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Indian Journal of Pathology &... Apr 2024Anemia coexisting with Gaucher disease (GD) is often associated with non-hemolytic processes. Few cases of GD with autoimmune hemolytic anemia have been reported....
Anemia coexisting with Gaucher disease (GD) is often associated with non-hemolytic processes. Few cases of GD with autoimmune hemolytic anemia have been reported. However, literature on GD with concomitant nonimmune hemolytic anemia is scarce. A 1-year 6-month-old male child presented in 2018 with complaints of palpable mass in left upper abdomen, fever, cough, and vomiting. On examination, he had pallor, hepatosplenomegaly of 2 cm and 8 cm below costal margin, respectively. A clinical diagnosis of hemolytic anemia was suspected. Complete blood count revealed Hb---6.7 g/dL, TLC---8.9 × 10 3 /μL, platelet count---180 × 10 3 /μL. Peripheral smear showed predominantly microcytic hypochromic anemia with moderate degree of anisocytosis, many nucleated red blood cells, few schistocytes, polychromatophils and corrected reticulocyte count 7.89%. S. Bilirubin was 1.1 mg/dL. Hb high-performance liquid chromatography (HPLC) of the child and his parents was within normal limit. Hematological work up revealed negative results for direct Coombs' test, osmotic fragility test, and sickling test. Test for Glucose-6-phosphate dehydrogenase deficiency was positive (39 units/trillion RBC, normal 146--376). He was transfused intermittently and given steroids to manage his anemia. He was on regular follow up during which his blood counts revealed persistent anemia and thrombocytopenia. In view of this, bone marrow was performed to exclude myelofibrosis. Aspirate smears were cellular and showed normoblastic erythroid hyperplasia. Numerous large histiocytes with basophilic fibrillary cytoplasm exhibiting "crumpled tissue paper" appearance were seen. Similar findings were seen on bone marrow trephine biopsy. Genetic testing revealed pathogenic variations in the GBA gene. Beta glucosidase enzyme levels were low while chitotriosidase was raised (1109.19 nmol/hr/mL). A final diagnosis of G6PD with GD was made. The present study shows rare association of GD with Glucose-6-phosphate dehydrogenase deficiency.
Topics: Humans; Gaucher Disease; Male; Glucosephosphate Dehydrogenase Deficiency; Infant; Bone Marrow
PubMed: 38391334
DOI: 10.4103/ijpm.ijpm_271_22 -
International Journal of Molecular... Feb 2021Liposomal technologies are used in order to improve the effectiveness of current therapies or to reduce their negative side effects. However, the liposome-erythrocyte...
Liposomal technologies are used in order to improve the effectiveness of current therapies or to reduce their negative side effects. However, the liposome-erythrocyte interaction during the intravenous administration of liposomal drug formulations may result in changes within the red blood cells (RBCs). In this study, it was shown that phosphatidylcholine-composed liposomal formulations of Photolon, used as a drug model, significantly influences the transmembrane potential, stiffness, as well as the shape of RBCs. These changes caused decreasing the number of stomatocytes and irregular shapes proportion within the cells exposed to liposomes. Thus, the reduction of anisocytosis was observed. Therefore, some nanodrugs in phosphatidylcholine liposomal formulation may have a beneficial effect on the survival time of erythrocytes.
Topics: Animals; Cell Shape; Chlorophyllides; Drug Compounding; Erythrocytes; Female; Hemolysis; Liposomes; Membrane Potentials; Phosphatidylcholines; Porphyrins; Radiation-Sensitizing Agents; Swine
PubMed: 33567766
DOI: 10.3390/ijms22041710 -
Frontiers in Cardiovascular Medicine 2022Recent studies suggest that the pivotal mechanism of sodium glucose co-transporter-2 inhibitors (SGLT-2i) favorable action in patients with heart failure (HF) and type 2...
BACKGROUND
Recent studies suggest that the pivotal mechanism of sodium glucose co-transporter-2 inhibitors (SGLT-2i) favorable action in patients with heart failure (HF) and type 2 diabetes mellitus (DM) is the stimulation of erythropoiesis an early increase in erythropoietin (EPO) production which leads to hematocrit rise. Red blood cell distribution width (RDW) is a simple hematological parameter which reflects the heterogeneity of the red blood cell size (anisocytosis). Since, EPO has been also implicated in the pathophysiology of RDW increase, the current mechanistic study examined the effect of SGLT-2i administration on red blood cells size (RDW) in patients with HF and DM.
METHODS
The present was a prospective single-center study. Patients (N=110) were randomly assigned to dapagliflozin (10 mg a day on top of antidiabetic treatment) or the control group. Inclusion criteria were: (a) age > 18 years, (b) history of type 2 DM and hospitalization for HF exacerbation within 6 months. The evaluation of patients (at baseline, 6 and 12 months) included clinical assessment, laboratory blood tests, and echocardiography. Data were modeled using mixed linear models with dependent variable the RDW index. In order to find factors independently associated with prognosis (1-year death or HF rehospitalization), multiple logistic regression was conducted with death or HF rehospitalization as dependent variable.
RESULTS
An RDW increase both after 6 and after 12 months was observed in the SGLT-2i (dapagliflozin) group ( < 0.001 for all time comparisons), whereas RDW didn't change significantly in the control group. The increase in RDW was positively correlated with EPO, while negatively correlated with ferritin and folic acid ( < 0.005 for all). Baseline RDW was significantly associated with 1-year death or rehospitalization, after adjusting for group (SGLT-2i vs. control), age, gender, smoking and BMI at baseline.
