Pub Date : 2022-06-30DOI: 10.26420/jblooddisordl.2022.1069
S. S., Humaira Mahmood, Aneel Kapoor, S. Masood, A. Firdous, Shaher Murad
Coronary artery disease is a complex syndrome to get initiation from formation of atherosclerotic plaques in systemic circulation to cardiac arrhythmia causing morbidity/mortality. This study was conducted to compare hypolipidemic effects of Niacin and Indian date (Jujuba) in hyperlipidemic patients. Study was conducted from July 2019 to December 2019 at National Hospital Lahore- Pakistan. Sixty participants were enrolled of both gender male and female patients’ age range from 25 to 60 years. Consent was taken from all patients. They were divided in two groups. Group-I was advised to take 2 grams Niacin in divided doses for the period of two months. Group-II was advised to take 500 grams of fruit Jujube daily for the period of two months. All patients’ systolic and diastolic blood pressure was noted. Their baseline LDL and HDL cholesterol was determined by conventional method of measuring Lipid Profile. After two months therapy, their post treatment blood pressure and lipid profile was measured and mean values with ± SEM were analyzed biostatistically. Group-I which was on Niacin their blood pressure was reduced but it was non-significant change, LDL cholesterol decreased significantly and HDL cholesterol was increased significantly. In group-II patients LDL cholesterol was decreased significantly but HDL increase was not significant with p-value of >0.05. It was concluded from the research work that Niacin is potent in lowering LDL and increasing HDL cholesterol, while Jujube has significant effect as LDL cholesterol lowering potential, but it does not increase HDL cholesterol significantly. Jujubes and vitamin B-3 did not reduced blood pressure, when analyzed statistically.
{"title":"FOR LIVER: Herbs in Pakistani Population","authors":"S. S., Humaira Mahmood, Aneel Kapoor, S. Masood, A. Firdous, Shaher Murad","doi":"10.26420/jblooddisordl.2022.1069","DOIUrl":"https://doi.org/10.26420/jblooddisordl.2022.1069","url":null,"abstract":"Coronary artery disease is a complex syndrome to get initiation from formation of atherosclerotic plaques in systemic circulation to cardiac arrhythmia causing morbidity/mortality. This study was conducted to compare hypolipidemic effects of Niacin and Indian date (Jujuba) in hyperlipidemic patients. Study was conducted from July 2019 to December 2019 at National Hospital Lahore- Pakistan. Sixty participants were enrolled of both gender male and female patients’ age range from 25 to 60 years. Consent was taken from all patients. They were divided in two groups. Group-I was advised to take 2 grams Niacin in divided doses for the period of two months. Group-II was advised to take 500 grams of fruit Jujube daily for the period of two months. All patients’ systolic and diastolic blood pressure was noted. Their baseline LDL and HDL cholesterol was determined by conventional method of measuring Lipid Profile. After two months therapy, their post treatment blood pressure and lipid profile was measured and mean values with ± SEM were analyzed biostatistically. Group-I which was on Niacin their blood pressure was reduced but it was non-significant change, LDL cholesterol decreased significantly and HDL cholesterol was increased significantly. In group-II patients LDL cholesterol was decreased significantly but HDL increase was not significant with p-value of >0.05. It was concluded from the research work that Niacin is potent in lowering LDL and increasing HDL cholesterol, while Jujube has significant effect as LDL cholesterol lowering potential, but it does not increase HDL cholesterol significantly. Jujubes and vitamin B-3 did not reduced blood pressure, when analyzed statistically.","PeriodicalId":73627,"journal":{"name":"Journal of blood disorders & transfusion","volume":"87 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72788445","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-06-14DOI: 10.26420/jblooddisordl.2022.1068
Panjikaran Rj, P. K, S. K., Kottoor Sj
Vaso-occlusive crisis is the most common complication in sickle cell disease and can cause multi organ damage. Here we present a case of sickle cell anemia with vaso- occlusive crisis. The patient is a 39-year-old tribal male, a known case of homozygous sickle cell disease who presented with acute respiratory infection, bilateral lower limb pain and decreased urine output. Peripheral blood smear showed sickled red cells with features of hemolysis and reticulocytosis. Hemoglobin electrophoresis showed prominent HbS band. His symptoms progressed rapidly and he developed hematuria. Urine examination showed sickled erythrocytes. Later the patient went into cardiac arrest and succumbed to the disease. While hematuria is a common symptom in sickle cell disease, sickled RBCs in urine microscopy should be considered a bad prognostic indicator.
