Acute splenic complications and implications of splenectomy in hemoglobin SC disease

Subbannan, Karthi; Üstün, Celalettin; Natarajan, Kavita; Clair, Betsy; Daitch, Lisa; Fields, Sabine; Kutlar, Ferdane; Kutlar, Abdullah

doi:10.1111/j.1600-0609.2009.01270.x

Cited by 6 publications

(6 citation statements)

References 13 publications

(25 reference statements)

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“…Splenectomy improves anaemia and thrombocytopenia. Whether this causes an injurious increase in viscosity for HbSC patients is unclear (Subbannan et al , ). Splenectomy is ideally performed at least 2 weeks after vaccination against encapsulated organisms, and post‐splenectomy antibiotic prophylaxis is recommended.…”

Section: Systemic Complicationsmentioning

confidence: 99%

Knowledge insufficient: the management of haemoglobin SC disease

2016

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Although haemoglobin SC (HbSC) accounts for 30% of sickle cell disease (SCD) in the United States and United Kingdom, evidence-based guidelines for genotype specific management are lacking. The unique pathology of HbSC disease is complex, characterized by erythrocyte dehydration, intracellular sickling and increased blood viscosity. The evaluation and treatment of patients with HbSC is largely inferred from studies of SCD consisting mostly of haemoglobin SS (HbSS) patients. These studies are underpowered to allow definitive conclusions about HbSC. We review the pathophysiology of HbSC disease, including known and potential differences between HbSS and HbSC, and highlight knowledge gaps in HbSC disease management. Clinical and translational research is needed to develop targeted treatments and to validate management recommendations for efficacy, safety and impact on quality of life for people with HbSC.

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Section: Systemic Complicationsmentioning

confidence: 99%

Knowledge insufficient: the management of haemoglobin SC disease

2016

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“…Natural history studies have demonstrated that Hb SC patients develop progressively larger spleens which can contribute to “hypersplenism” as noted by mild thrombocytopenia and spleen dysfunction. 17 – 19 In addition, HU may induce splenic regrowth in patients with Hb SC which may exacerbate this thrombocytopenia, limit dosing of HU, and increase risk of splenic complications. 19 – 22 Patients with Hb SB+ thalassemia may also suffer from dysfunctional splenic function but this research is limited.…”

Section: Discussionmentioning

confidence: 99%

Hydroxyurea decreases hospitalizations in pediatric patients with Hb SC and Hb SB+ thalassemia

Lebensburger¹,

Patel²,

Palabindela³

et al. 2015

JBM

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PurposePatients with hemoglobin SC (Hb SC) and hemoglobin SB+ (Hb SB+) thalassemia suffer from frequent hospitalizations yet strong evidence of a clinical benefit of hydroxyurea (HU) in this population is lacking. Patients with recurrent hospitalizations for pain crisis are offered HU at our institution based on small cohort data and anecdotal benefit. This study identifies outcomes from a large cohort of patients with Hb SC and SB+ thalassemia who were treated with HU for 2 years.Materials and methodsA retrospective review was conducted of 32 patients with Hb SC and SB+ thalassemia who were treated with HU. We reviewed the number, and reasons for hospitalization in the 2 years prior to, and 2 years post-HU treatment as well as laboratory changes from baseline, over 1 year.ResultsPatients with Hb SC and SB+ thalassemia started on HU for frequent pain, had a significant reduction in hospitalizations over 2 years as compared to the 2 years prior to HU initiation (mean total hospitalizations/year: pre-HU: 1.6 vs post-HU 0.4 hospitalizations, P<0.001; mean pain hospitalizations/year: pre-HU 1.5 vs post-HU 0.3 hospitalizations, P<0.001). Patients demonstrated hematologic changes including an increase in percent fetal hemoglobin (%HbF) pre–post HU (4.5% to 7.7%, P=0.002), mean corpuscular volume (74 to 86 fL, P<0,0001), and decrease in absolute neutrophil count (5.0 to 3.2×109/L, P=0.007). Patients with higher doses of HU demonstrated the greatest reduction in hospitalizations but this was unrelated to absolute neutrophil count.ConclusionThis cohort of patients with Hb SC and SB+ thalassemia provides additional support for using HU in patients with recurrent hospitalizations for pain. A large randomized multicenter trial of HU to reduce pain admissions should be conducted to confirm these data and provide much needed evidence based recommendations for this population.

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“…Splenic sequestration in adults may develop insidiously often with abdominal pain. Patients with chronic splenomegaly can develop hypersplenism and may be predisposed to acute splenic sequestration (Subbannan et al, 2009). The largest case series of splenic complications in 124 adults with sickle cell/haemoglobin C compound heterozygosity reported a splenectomy rate of 9.6 per cent for infarction, sequestration, hypersplenism and subcapsular bleeding (Subbannan et al, 2009).…”

Section: Splenic Complicationsmentioning

confidence: 99%

Sickle cell disease: causes, treatments and the patient experience

2024

Pharmaceutical Journal

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Chapter 2: Organisation of care "We require respect, care, dignity and candour. We require mandated standards that can be challenged by patients when failures occur. Commissioning that reflects need that has parity with other specialised illnesses given SCD is the world's most common genetic condition." General principles"We need more nurses, more community services, more adult care support services and a dedicated team to support us with the phases of change we experience as sickle patients." Community nursing"More home visits should be encouraged by support workers and nurses" "My community specialist nurse gave me a card which I carry in my wallet and shows I am on the national haemoglobinopathy registry and has all my medical details at hand in case of an emergency" Prevention of infection: immunisations and prophylactic antibiotics"The penicillin and folic acid helps me. The six monthly clinic at the hospital is usually for blood tests and checks. I have not been in hospital with a crisis for nearly two years.""I get my pneumococcal vaccines every five years and my flu jabs yearly. I don't tend to have chest infections lately" Blood pressure monitoring"My blood pressure is always monitored when I attend my outpatient appointments. However, this is not very regular." Recommendations Hypertension in SCD should be monitored and treated in primary care. Patients with hypertension and ACR <3.5 mg/mmol should be treated with a BP target of <140/90 mmHg. Patients with hypertension and ACR ≥3.5 mg/mmol should be treated with a BP target of <130/80 mmHg.  In the absence of proteinuria initial treatment should be with calcium channel blockers. Folic acid"I have been taking folic acid since I was a child, I am now in my 40s" Nutrition and lifestyle"We could do with some help with food management and what not to eat e.g. foods with iron if you are on an exchange programme." "I have been fortunate enough to have a research background, so I have researched myself treatments, management and why I need to eat certain foods etc." Health education'… the competencies that 'lay' contributors can offer should not be permitted to draw attention away from the need to change professional attitudes and practices, and the value of constructive, 'grown up ', professional/patient relationships.'

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Acute splenic complications and implications of splenectomy in hemoglobin SC disease

Cited by 6 publications

References 13 publications

Knowledge insufficient: the management of haemoglobin SC disease

Knowledge insufficient: the management of haemoglobin SC disease

Hydroxyurea decreases hospitalizations in pediatric patients with Hb SC and Hb SB+ thalassemia

Sickle cell disease: causes, treatments and the patient experience

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