In this study, we are describing a female patient with paroxysmal nocturnal hemoglobinuria (PNH) and glucose-6-phosphate dehydrogenase (G6PD) deficiency. Both diseases are known to cause hemolytic anemia that mediates the hemolysis of RBCs through several mechanisms. In PNH the hemolysis is mediated through complement activation and oxidative stress. G6PD enzyme is crucial in preventing damage to cellular structures caused by oxygen-free radicles. In G6PD deficiency the hemolysis is mediated through the oxidative stress created by oxygen-free radicles. Since both diseases mediate hemolysis through the oxidative stress, we hypothesize that both conditions have facilitated an effect on each other and this will reflect on the response to treatment, and this response to treatment could vary based on whether the two mutations occurred in the same gene or in two different X chromosomes. Having diagnosed PNH, the management is very expensive and not all the patients can afford it, especially our patient who is a maid by occupation. So, the real challenge in our case is to monitor her in subsequent visits and to plan the treatment keeping in mind her financial status.
Aim: the aim of this study was to compare the impact of silymarin on the liver fibrosis induced by diethylnitrosamine (DEN) between both sexes of Wistar rats and proposing possible mechanisms.Main Methods: twenty-four Wistar male and twenty-four Wistar female rats were randomly assigned into 8 groups according to their sex (n=6) for administration of vehicle, DEN, silymarin or both DEN and silymarin for 8 weeks. At the end of the experiment, the traditional rat body and liver weight parameters, liver injury biomarkers (serum ALT, AST, ALP, and total bilirubin) were measured. Furthermore, hematological parameters, lipid profiles (TC, LDL-C, HDL-C, and TG) and oxidative stress biomarkers (TBARS, SOD, CAT, and GSH) were determined. Also, the inflammatory biomarkers in liver tissue homogenate (TNF-α, TGF-β) were evaluated. Histopathological subjective scoring system graded the damage markers of liver tissue. Expression of NF-kB was measured immunohistochemically.Results: Markedly diminished DEN induced liver fibrosis markers in female groups while worsened in male groups. Silymarin regimen improved liver functions and fibrosis markers. Additionally, it counteracted DEN-induced oxidative stress, lipid peroxidation and inflammations, silymarin provided these ameliorative effects either in males or females rats. Conclusion: Silymarin plays an ameliorative role of DEN-induced liver fibrosis in male and female rats via reducing oxidative stress and inflammations.
Introduction Severe IDA can cause many complications and impair the quality of life. Iron is an essential micronutrient required for catalysis, DNA synthesis, redox reactions and oxygen transport1. It is important for an early step in embryonic haematopoiesis, which is common for all developing blood cells. The link between IDA and leukopenia is not well recognized in the literature. Objectives To assess the prevalence and clinical significance of leukopenia in patients with IDA and effect of iron replacement and correction of anemia on the WBCs count. Materials and Methods We retrospectively reviewed the electronic medical records of all patients attended haematology clinic with the diagnosis of iron deficiency anemia (IDA) over 2 years in Hamad Medical Corporation, Qatar. All other causes of anemia and patients with systemic or chronic diseases were excluded. Age, nationality, BMI, Complete blood count and iron parameters were collected before and after treatment with IV iron therapy. Associated infections at the time of presentation (IDA and leukopenia) were noted including the course of the infection and response to treatment. Leukopenia was defined as WBCs count below 4000/microlitre. Statistical analysis was done using paired t test to compare variables after versus before iron therapy. Results Out of 1567 case of iron deficiency anemia, 80 case had leukopenia (5.105%) Their mean Leukocytes count was 3.35 +/- 0.48 ×103 before iron replacement. 7 patients had infections; 4 had upper respiratory tract infection, 1 urinary tract infection, 1 gastroenteritis, 1 lymphadenitis. Six of them received antibiotics and they had no complications. After iron therapy and correction of anemia the leukocyte count increased significantly to 4.38 +/- 1.82×103 (P < 0.05). There was no significant correlation between WBC count and iron parameters (Hb, TIBC, serum iron concentration). Discussion High level of erythropoietin in IDA is thought to cause down regulation of neutrophils in animal models. In our study leukopenia occurred in 5.1% of the big cohort with IDA. A previous study on 516 patients with IDA recorded leukopenia in 17.6% of them. Their cases with leukopenia occurred more in patients with severe anemia. The increase of WBC count with correction of anemia suggested a physiologic link between erythropoiesis and leukopoiesis. However, our study did not show correlation between WBC count and Hb or any of the iron parameters. In concert with our finding, a study in healthy children (n = 556) did not find associations between the measured iron markers and WBC In addition, the association between IDA and leukopenia did not significantly increase the risk of infections in our patients. The link between leukopenia and IDA needs to be addressed in more studies. Conclusions: The prevalence of leukopenia in this big cohort with IDA was 5.1%. This leukopenia was not associated with severe or complicated infections. There were no associations between the measured iron markers and white blood cell counts in healthy adults Figure Disclosures No relevant conflicts of interest to declare.