{"title":"Urinary Sickle Cells in a Patient with Vaso-Occlusive Crisis: A Case Report","authors":"Panjikaran Rj, P. K, S. K., Kottoor Sj","doi":"10.26420/jblooddisordl.2022.1068","DOIUrl":"https://doi.org/10.26420/jblooddisordl.2022.1068","url":null,"abstract":"Vaso-occlusive crisis is the most common complication in sickle cell disease and can cause multi organ damage. Here we present a case of sickle cell anemia with vaso- occlusive crisis. The patient is a 39-year-old tribal male, a known case of homozygous sickle cell disease who presented with acute respiratory infection, bilateral lower limb pain and decreased urine output. Peripheral blood smear showed sickled red cells with features of hemolysis and reticulocytosis. Hemoglobin electrophoresis showed prominent HbS band. His symptoms progressed rapidly and he developed hematuria. Urine examination showed sickled erythrocytes. Later the patient went into cardiac arrest and succumbed to the disease. While hematuria is a common symptom in sickle cell disease, sickled RBCs in urine microscopy should be considered a bad prognostic indicator.","PeriodicalId":73627,"journal":{"name":"Journal of blood disorders & transfusion","volume":"17 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89571884","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-04-13DOI: 10.26420/jblooddisordl.2022.1066
Gomes Rr
Dengue is a prevalent arthropod-borne viral disease in tropical and subtropical areas of the globe. Dengue clinical manifestations include asymptomatic infections; undifferentiated fever; dengue fever, which is characterized by fever, headache, retro orbital pain, myalgia, and arthralgia; and a severe form of the disease denominated dengue hemorrhagic fever/dengue shock syndrome, characterized by hemoconcentration, thrombocytopenia, and bleeding tendency. However, atypical manifestations, such as liver, central nervous system, and cardiac involvement, have been increasingly reported called expanded dengue syndrome. We report a 42 years old lady with atypical and rare presentation of dengue disease marked by non immune hemolysis following the critical phase of infection. Condition improved after conservative treatment. Hematological complications in dengue are now increasingly observed with the most common case is cytopenias and bleeding. Non immune hemolytic anemia in dengue is self-limiting in almost all cases. The main mechanism of hemolysis is still unknown though both direct viral infection and immune mediated damage have been suggested to be the cause. To avoid otherwise preventable morbidity and mortality, physicians should have a high index of suspicion for hematological complications in patients with dengue illness and should manage this accordingly.
