Late Onset Combined Immune Deficiency (LOCID) Revealed by a Haemolytic Anaemia in a Child: A Case Report

Variable Common Immune Deficiency (VCID) is a very heterogeneous condition both clinically and immunologically. It is a group of molecular abnormalities responsible for a defect in antibody production leading to hypogammaglobulinemia often associated with autoimmune and/or lymphoproliferative manifestations. Late Onset Combined Immune Deficiency (LOCID) is a type of Variable Common Immune Deficiency (VCID) defined by a defect in antibody production (IgG and IgA ± IgM type), profound CD4 T-cell lymphopenia and frequent opportunistic infections. LOCID has been considered as a distinct entity from VCID due to its particular clinical and immunological profile.

Factors Associated with Haemolytic Anaemia in the Internal Medicine Department of the Douala General Hospital in Cameroon

Introduction: Haemolytic anaemia (HA) is defined as a decrease in haemoglobin (Hb) levels below baseline due to excessive and premature destruction of red blood cells (RBCs) in the periphery resulting in a shortened life span of less than 120 days. Haemolysis can be corpuscular or extra-corpuscular. The aim of our study was to investigate the factors associated with it for optimal management of patients hospitalised in internal medicine at the Douala General Hospital (DGH). Methodology: We conducted an analytical cross-sectional study, including all patients admitted to the internal medicine department of the DGH from 11 February to 20 May 2022, and excluding patients with non-compliant samples. The search for haemolytic anaemia was carried out by means of blood count, reticulocyte count, blood smear, unconjugated bilirubin, lactate dehydrogenase and direct Coombs test. Sociodemographic, clinical and biological parameters were collected and analysed. Correlation was defined for a p value 0.05. Results: This study included 147 patients, 50.34% of whom were men, for a sex ratio of 1.01. The mean age was 52 ± 17.9 years. The most represented age group was 56 - 70 years (n = 49;33%) with extremes from 15 to 90 years. We counted 29.3% cases of haemolytic anaemia (HA) and 13.9% cases of autoimmune haemolytic anaemia (AIHA). Haemolytic anaemia (HA) was present in 54.14% of men for a sex ratio of 1.38;the most represented age group was 40 - 55 years, 37.2%. HA was associated with jaundice (OR: 3.74, CI: [1.70 - 8.22], p = 0.001), HIV - AIDS (OR: 2.72, CI: [0.98 - 7.53], p = 0.05), thrombocytopaenia (OR: 3.53, CI: [1.58 - 7.89], p = 0.02). LDH was elevated (OR: 2.86, CI: [1.30 - 6.26], p = 0.00) as well as elevated reticulocyte count (OR: 3.84, CI: [1.75 - 8.44], p = 0.01). Unconjugated bilirubin was elevated in all these patients. In multivariate analysis, factors associated with HA were a history of HIV/AIDS, jaundice, thrombocytopaenia and elevated reticulocyte count. Conclusion: Hemolytic anaemia is common in internal medicine and is significantly associated with thrombocytopenia, HIV/AIDS infection and jaundice.

Cholelithiasis associated with haemolytic-uraemic syndrome

Cholelithiasis occurs infrequently in the paediatric age group. Hereditary spherocytosis, sickle cell anaemia and thalassemia are the haemolytic disorders most commonly associated with development of gall stones in paediatric age group. The question is whether an isolated episode of haemolysis can cause gallstones.

Antimicrobial,antiplasmodial,haemolytic and antioxidant activities of crude extracts from three selected Togolese medicinal plants

Objective:To investigate the antioxidant,antimicrobial,antiplasmodial,acute toxicity and haemolytic activities of methanolic extracts of three plants.Phytochemical analysis to determine the phenolic contents was also carried out.Methods:The 2,2-diphenyl-1- picryl-hydrazyl（DPPH） free radical scavenging,NCCLS broth microdilution and Plasmodium Lactate Dehydrogenase（pLDH） assays were used to determine antioxidant,antimicrobial and antiplasmodial activities,respectively.Haemolysis assay was conducted on A+ human red blood cells and acute toxicity on male Swiss albino mice.Phenolics were quantitatively determined using spectrophotometric methods.Results:The DPPH assay yielded interesting antioxidant activities of methanolic extract of Parinari curatellifolia（P.curatellifolia） and Entada africana （E.africana）(IC50 were 0.20±0.01μg/mL and 0.47±0.01μg/mL,respectively).This activity was highly correlated with phenolic contents of extracts.The antimicrobial tests displayed minimal inhibitory concentrations（MICs） values ranging from 0.90 to 1.80 mg/mL for Serratia marcescens （5.marcescens） the most susceptible bacterial strain.MIC value was 1.20 mg/mL for susceptible fungal strains including Mucor rouxi（M.rouxi）,Fusarium oxyporum（F.oxyporum） and Rhizopus nigricans（R.nigricans）.pLDH assay showed moderate antiplasmodial activity of Balanites aegyptiaca（B.aegyptiaca）(IC50= 24.56±3.45μg/mL),however this extract was highly haemolytic and toxic in mice(LD50= 625±128mg/kg).Conclusions:Our results support in part the use of the selected plants in the treatment of microbial infections.In addition the plant showed interesting antioxidant activity that could be useful in the management of oxidative stress.

