Hemophagocytosis in a patient with sickle cell disease

Thung, Irene; Broome, H. Elizabeth

doi:10.1182/blood-2015-11-680082

Cited by 5 publications

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“…In spite of the fact that SCD is associated with high incidence of infection (20), the literature regarding HLH in patients with SCD is surprisingly sparse (102,(106)(107)(108)(109)(110)(111), which would suggest a paradoxically low incidence of HLH in SCD. It is not clear whether this paradox is due to under-reporting or under-diagnosis of HLH in patients with SCD.…”

Section: Infections Associated Intra-medullary Haemolysis In Scd: The...mentioning

confidence: 99%

“…It is not clear whether this paradox is due to under-reporting or under-diagnosis of HLH in patients with SCD. While it has been reported that VOC (106) and blood transfusion (107) independently trigger HLH in patients with SCD, most of the remaining few cases of HLH were reported in SCD have been associated with infections due a myriad of pathogens such as unspecified periodontal bacteria (108), Epstein-Barr virus and Cytomegalovirus (109), Parvovirus-B19 (102), Histoplasma spp. (110), and atypical mycobacteria (111).…”

Section: Infections Associated Intra-medullary Haemolysis In Scd: The...mentioning

confidence: 99%

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A review of the role of infections in the aetiology of haemolysis in patients with sickle cell diseases: pathogenesis, management, and prevention

Ahmed¹,

Ibrahim²

2022

Af J Clin Exp Micro

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Background: Sickle cell disease (SCD) is associated with chronic haemolysis, immuno-suppression and susceptibility to infections, which may trigger infection-associated haemolysis (IAH). SCD patients are vulnerable to anaemic effect of IAH due to vicious interaction between pre-existing ‘inherited’ chronic haemolysis and ‘acquired’IAH. IAH in SCD manifests as febrile haemolytic crisis with clinical and laboratory features of severe anaemia or pancytopenia. Clinico-pathological perspectives of IAH in SCD are fragmented. This review presents a comprehensive but concise overview of pathogenesis, management and prevention of IAH in SCD.Methodology and results: Online literature search using search terms such as ‘sickle cell disease, viral, bacterial, parasitic, fungal, infections, hyperhaemolytic crisis, haemophagocytic syndrome, severe anaemia, pancytopenia’ in various combinations was done on PubMed/Medline, Google, Google-Scholar and Bing. Overall, 112 relevant publications were retrieved, which included 109 peer reviewed journal articles, 2 World Health Organization (WHO) technical reports, and 1 edited text book. A range of bacterial (Bartonella spp, Mycoplasma spp., Mycobacterium avium complex), viral (Dengue, SARS-CoV-2, Parvovirus-B19, Cytomegalovirus, Epstein-Barr virus), parasitic(Plasmodium spp., Babesia spp.), and fungal (Histoplasma spp.) infections were associated with IAH in SCD. There are two broad types of IAH in patients with SCD; infection associated extra-medullary haemolysis (IAEMH) and infection associated intra-medullary haemolysis (IAIMH). While IAEMH is associated with severe anaemia due to intravascular haemolysis caused by red cell invasion, oxidative injury, auto-antibodies, and/or pathogen-haem interaction, IAIMH is associated with haemophagocytic tri-lineage destruction of haematopoietic precursors in the bone marrow.Conclusion: Various microbial pathogens have been associated with IAH in SCD. SCD patients with fever, severe anaemia or pancytopenia should be investigated for early diagnosis and prompt treatment of IAH, which is a lifethreateninghaematological emergency for which transfusion therapy alone may not suffice. Prompt and sustainable termination of IAH may require therapeutic combination of transfusion, anti-microbial chemotherapy, and immune modulation therapy. SCD patients should also receive counselling on hygiene, barrier protection against vectors, routine chemoprophylaxis for locally endemic diseases, and immunization for vaccine-preventable infections as a long-term preventive strategy against IAH. Contexte: La drépanocytose (SCD) est associée à une hémolyse chronique, à une immunosuppression et à une susceptibilité aux infections, ce qui peut déclencher une hémolyse associée à une infection (HIA). Les patientsatteints de SCD sont vulnérables à l'effet anémique de l'HIA en raison de l'interaction vicieuse entre l'hémolyse chronique "héréditaire" préexistante et l'HIA "acquise". L'HIA dans la SCD se manifeste par une crise hémolytique fébrile avec des caractéristiques cliniques et de laboratoire d'anémie sévère ou de pancytopénie. Les perspectives clinico-pathologiques de l'HIA dans la SCD sont fragmentées. Cette revue présente un aperçu complet mais concis de la pathogenèse, de la gestion et de la prévention de l'HIA dans la drépanocytose.Méthodologie et résultats: Une recherche documentaire en ligne à l'aide de termes de recherche tels que "drépanocytose, virale, bactérienne, parasitaire, fongique, infections, crise hyperhémolytique, syndrome hémophagocytaire,anémie sévère, pancytopénie" dans diverses combinaisons a été effectuée sur PubMed/Medline, Google, Google-Scholar et Bing. Au total, 112 publications pertinentes ont été récupérées, dont 109 articles de revues à comité de lecture, 2 rapports techniques de l'Organisation mondiale de la santé (OMS) et 1 manuel édité. Unegamme bactérienne (Bartonella spp, Mycoplasma spp., Mycobacterium avium complex), virale (Dengue, SARS-CoV-2, Parvovirus-B19, Cytomegalovirus, Epstein-Barr virus), parasitaire (Plasmodium spp., Babesia spp.), et lesinfections fongiques (Histoplasma spp) étaient associées à l'IAH dans la SCD. Il existe deux grands types d'HIA chez les patients atteints de SCD; hémolyse extra-médullaire associée à une infection (IAEMH) et hémolyse intramédullaireassociée à une infection (IAIMH). Alors que l'IAEMH est associée à une anémie sévère due à une hémolyse intravasculaire causée par l'invasion des globules rouges, une lésion oxydative, des auto-anticorps et/ou une interaction pathogène-hème, l'IAEMH est associée à la destruction tri-lignée hémophagocytaire des précurseurshématopoïétiques dans la moelle osseuse.Conclusion: Divers agents pathogènes microbiens ont été associés à l'IAH dans la SCD. Les patients atteints de SCD avec de la fièvre, une anémie sévère ou une pancytopénie doivent être examinés pour un diagnostic précoce et un traitement rapide de l'HIA, qui est une urgence hématologique potentiellement mortelle pour laquelle la thérapie transfusionnelle seule peut ne pas suffire. L'arrêt rapide et durable de l'HIA peut nécessiter une combinaison thérapeutique de transfusion, de chimiothérapie antimicrobienne et de thérapie de modulation immunitaire. Lespatients atteints de drépanocytose devraient également recevoir des conseils sur l'hygiène, la barrière de protection contre les vecteurs, la chimioprophylaxie de routine pour les maladies endémiques locales et la vaccination contreles infections évitables par la vaccination en tant que stratégie préventive à long terme contre l'HIA.