{"title":"Dengue Hemorrhagic Fever and Non Immune Hemolytic Anemia: Two Recherché in Alliance","authors":"Gomes Rr","doi":"10.26420/jblooddisordl.2022.1066","DOIUrl":"https://doi.org/10.26420/jblooddisordl.2022.1066","url":null,"abstract":"Dengue is a prevalent arthropod-borne viral disease in tropical and subtropical areas of the globe. Dengue clinical manifestations include asymptomatic infections; undifferentiated fever; dengue fever, which is characterized by fever, headache, retro orbital pain, myalgia, and arthralgia; and a severe form of the disease denominated dengue hemorrhagic fever/dengue shock syndrome, characterized by hemoconcentration, thrombocytopenia, and bleeding tendency. However, atypical manifestations, such as liver, central nervous system, and cardiac involvement, have been increasingly reported called expanded dengue syndrome. We report a 42 years old lady with atypical and rare presentation of dengue disease marked by non immune hemolysis following the critical phase of infection. Condition improved after conservative treatment. Hematological complications in dengue are now increasingly observed with the most common case is cytopenias and bleeding. Non immune hemolytic anemia in dengue is self-limiting in almost all cases. The main mechanism of hemolysis is still unknown though both direct viral infection and immune mediated damage have been suggested to be the cause. To avoid otherwise preventable morbidity and mortality, physicians should have a high index of suspicion for hematological complications in patients with dengue illness and should manage this accordingly.","PeriodicalId":73627,"journal":{"name":"Journal of blood disorders & transfusion","volume":"3 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-04-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73195259","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background and purpose: Since growing intracranial aneurysms (IA) are more likely to rupture, detecting growth is an important part of unruptured IA follow-up. Recent studies have consistently shown that detecting IA growth can be challenging, especially in smaller aneurysms. In this study, we present an automated computational method to assist detecting aneurysm growth.
Materials and methods: An analysis program, Aneurysm Growth Evaluation & Detection (AGED) based on IA images was developed. To verify the program can satisfactorily detect clinical aneurysm growth, we performed this comparative study using clinical determinations of growth during IA follow-up as a gold standard. Patients with unruptured, saccular IA followed by diagnostic brain CTA to monitor IA progression were reviewed. 48 IA image series from twenty longitudinally-followed ICA IA were analyzed using AGED. A set of IA morphologic features were calculated. Nonparametric statistical tests and ROC analysis were performed to evaluate the performance of each feature for growth detection.
Results: The set of automatically calculated morphologic features demonstrated comparable results to standard, manual clinical IA growth evaluation. Specifically, automatically calculated HMAX was superior (AUC = 0.958) at distinguishing growing and stable IA, followed by V, and SA (AUC = 0.927 and 0.917, respectively).
Conclusion: Our findings support automatic methods of detecting IA growth from sequential imaging studies as a useful adjunct to standard clinical assessment. AGED-generated growth detection shows promise for characterization and detection of IA growth and time-saving comparing with manual measurements.
{"title":"Computer-Assisted Aneurysm Growth Evaluation and Detection (AGED): Comparison with Clinical Aneurysm Follow-Up.","authors":"Aichi Chien, Žiga Špiclin, Žiga Bizjak, Kambiz Nael","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background and purpose: </strong>Since growing intracranial aneurysms (IA) are more likely to rupture, detecting growth is an important part of unruptured IA follow-up. Recent studies have consistently shown that detecting IA growth can be challenging, especially in smaller aneurysms. In this study, we present an automated computational method to assist detecting aneurysm growth.</p><p><strong>Materials and methods: </strong>An analysis program, Aneurysm Growth Evaluation & Detection (AGED) based on IA images was developed. To verify the program can satisfactorily detect clinical aneurysm growth, we performed this comparative study using clinical determinations of growth during IA follow-up as a gold standard. Patients with unruptured, saccular IA followed by diagnostic brain CTA to monitor IA progression were reviewed. 48 IA image series from twenty longitudinally-followed ICA IA were analyzed using AGED. A set of IA morphologic features were calculated. Nonparametric statistical tests and ROC analysis were performed to evaluate the performance of each feature for growth detection.