Gemcitabine-induced haemolytic uremic syndrome,although infrequent,can it be prevented:A case report and review of literature

Gemcitabine is an antineoplastic used to treat several malignancies including pancreatic cancer. Its toxicity profile is well known with myelotoxicity, increased vascular permeability and peripheral oedema as most frequent adverse events. However, several cases of acute renal failure have been reported and haemolytic uremic syndrome(HUS) seems to be the underlying process. The cause of HUS remains unknown but its consequences can be lethal. Therefore, a high grade of suspicion is crucial to diagnose it and promptly treat it. This hopefully will reduce its morbidity. HUS is characterized by progressive renal failure associated with microangiopathic haemolytic anaemia and thrombocytopenia. The primary event is damage to endothelial cells and thrombotic microangiopathy(TMA) is the histopathological lesion. TMA affects mainly renal microvasculature. However, some cases evolve with central nervous or cardiovascular systems involvement. We present here a case of gemcitabine-induced HUS, with renal and cardiovascular system affected at the time of diagnosis which to our knowledge this is the first time of such case to be reported.

An Assessment of Frequent Haemolytic Crisis in G6PD Deficiency Subjects Linked with Chronic Disease States in Al Ahsa Community, Saudi Arabia

The main treatment strategy for glucose-6-phosphate dehydrogenase (G6PD) deficiency is averting the use of oxidative precipitants like drugs or foods that trigger haemolysis. However, subjects could experience acute crisis in a comorbidity state of diabetes, hypertension or infection. In addition, the condition could in turn predispose carriers to these chronic diseases. Aim of the study was to evaluate the frequency of haemolytic crisis in carriers with attendant chronic diseases, and also assess awareness and knowledge of aggravated reactions from these conditions. 282 subjects consented and participated in the study. A cross-sectional semi-structured interview questionnaire conducted from May to August 2013 was adopted. Questions focused on demographics, awareness and when disease was diagnosed, type and frequency of crisis, family history, screening and presence of other diseases and awareness of trigger from these conditions. A total of 69.9% were G6PD deficiency gene carriers and mostly fell within the age group of 31 - 40 (43.6%). 75.6% of the carriers were not aware that other disease conditions could exacerbate crisis. Non-drug related crisis was 85.3% compared with 14.7 drug related reactions. Out of the 69.9% carriers, 17.3% are diabetics, 18.3% of them are hypertensive and 25.9% are with bleeding disorders. Also, 29.1% had frequent episodes with bacterial infection. Symptoms experienced that were not drug related included, 44.8% attacks of pallor, 25.3% jaundice, 28.5% with shortness of breath, while 21.7% had dark urine frequently. No routine screening for other diseased conditions was carried out for these subjects despite experiencing frequent crisis that were not drug related. Carriers with other diseased conditions experienced more non-drug related crisis and were not aware of it.

Haemolytic Anaemia Following High Dose Intravenous Immunoglobulin Treatment for Epidermolysis Bullosa Acquisita

Background: Epidermolysis bullosa aquisita (EBA) is a severe acquired blistering skin disease that is often resistant to prednisolone but can respond well to intravenous immunoglobulin infusion (IVIg). Main Observations: We describe the case of a 35 years old male patient with EBA who developed clinically significant haemolytic anaemia with a drop in Hb from 15.3 g/dL to a nadir of 8.4 g/dL within 5 days post IVIg infusion. The patient was blood group A and the IVIg batch was found to have a high titre of anti-A immunoglobulin. Conclusions: IVIg is an effective treatment for EBA. Haemolysis associated with IVIg has not previously been reported in the dermatology literature but review of data from other specialties shows that the problem is well recognised. Dermatologists using IVIg should be aware of this potential complication and patients should be consented appropriately and warned about this potential side effect.

Pharmacological and biomedical properties of sea anemones Paracondactylis indicus,Paracondactylis sinensis,Heteractis magnifica and Stichodactyla haddoni from East coast of India