CONCLUSION
RDW increased with time in patients with HF and DM who received SGLT-2i (dapagliflozin). The increased RDW rates in these patients may stem from the induction of hemopoiesis from dapagliflozin. Baseline RDW was found to be independently associated with outcome in patients with HF and DM.
PubMed: 36247420
DOI: 10.3389/fcvm.2022.984092 -
Diagnostics (Basel, Switzerland) Aug 2020Since previous evidence has demonstrated that red blood cell distribution width (RDW) may be a useful prognostic parameter in many critical illnesses and infectious...
Since previous evidence has demonstrated that red blood cell distribution width (RDW) may be a useful prognostic parameter in many critical illnesses and infectious diseases, we investigated the utility of RDW for monitoring patients with coronavirus disease 2019 (COVID-19). The study population consisted of 49 COVID-19 patients, including 16 (32.6%) with severe illness, 12 (24.5%) with severe acute kidney injury (AKI), and 8 (16.3%) requiring renal replacement therapy (RRT). The predictive value of blood tests, performed during emergency department evaluation, was then addressed. A progressive increase of RDW was observed with advancing COVID-19 severity. The area under the curve (AUC) of RDW was 0.73 for predicting severe illness, 0.80 for severe AKI, and 0.83 for RRT, respectively. In multivariate analysis, elevated RDW was associated with 9-fold and 16-fold increased odds of severe COVID-19 and AKI, respectively. The results of this study suggest that RDW should be part of routine laboratory assessment and monitoring of COVID-19.
PubMed: 32825629
DOI: 10.3390/diagnostics10090618 -
Journal of Clinical Medicine Nov 2019Autoimmune atrophic gastritis (AAG) leads to iron and/or vitamin B12 malabsorption, with subsequent haematological alterations which could represent the sole clinical...
BACKGROUND
Autoimmune atrophic gastritis (AAG) leads to iron and/or vitamin B12 malabsorption, with subsequent haematological alterations which could represent the sole clinical manifestation. We aimed to assess patterns of anaemia and micronutrient deficiencies in patients with AAG at the time of diagnosis.
METHODS
Observational, multicentre, cross-sectional study including consecutive adult patients diagnosed with AAG within the last ten years. Cell blood count, red cell distribution width, serum vitamin B12, and ferritin were collected. Multivariate analysis for predictive factors of anaemia was computed.
RESULTS
654 AAG patients (mean age 59.2 ± 13.8 years, female (F): male (M) ratio = 2.3:1) were included. Anaemia was present in 316 patients (48.3%; mean age 60.1 ± 15.8 years, F:M ratio = 2.3:1). Pernicious anaemia (132/316 cases, 41.7%) was more common in males (27.1% versus 12.4%; = 0.001) and in older patients (63.0 ± 14.6 versus 58.9 ± 14.9 years; = 0.014), while iron deficiency anaemia (112/316 cases, 35.4%) was more common in females (16.9% versus 10.0%; = 0.039) and in younger patients (56.8 ± 16.6 versus 60.2 ± 14.6 years; = 0.043). The prevalence of iron deficiency was equally distributed between anaemic and non-anaemic patients ( = 0.9). Anisocytosis (odds ratio: 10.65, 95% confidence interval: 6.13-18.50, < 0.0001) was independently associated with anaemia.
CONCLUSIONS
Anaemia is a common manifestation in AAG patients, mostly due to micronutrient deficiencies. Scant haematologic alterations and micronutrient deficiencies may precede overt anaemia.
PubMed: 31731715
DOI: 10.3390/jcm8111992 -
Veterinary Medicine International 2021Hepatocellular carcinoma (HCC) is one of the most common neoplasms that has been described in many domestic animal species. Hence, the disease has significant economic...
Hepatocellular carcinoma (HCC) is one of the most common neoplasms that has been described in many domestic animal species. Hence, the disease has significant economic importance; thus, this study aimed to describe the cytopathological characteristics of hepatocellular carcinomas in cattle slaughtered at Bishoftu Elfora Abattoir, Central Ethiopia. A cross-sectional study design with a purposive sampling technique was performed from October 2017 to May 2018 using macroscopic, histopathologic, and cytological methods. For that matter, a total of sixty cattle were assessed for the presence of a hepatic tumor; however, only 1/60 (0.6%) case was found to be affected by hepatocellular carcinomas. On gross examination, hepatocellular carcinomas exhibited soft, white, multifocal nodules (10-40 mm in diameter) on different lobes of the liver. On the cut surface, the tumor revealed a sharply circumscribed border and was divided into lobules by thin connective tissue. The central zone of the tumors exhibited depression with a whitish fibrous area. Moreover, on histopathology, the tumors divulged unencapsulated carcinomatous lesions consisting of a thick, compact, somewhat ambiguous trabecular pattern of arrangement that was unglued by thin collagenous stroma. Cytological studies suggest that the tumor cells showed anisocytosis, anisokaryosis, prominent nucleoli, multinuclearity, palisading arrangements of neoplastic cells, increased N : C ratios, light eosinophilic cytoplasm, high mitotic index, and cytoplasmic and intranuclear vacuoles. In conclusion, cytopathological findings support a diagnosis of HCC in the liver; thus, further studies with a large sample size and use of immunohistochemistry are important for further characterization of hepatocellular carcinomas in cattle.
PubMed: 34035888
DOI: 10.1155/2021/6649172