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Section: Infections Associated Intra-medullary Haemolysis In Scd: The...mentioning

confidence: 99%

Section: Infections Associated Intra-medullary Haemolysis In Scd: The...mentioning

confidence: 99%

A review of the role of infections in the aetiology of haemolysis in patients with sickle cell diseases: pathogenesis, management, and prevention

Ahmed¹,

Ibrahim²

2022

Af J Clin Exp Micro

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“…HLH in sickle cell disease and related hemoglobinopathies has been described but is not well characterized and has been associated with mycobacterium avium-complex infection, malaria, transfusion and bone pain crises. [8][9][10][11] HLH in sickle cell patients is under-recognized so the true incidence is not known. HLH is a fatal syndrome that requires a timely diagnosis in order to initiate life-saving therapy.…”

Section: Recognition Of Hemophagocytic Lymphohistiocytosis In Sickle Cell Vaso-occlusive Crises Is a Potentially Lifesaving Diagnosismentioning

confidence: 99%

Recognition of hemophagocytic lymphohistiocytosis in sickle cell vaso-occlusive crises is a potentially lifesaving diagnosis

et al. 2019

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“…Sickle cell disease (SCD) is characterized by hemolytic anemia and painful vaso-occlusive crises (VOC), caused by increased adherence of sickle RBCs to the underlying activated vascular endothelium (1,2) Accumulating evidence highlights the pivotal role of the mononuclear phagocyte system, encompassing blood monocytes, spleen red pulp macrophages, and liver Kupffer cells, in erythrophagocytosis of sickle RBCs and in the clearance of hemolytic byproducts in SCD (3)(4)(5)(6)(7)(8)(9)(10)(11). Our recent findings demonstrate that, a subset of blood monocytes, known as nonclassical monocytes or patrolling monocytes (PMo), are instrumental in mitigating VOCs in SCD by scavenging endothelial cell-attached (EC-attached) sickle RBCs and debris from hemolysis-damaged endothelium (12)(13)(14).…”

Section: Introductionmentioning

confidence: 99%

Hemolysis dictates monocyte differentiation via two distinct pathways in sickle cell disease vaso-occlusion

Liu,

Su,

Shayo

et al. 2023

Journal of Clinical Investigation

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Hemophagocytosis in a patient with sickle cell disease

Cited by 5 publications

References 0 publications

A review of the role of infections in the aetiology of haemolysis in patients with sickle cell diseases: pathogenesis, management, and prevention

A review of the role of infections in the aetiology of haemolysis in patients with sickle cell diseases: pathogenesis, management, and prevention

Recognition of hemophagocytic lymphohistiocytosis in sickle cell vaso-occlusive crises is a potentially lifesaving diagnosis

Hemolysis dictates monocyte differentiation via two distinct pathways in sickle cell disease vaso-occlusion

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