A combination of two novel alpha globin variants Hb Bridlington (<i>HBA1</i>) and Hb Taybe (<i>HBA2</i>) resulting in severe hemolysis, pulmonary hypertension, and death

Hill, Quentin A.; Farrar, Lisa; Lordan, Jim; Gallienne, Alice; Henderson, Shirley

doi:10.1179/1607845414y.0000000164

Cited by 10 publications

(8 citation statements)

References 9 publications

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“…World Health Organization (WHO) group IV pulmonary hypertension and WHO group I pulmonary hypertension (pulmonary arterial hypertension, PAH) have been demonstrated in patients who have undergone splenectomy for pyruvate kinase deficiency, stomatocytosis, hereditary spherocytosis, unstable hemoglobinopathies, paroxysmal nocturnal hemoglobinuria, myeloid metaplasia, thalassemia, Gaucher disease, trauma, and nonhemolytic conditions, as well as in a case of congenital asplenia (13,15,21,(37)(38)(39)(40)(41)(42)(43)(44)(45)(46)(47)(48)(49)(50)(51)(52)(53).…”

Section: Association Of Splenectomy With Pulmonary Hypertensionmentioning

confidence: 99%

Review of the Association between Splenectomy and Chronic Thromboembolic Pulmonary Hypertension

Kimmig

Palevsky

2016

Annals ATS

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Recent evidence suggests that there may be a link between splenectomy and the later development of pulmonary hypertension, in particular World Health Organization group IV pulmonary hypertension (chronic thromboembolic pulmonary hypertension). Epidemiological studies have demonstrated an odds ratio as high as 18 for the development of chronic thromboembolic pulmonary hypertension after splenectomy in comparison with matched control subjects who have not undergone splenectomy. The mechanisms governing the association between removal of the spleen and the subsequent development of chronic thromboembolic pulmonary hypertension remain incompletely understood; however, recent advances in understanding of coagulation homeostasis have shed some light on this association. Splenectomy increases the risk of venous thromboembolic disease, a necessary precursor of chronic thromboembolic pulmonary hypertension, by generating a prothrombotic state. This prothrombotic state likely results from a reduction in the removal of circulating procoagulant factors from the bloodstream after splenectomy. Although much is to be learned, circulating microparticles have emerged as the most likely mediator for the development of thrombosis after splenectomy. Apparently because of a reduction in reticuloendothelial cell clearance, microparticle levels are elevated in patients after splenectomy. Elevated circulating microparticle levels have been linked to thromboembolism and pulmonary hypertension in a dose-dependent fashion. It is important for health care providers to be aware of the link between splenectomy and chronic thromboembolic pulmonary hypertension. We are optimistic that clarification of the exact mechanisms that govern this association will yield clinical guidelines and potential treatments.

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Section: Association Of Splenectomy With Pulmonary Hypertensionmentioning

confidence: 99%

Review of the Association between Splenectomy and Chronic Thromboembolic Pulmonary Hypertension

Kimmig

Palevsky

2016

Annals ATS

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“…Thrombotic complications following splenectomy have been described in nine patients (with Hb Bridlington/HbTaybe, Hb Taybe, Hb Mainz, Hb Olmsted, Hb Madrid and Hb Perth). 101 – 106 The thrombotic events, including pulmonary emboli, pulmonary arterial hypertension, arterial stroke and priapism, occurred even 4–32 years after splenectomy. The majority of patients (7/9) had no or only partial improvement in hemoglobin levels.…”

Section: Disease-specific Recommendationsmentioning

confidence: 99%

Recommendations regarding splenectomy in hereditary hemolytic anemias

et al. 2017

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Hereditary hemolytic anemias are a group of disorders with a variety of causes, including red cell membrane defects, red blood cell enzyme disorders, congenital dyserythropoietic anemias, thalassemia syndromes and hemoglobinopathies. As damaged red blood cells passing through the red pulp of the spleen are removed by splenic macrophages, splenectomy is one possible therapeutic approach to the management of severely affected patients. However, except for hereditary spherocytosis for which the effectiveness of splenectomy has been well documented, the efficacy of splenectomy in other anemias within this group has yet to be determined and there are concerns regarding short- and long-term infectious and thrombotic complications. In light of the priorities identified by the European Hematology Association Roadmap we generated specific recommendations for each disorder, except thalassemia syndromes for which there are other, recent guidelines. Our recommendations are intended to enable clinicians to achieve better informed decisions on disease management by splenectomy, on the type of splenectomy and the possible consequences. As no randomized clinical trials, case control or cohort studies regarding splenectomy in these disorders were found in the literature, recommendations for each disease were based on expert opinion and were subsequently critically revised and modified by the Splenectomy in Rare Anemias Study Group, which includes hematologists caring for both adults and children.

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“…Two patients had to have a splenectomy in order to ameliorate the transfusion requirement. A recent report raises the question whether splenectomy is beneficial in those patients or contributes to the development of pulmonary hypertension and death (19). This is the largest report of patients with this condition and demonstrates that the entity has distinct features which are different from other hemoglobinopathies, CDA, or aand b-thalassemia.…”

Section: Discussionmentioning

confidence: 86%

Section: Discussionmentioning

confidence: 99%

Hb TAYBE: clinical and morphological findings IN 43 patients

Koren

Levin

Zalman

et al. 2015

European J of Haematology

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A combination of two novel alpha globin variants Hb Bridlington (HBA1) and Hb Taybe (HBA2) resulting in severe hemolysis, pulmonary hypertension, and death

Abstract: Splenectomy was not clearly beneficial and may have contributed to the development of pulmonary hypertension. The case favors a cautious approach when considering splenectomy for patients with Hb Taybe.

Cited by 10 publications

References 9 publications

Review of the Association between Splenectomy and Chronic Thromboembolic Pulmonary Hypertension

Review of the Association between Splenectomy and Chronic Thromboembolic Pulmonary Hypertension

Recommendations regarding splenectomy in hereditary hemolytic anemias

Hb TAYBE: clinical and morphological findings IN 43 patients

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