Objective:To explore the biomedical and pharmacological activity of Paracondactylis indicus (P.indicus).Paracondactylis sinensis(P.sinensis).Heteraclis magnified(H.magnified) and Stichadactyla haddoni(S.haddoni).Methods:The live sea anemones were kept inside the glass howl along with some amount of distilled water in an ice container for 15 min.During stress Condition,nemalocysts released from the tentacles were collected and centrifuged al 5 000 rpm for 15 min.The supernatant were collected ill separate cleaned beakers for lyophilisation.Results: The protein content of crude extracts was 15.2.28.7.18.2 and 35.4μg/mL.In hemolytic assay,the P.indicus was sensitive(16.842 HT/mg) on chicken blood but P.sinensis was less sensitive(1.114 HT/mg) on chicken and goal blood.Whereas H.magnified and S.haddoni showed hemolysis (0.879.0.903 HT/mg and 56.263.0.451 HT/mg) in chicken and goat blood.In antimicrobial assay, the methanol extract of P.indicus showed maximum inhibition zone of 9.7 mm against S.typhii and P.sinensis showed 9.8 mm against K.pneumonia in methanol and ethanol extracts.Whereas the H.magnified and S.haddoni showed maximum of 10 mm against S.typhii.K.pneumonia in methanol and ethanol extracts.Conclusions:The high toxic sea anemones may also contain some biologically active agents which has haemolytic,analgesic and anti-infilamatory activity.

Negative effects of Phaeocystis globosa on microalgae

The potential allelopathic effects of the microalga,Phaeocystis globosa Scherffel,on three harmful bloom algae,Prorocentrum donghaiense Lu,Chattonella marina(Subrahmanyan) Hara et Chihara and Chattonella ovata Hara et Chihara were studied.The growth of C.marina and C.ovata was markedly reduced when the organisms were co-cultured with P.globosa or cultured in cell-free spent medium.Haemolytic extracts from P.globosa cells in the senescence phase had a similar inhibitory effect on the three harmful bloom algae.However,P.globosa had less influence on the brine shrimp,Artemia salina.These results indicate that P.globosa may have an allelopathic effect on microalgae,which would explain the superior competitive abilities of P.globosa.Because the addition of the haemolytic toxins from P.globosa had similar effects on algae as spent media,these compounds may be involved in the allelopathic action of P.globosa.

Microangiopathic Hemolytic Anemia and Diffuse Bone Metastasis by Signet Ring Cell Adenocarcinoma

Microangiopathic hemolytic anemia (MAHA) is a rare paraneoplastic syndrome and is typically associated with gastric adenocarcinoma. We report a 47-year-old woman who presented with asthenia, lower back pain and bleeding. Twelve years ago the patient underwent total gastrectomy due to gastric adenocarcinoma and achieved complete remission. The patient was diagnosed with MAHA and diffuses bone metastasis of signet ring cell adenocarcinoma of unknown origin and was treated successfully with polichemotherapy based on cisplatin and 5-fluorouracil.

Bioactive potency of epidermal mucus extracts from greasy grouper,Epinephelus tauvina(Forsskal,1775)

Objective:To study the bio-potency of epidermal mucus fromEpinephelus tauvina.Methods:Mucus was extracted with acidic,organic and aqueous solvents.Protein,carbohydrate,lipid,amino acid and fatty acid content of mucus extracts were quantified by UV-spectrophotometer,high performance liquid chromatography and gas chromatography-mass spectrometer,respectively.Antimicrobial activity was tested against five human and fish pathogens by using agar well diffusion method.The molecular weight of peptides was determined using sodium dodecyl sulfate-polyacrylamide gel electrophoresis.The haemolytic activity of extracts was tested against chick,goat,cow and human red blood cell.Results:Protein contributed with maximum of 26.25%in crude mucus.Arginine was recorded maximum of(133.9 nmol/mL)in crude mucus.2,4,6-Decatrienoic acid and bis(a-chloroethyl)sulfone were confirmed in organic extract.The antimicrobial activity of acidic extract was significant.Among the human pathogens,maximum zone of inhibition[(26.0±0.3)mm]was observed againstProteus mirabilis.Whereas,among fish pathogens maximum zone of inhibition[(25.0±0.1)mm]was observed againstVibrio parahemolyticus.The activity of other two extracts was not remarkable.The molecular weight of peptides ranged from 115.5-37.1 kDa in acidic extract was determined.Chicken and goat blood were found to be highly vulnerable to the lysis.Conclusions:The whole mucus could be a promising source with numerous bioactive-potency.Consequently,this preliminary information suggested that mucus is a source of novel antimicrobial agents for fish and human health related applications.

Preliminary screening of Ni(II)metal tolerance and dye-decolorizing by Nocardiopsis sp.SD8

Objective:To reveal the screening of metal tolerance and dye-decolorizing of Nocardiopsis sp.Methods:NiSO_(4) and Congo red dye were used for evaluating the metal tolerance and dyedecolorizing of the randomly selected actinobacterial isolates.Results:Nocardiopsis sp.SD8 showed a better efficiency in Ni(II)tolerance,though a longer lag phase was observed for this microorganism grown for 7 days in integrated mismatch negativity.Interestingly,we also found that Nocardiopsis sp.SD8 had dye-decolorizing,hemolytic,lipase and protease activity.Conclusions:The present results revealed the bioremediation of metal resistant and diverse properties of Nocardiopsis sp.SD8 and further investigations are needed to extract and identify the potent molecule.