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

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 lifethreatening haematological 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.

The Psychological Experience of Women who Survived HELLP Syndrome in Cape Town; South Africa

Background: Haemolysis; elevated liver enzymes and low platelet count (HELLP syndrome) is a high-risk pregnancy condition that could be fatal to mother and/or baby. It is characterised; as the acronym indicates; by haemolysis; elevated liver enzymes and low blood platelets.Objective: This study explored women in Cape Town's psychological experience of HELLP syndrome. Method: Six participants who previously experienced HELLP syndrome were interviewed. Using a grounded theory approach; themes emerged and a model illlustrating the psychological experience of HELLP syndrome was constructed. Results: The major themes that emerged were the perceived lack of information; a need to assign blame and a shift in focus. Themes of not knowing and trance and/or surreal experience underpin the cognitive aspects of the HELLP syndrome experience. Themes that expressed feelings of an inability to control; whirlwind and/or rapid pace and support acted together to bind the experience. Finally; emotions such as anger; ambivalence; disbelief; anxiety; guilt; loneliness and fear were present throughout the experience. Conclusion: This study developed an initial exploratory model representing the psychological experience of HELLP syndrome in a sample of South African women. Underlying this entire experience was a perceived lack of information which had a profound effect on numerous aspects of the experience ranging from where to locate blame to the varied emotions experienced

Autoimmune Hemolytic Anemia in HIV-infected Patients: a Hospital Based Study

Background: The prevalence of anemia in HIV/ AIDS patients is high; with a multitude of possible etiologies; autoimmune hemolytic anemia (AIHA) in HIV/AIDS patients has been associated with a poor prognosis when treated with red cell transfusion. Our aim was to demonstrate the frequency of AIHA in a cohort of adult Nigerian HIV/AIDS patients and to see if the presence or not of AIHA is related to the severity of the disease with regards to the CD4 counts and the presence or absence of opportunistic infections. Method: Ninety- eight adult patients with HIV infection were screened for the presence of AIHA using the packed cell volume (PCV); direct antiglobulin test (DAT) and reticu- locyte count (RC). Results: The frequency of AIHA was 3.06; 36.74of our study population were anemic; 11.22had a positive DAT. Mean RC was 2.22 +/- 0.90 for all the patients. There was no statistically significant difference in the PCV of patients that had positive and negative DAT. There was no correlation between the presence of AIHA; use of ART; presence of opportunistic infections or CD4 counts. Conclusion: We conclude that in spite of the low frequency of AIHA in HIV/AIDS patients; the fact that most patients will respond to standard treatment makes it imperative to screen HIV/AIDS patients with anemia for the presence of AIHA. Again since HIV/AIDS patients with AIHA may have a fatal reaction to red cell transfusion; we suggest that anemic patients with HIV/AIDS in non-emergency situations be screened for the presence of AIHA before receiving red cell transfusions when indicated

Autoimmune Hemolytic Anemia in HIV-infected Patients: a Hospital Based Study

Background: The prevalence of anemia in HIV/ AIDS patients is high; with a multitude of possible etiologies; autoimmune hemolytic anemia (AIHA) in HIV/AIDS patients has been associated with a poor prognosis when treated with red cell transfusion. Our aim was to demonstrate the frequency of AIHA in a cohort of adult Nigerian HIV/AIDS patients and to see if the presence or not of AIHA is related to the severity of the disease with regards to the CD4 counts and the presence or absence of opportunistic infections. Method: Ninety- eight adult patients with HIV infection were screened for the presence of AIHA using the packed cell volume (PCV); direct antiglobulin test (DAT) and reticulocyte count (RC). Results: The frequency of AIHA was 3.06; 36.74of our study population were anemic; 11.22had a positive DAT. Mean RC was 2.22 +/- 0.90 for all the patients. There was no statistically significant difference in the PCV of patients that had positive and negative DAT. There was no correlation between the presence of AIHA; use of ART; presence of opportunistic infections or CD4 counts. Conclusion: We conclude that in spite of the low frequency of AIHA in HIV/AIDS patients; the fact that most patients will respond to standard treatment makes it imperative to screen HIV/AIDS patients with anemia for the presence of AIHA. Again since HIV/AIDS patients with AIHA may have a fatal reaction to red cell transfusion; we suggest that anemic patients with HIV/AIDS in non-emergency situations be screened for the presence of AIHA before receiving red cell transfusions when indicated