Introduction Iron deficiency anemia (IDA) is a major public health issue, with widespread prevalence and negative impact on health care system. IDA occurs when iron stores diminish to the level that disturbs erythropoiesis and causes anemia. Neutropenia is an abnormal reduction in the number of neutrophils. A little is known about the association between iron deficiency anemia and neutropenia. The aim of this study is to investigate the prevalence of neutropenia in a large cohort of adult patients with IDA and to find possible correlation between neutrophil levels with haemoglobin concentration and iron stores. We studied associated infections in this neutropenic group. Materials and Methods We retrospectively reviewed the electronic medical records of 1567 patients attended haematology clinic with the diagnosis of IDA ((haemoglobin level less than 12 gm/dl for women, less than 13 gm/dl for men) over the past 2 years in Hamad Medical Corporation, Qatar. Other causes of anemia and anemia associated with any systemic or endocrine disease were excluded. The values of complete blood count (CBC) and iron parameters were collected. Neutropenia was defined as neutrophils count to be less than 1.5x 109/L Results Sixty four patients of the 1567 cohort with IDA had neutropenia (4.084%) Their mean neutrophils count = 1.18 +/- 0.28x 109/L before iron replacement. Neutrophil count increased significantly to 2.33 +/- 1x 109/L after iron therapy (p< 0.05) . No significant correlation was detected between neutrophil count on the one hand and iron level, iron saturation, TIBC, Transferrin and ferritin level on the other hand. Eight out of the 64 patients with neutropenia had infection at the time of presentation; 5 upper respiratory tract infections, 1 gastroenteritis, 1 lymphadenitis, 1 urinary tract infections. Five of these patients received antibiotics, with no complications reported. Discussion In our cohort with IDA the prevalence of neutropenia was 4.08%. In all patients, the neutrophil count returned to normal after proper iron therapy. The effect of iron deficiency on neutrophils count is through its effect on haematopoiesis progenitors and bone marrow microenvironment which regulates the production of cell lineages. In addition, the high level of erythropoietin (observed in IDA) has been shown to down-regulate neutrophil production in animal models. In another relatively smaller study on 516 patients with IDA, neutropenia was found in 17.6% However, unlike in our study the neutrophil count was correlated with Hb level. On the other hand in 97 patients with unexplained neutropenia, IDA was found in 2.1% with correction of neutrophil count after correction of the anemia. In addition, the associated infection rate was low and was treated without complications. Our findings support a possible link between IDA and neutropenia evident by the improvement of neutrophils count after iron replacement, (1.18 vs 2.33x 109/). Conclusions: The finding of neutropenia is not uncommon in patients with IDA. This neutropenia markedly improved after iron replacement. Iron is essential for proper development and maintenance of the immune system in general and further studies are required to elaborate further in this unique association. Figure 1 Disclosures No relevant conflicts of interest to declare.
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