Sickle Cell Disease Complicated by Iron Overload: An Under-Recognized Risk Factor for Vibrio vulnificus Infection

A 29-year-old woman with known sickle cell disease (SCD) and iatrogenic iron overload presented to the emergency department with a recurrent pain crisis and fever. Blood cultures obtained at a recent prior admission for the same complaints grew M avium. Bone marrow biopsy revealed non-caseating granulomas, but stains for mycobacteria and fungi were negative. Disseminated non-tuberculous mycobacterial infections (NTMIs) occur almost exclusively in immunosuppressed patients. SCD is not considered a risk factor for the development of disseminated NTMIs, making diagnosis challenging in this population. However, a number of case reports describing disseminated NTMIs in patients with SCD have been published. This case adds to the current literature, suggesting SCD with iatrogenic iron overload is a possible risk factor for disseminated NTMIs. Potential mechanisms for this increased risk include 1) functional asplenia, 2) iatrogenic iron overload, 3) chronic indwelling central venous catheters, and 4) hydroxyurea use. Further investigation is required to describe the strength and mechanism of the relationship between SCD and disseminated NTMIs.

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Poor Access to Care Is a Risk Factor for Iron Overload in Adults with Sickle Cell Disease in Southern California

Blood ◽

10.1182/blood-2018-99-112292 ◽

2018 ◽

Vol 132 (Supplement 1) ◽

pp. 3527-3527

Author(s):

Susan Claster ◽

Michael Roland ◽

Jeri Tucker ◽

Marlene Espinoza ◽

Ellen Rothman ◽

...

Keyword(s):

Risk Factor ◽

Los Angeles ◽

Sickle Cell Disease ◽

Iron Overload ◽

Access To Care ◽

Sickle Cell ◽

Southern California ◽

Morbidity And Mortality ◽

Beta Thalassemia ◽

Cell Disease

Abstract The lack of access to quality comprehensive care is a major problem for adults with sickle cell disease (SCD) and contributes to increased morbidity and mortality. This problem is especially acute in Los Angeles where the average lifespan is 10 years shorter than the national average. Without access to disease modifying therapies, patients utilize Emergency Department services and are repeatedly hospitalized. During inpatient stays many may be inappropriately transfused. The result can be severe iron overload, a significant cause of morbidity and mortality. Inattention to iron chelation in patients who are not cared for by knowledgeable SCD providers perpetuates this issue. In 2016 we cared for a new patient at our clinic in South Los Angeles who previously had a liver transplant due iron overload. This patient received multiple transfusions during her lifetime and was never seen in a comprehensive SCD clinic. This experience prompted us to evaluate our population of clinic patients to assess how lack of access to care might be a risk factor for iron overload. Methods: We performed a chart review of all patients with SCD who presented to either of two adult sickle cell clinics in Southern California: MLK Jr. Outpatient Center in Los Angeles and Center for Inherited Blood Disorders (CIBD) in Orange. We examined records from 2013-2018 from CIBD and from 2016-2018 from MLK. Patients were considered evaluable if they had a least one visit to clinic with labs that included iron studies and /or MRI. Iron saturations greater than 50% and /or a ferritin greater than 1000 was deemed indicative of iron overload when combined with a history of prior transfusions. Lack of access was defined as the absence of appropriate care for SCD for at least 12 months prior to the initial visit in our clinics. Patients were deemed in good SCD care if they had been transitioned from a pediatric SCD program or another adult SCD program where they received team based comprehensive services. Results: 74 patients were able to be evaluated. (Table 1). 53 patients were Hgb SS. Of those, 27 (50.9%) were not receiving appropriate SCD care for the prior 12 months when they first presented, 20 (74.1%) of those patients were iron overloaded. There were 9 patients with Hgb S/beta thalassemia 0 or +. Five (55.6%) of those patients were not receiving appropriate SCD care for the prior 12 months; one (35.6%) was iron overloaded. 12 patients had Hgb SC. Although 5 (41.7%) were not receiving appropriate SCD care at the time of presentation, none had evidence of iron overload. Using these data, the odds ratio of having iron overload if a patient was out of appropriate SCD care for the 12 months prior to evaluation was 6.8 (95% confidence interval 2,23.13), indicating that being out of good care is a risk factor for iron overload. Discussion: Although guidelines exist to prevent unnecessary transfusions in adult patients with SCD; lack of access to knowledgeable providers both in the inpatient and outpatient setting places them at a nearly 7 times higher risk for iron overload, a serious and preventable comorbidity. Our study indicates that many adults with SCD are not appropriately monitored or treated for transfusion related iron overload using guideline-based care, placing avoidable burdens on individual health and adding to healthcare costs. Further education of providers to address this issue is warranted. Disclosures Baker: Genentech: Research Funding.