</p><p><strong>Results: </strong>The set of automatically calculated morphologic features demonstrated comparable results to standard, manual clinical IA growth evaluation. Specifically, automatically calculated HMAX was superior (AUC = 0.958) at distinguishing growing and stable IA, followed by V, and SA (AUC = 0.927 and 0.917, respectively).</p><p><strong>Conclusion: </strong>Our findings support automatic methods of detecting IA growth from sequential imaging studies as a useful adjunct to standard clinical assessment. AGED-generated growth detection shows promise for characterization and detection of IA growth and time-saving comparing with manual measurements.</p>","PeriodicalId":73627,"journal":{"name":"Journal of blood disorders & transfusion","volume":"13 8","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10174624/pdf/nihms-1889677.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9875094","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-11-25DOI: 10.26420/jblooddisordl.2021.1065
C. F
Background: Anemia induced by iron deficiency is a frequent co-morbidity of cardiovascular disease and is called as sideropenic anaemia. This is caused by low iron serum levels, with reduction of the hemoglobin value. Methods: Iron-deficiency reduces the oxygen carry throughout the body. The reduction of oxygen supply to the cardiac and pulmonary systems can point out some clinical symptoms, such as precordial angor, dyspnea, tachycardia and edema at lower limbs. It also induces an increase of some hemodynamic data, such as pulmonary and capillary pressures and worsens the prognosis of patients subjected to cardiac surgery. Results: Several data confirm that iron deficiency exerts detrimental effects in patients with coronary artery disease, heart failure, pulmonary hypertension and in those undergoing cardiac surgery. Conclusions: Iron deficiency further worsen the outcome and the complications of some cardiovascular disease. In addition, the manner and the advantages of i.v. iron administration (ferric carboxymaltose especially) were displayed. This administration may be requested in some conditions of cardiovascular disease and in the coexistence of these with kidney failure.
{"title":"Iron-Deficiency Anemias Worsen Some Cardiovascular Diseases: The Role of Intravenous Ferric Carboxymaltose","authors":"C. F","doi":"10.26420/jblooddisordl.2021.1065","DOIUrl":"https://doi.org/10.26420/jblooddisordl.2021.1065","url":null,"abstract":"Background: Anemia induced by iron deficiency is a frequent co-morbidity of cardiovascular disease and is called as sideropenic anaemia. This is caused by low iron serum levels, with reduction of the hemoglobin value. Methods: Iron-deficiency reduces the oxygen carry throughout the body. The reduction of oxygen supply to the cardiac and pulmonary systems can point out some clinical symptoms, such as precordial angor, dyspnea, tachycardia and edema at lower limbs. It also induces an increase of some hemodynamic data, such as pulmonary and capillary pressures and worsens the prognosis of patients subjected to cardiac surgery. Results: Several data confirm that iron deficiency exerts detrimental effects in patients with coronary artery disease, heart failure, pulmonary hypertension and in those undergoing cardiac surgery. Conclusions: Iron deficiency further worsen the outcome and the complications of some cardiovascular disease. In addition, the manner and the advantages of i.v. iron administration (ferric carboxymaltose especially) were displayed. This administration may be requested in some conditions of cardiovascular disease and in the coexistence of these with kidney failure.","PeriodicalId":73627,"journal":{"name":"Journal of blood disorders & transfusion","volume":"79 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-11-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73342527","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-11-23DOI: 10.26420/jblooddisordl.2021.1064
R. V, Y. U, K. P
More than 700 hemoglobinopathies are reported globally. In India, the most predominant hemoglobinopathies are HbS, and HbE. The objective of the present study was to determine the frequency of HbS in Eastern Uttar Pradesh population. The written consent was taken from each subject. 3 ml blood samples was collected from 350 selected anemic patients and genomic DNA was extracted from all the 350 collected blood samples. HbS mutation was analysed by PCR-RFLP method. Out of 350 samples analyzed, four individual was heterozygous (HbS/N) and one individuals were homozygous (HbS/S) for Hb S mutation. In conclusion, the overall βS allele frequency in Eastern Uttar Pradesh was observed as 0.86%.
