of splenectomy in children with hereditary spherocytosis and sickle cell disease

Bacterial Etiology of Fever Episodes of Splenectomized Patients in Three Medical Centers in the City of Mashhad in Northeastern Iran

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Single-cell O ₂ exchange imaging shows that cytoplasmic diffusion is a dominant barrier to efficient gas transport in red blood cells

Single-cell O ₂ exchange imaging shows that cytoplasmic diffusion is a dominant barrier to efficient gas transport in red blood cells