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Iron overload is a determinant of morbidity and mortality in adult patients with sickle cell disease

Seminars in Hematology ◽

10.1053/shem.2001.20142 ◽

2001 ◽

Vol 38 (1, Suppl 1) ◽

pp. 30-36 ◽

Cited By ~ 14

Author(s):

Samir K. Ballas

Keyword(s):

Sickle Cell Disease ◽

Iron Overload ◽

Sickle Cell ◽

Adult Patients ◽

Morbidity And Mortality ◽

Cell Disease

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Growth differentiation factor-15 in young sickle cell disease patients: Relation to hemolysis, iron overload and vascular complications

Blood Cells Molecules and Diseases ◽

10.1016/j.bcmd.2014.07.003 ◽

2014 ◽

Vol 53 (4) ◽

pp. 189-193 ◽

Cited By ~ 6

Author(s):

Azza Abdel Gawad Tantawy ◽

Amira Abdel Moneam Adly ◽

Eman Abdel Rahman Ismail ◽

Yasser Wagih Darwish ◽

Marwa Ali Zedan

Keyword(s):

Sickle Cell Disease ◽

Iron Overload ◽

Sickle Cell ◽

Vascular Complications ◽

Cell Disease ◽

Growth Differentiation Factor 15 ◽

Differentiation Factor

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Serum ferritin and total units transfused for assessing iron overload in adults with sickle cell disease

British Journal of Haematology ◽

10.1111/j.1365-2141.2012.09060.x ◽

2012 ◽

Vol 157 (5) ◽

pp. 645-647 ◽

Cited By ~ 15

Author(s):

Emma Drasar ◽

Nisha Vasavda ◽

Norris Igbineweka ◽

Moji Awogbade ◽

Marlene Allman ◽

...

Keyword(s):

Sickle Cell Disease ◽

Iron Overload ◽

Sickle Cell ◽

Serum Ferritin ◽

Cell Disease

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Approaches to Transfusion Therapy and Iron Overload in Patients with Sickle Cell Disease: Results of an International Survey

Pediatric Hematology and Oncology ◽

10.3109/08880018.2010.505497 ◽

2010 ◽

Vol 28 (1) ◽

pp. 37-42 ◽

Cited By ~ 14

Author(s):

Elliott P. Vichinsky ◽

Kwaku Ohene-Frempong

Keyword(s):

Sickle Cell Disease ◽

Iron Overload ◽

Sickle Cell ◽

International Survey ◽

Cell Disease ◽

Transfusion Therapy

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American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support

Blood Advances ◽

10.1182/bloodadvances.2019001143 ◽

2020 ◽

Vol 4 (2) ◽

pp. 327-355 ◽

Cited By ~ 22

Author(s):

Stella T. Chou ◽

Mouaz Alsawas ◽

Ross M. Fasano ◽

Joshua J. Field ◽

Jeanne E. Hendrickson ◽

...

Keyword(s):

Sickle Cell Disease ◽

Iron Overload ◽

Sickle Cell ◽

Conflicts Of Interest ◽

Guideline Development ◽

Practice Research ◽

Evidence Based ◽

Red Cell ◽

Cell Disease ◽

American Society

Background: Red cell transfusions remain a mainstay of therapy for patients with sickle cell disease (SCD), but pose significant clinical challenges. Guidance for specific indications and administration of transfusion, as well as screening, prevention, and management of alloimmunization, delayed hemolytic transfusion reactions (DHTRs), and iron overload may improve outcomes. Objective: Our objective was to develop evidence-based guidelines to support patients, clinicians, and other healthcare professionals in their decisions about transfusion support for SCD and the management of transfusion-related complications. Methods: The American Society of Hematology formed a multidisciplinary panel that was balanced to minimize bias from conflicts of interest and that included a patient representative. The panel prioritized clinical questions and outcomes. The Mayo Clinic Evidence-Based Practice Research Program supported the guideline development process. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used to form recommendations, which were subject to public comment. Results: The panel developed 10 recommendations focused on red cell antigen typing and matching, indications, and mode of administration (simple vs red cell exchange), as well as screening, prevention, and management of alloimmunization, DHTRs, and iron overload. Conclusions: The majority of panel recommendations were conditional due to the paucity of direct, high-certainty evidence for outcomes of interest. Research priorities were identified, including prospective studies to understand the role of serologic vs genotypic red cell matching, the mechanism of HTRs resulting from specific alloantigens to inform therapy, the role and timing of regular transfusions during pregnancy for women, and the optimal treatment of transfusional iron overload in SCD.

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Iron Depletion: An Ameliorating Factor for Sickle Cell Disease?

ISRN Hematology ◽

10.5402/2011/473152 ◽

2011 ◽

Vol 2011 ◽

pp. 1-4 ◽

Cited By ~ 5

Author(s):

P. C. Giordano ◽

W. Huisman ◽

C. L. Harteveld

Keyword(s):

Sickle Cell Disease ◽

Iron Overload ◽

Beneficial Effect ◽

Sickle Cell ◽

Chelation Therapy ◽

Cell Disease ◽

Laboratory Practice ◽

Iron Depletion ◽

Severity Of The Disease

We report some observations from our laboratory practice that might be important for the treatment of sickle cell disease (SCD). We describe data from two cases indicating that iron depletion might have a beneficial effect diminishing the formation of HbS in favor of HbF, possibly reducing the severity of the disease. We believe that it would be worthwhile to monitor the course of the disease comparing cases with identical genotypes with and without iron depletion, and we advise to consider chelation therapy to reduce iron overload in patients with SCD.

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