{"title":"Molecular Screening of Hemoglobin S Variant in Anemia Patients of Eastern UP Population","authors":"R. V, Y. U, K. P","doi":"10.26420/jblooddisordl.2021.1064","DOIUrl":"https://doi.org/10.26420/jblooddisordl.2021.1064","url":null,"abstract":"More than 700 hemoglobinopathies are reported globally. In India, the most predominant hemoglobinopathies are HbS, and HbE. The objective of the present study was to determine the frequency of HbS in Eastern Uttar Pradesh population. The written consent was taken from each subject. 3 ml blood samples was collected from 350 selected anemic patients and genomic DNA was extracted from all the 350 collected blood samples. HbS mutation was analysed by PCR-RFLP method. Out of 350 samples analyzed, four individual was heterozygous (HbS/N) and one individuals were homozygous (HbS/S) for Hb S mutation. In conclusion, the overall βS allele frequency in Eastern Uttar Pradesh was observed as 0.86%.","PeriodicalId":73627,"journal":{"name":"Journal of blood disorders & transfusion","volume":"18 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-11-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90837766","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-06-25DOI: 10.26420/jblooddisordl.2021.1063
Z. Shahab-Movahed, A. Majd, Torbati Es, S. Zeinali
Introduction: Hereditary spherocytosis is a heterogeneous disorder with mild to moderate anemia. The aim of this study was to evaluate the inherited spherocytosis gene mutations in patients with RBC cytoplasmic disorders in Iranian population. Materials and Methods: In this study, Whole Exome Sequencing (WES) was performed for patients suspected to hereditary spherocytosis and their relatives. Results: Sequence analysis of the probands and their parents identified variations in ANK1 gene (NM_001142446.1:c.127-2A>G), SPTB (c. 14delC, p.Thr5LysfsTer41), SPTA1 (c.466C>T), SLC4A1 (c.2494C>T) and SLC25A38 gene (c.683G>T, NP_060345.2:p.Gly228Val that could be related to the patients clinical manifestation. Conclusion: Findings are in line with the appropriate diagnostic yield of WES in determining the causative variant especially in those disorders that many genes are involved like anemia. This is the first report of a cohort of Iranian patients with anemia suspected to that were investigated using WES technology. Further studies are needed to investigate the distribution of gene mutations in patients with RBC membrane disorders in Iran
{"title":"Molecular Investigation of Iranian Patients Suspected to Hereditary Spherocytosis","authors":"Z. Shahab-Movahed, A. Majd, Torbati Es, S. Zeinali","doi":"10.26420/jblooddisordl.2021.1063","DOIUrl":"https://doi.org/10.26420/jblooddisordl.2021.1063","url":null,"abstract":"Introduction: Hereditary spherocytosis is a heterogeneous disorder with mild to moderate anemia. The aim of this study was to evaluate the inherited spherocytosis gene mutations in patients with RBC cytoplasmic disorders in Iranian population. Materials and Methods: In this study, Whole Exome Sequencing (WES) was performed for patients suspected to hereditary spherocytosis and their relatives. Results: Sequence analysis of the probands and their parents identified variations in ANK1 gene (NM_001142446.1:c.127-2A>G), SPTB (c. 14delC, p.Thr5LysfsTer41), SPTA1 (c.466C>T), SLC4A1 (c.2494C>T) and SLC25A38 gene (c.683G>T, NP_060345.2:p.Gly228Val that could be related to the patients clinical manifestation. Conclusion: Findings are in line with the appropriate diagnostic yield of WES in determining the causative variant especially in those disorders that many genes are involved like anemia. This is the first report of a cohort of Iranian patients with anemia suspected to that were investigated using WES technology. Further studies are needed to investigate the distribution of gene mutations in patients with RBC membrane disorders in Iran","PeriodicalId":73627,"journal":{"name":"Journal of blood disorders & transfusion","volume":"28 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80746701","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-06-03DOI: 10.26420/jblooddisordl.2021.1062
S. Coimbra, Porto Portugal Ucibio Requimte, M. Mirand, M. Abreu, M. Lima, A. Santos-Silva
Pruritus is a major distressing symptom, common in inflammatory diseases, like Cutaneous T-Cell Lymphoma (CTCL) and mastocytosis. We aimed to study the involvement of some molecules, namely, cytokines, neuromediators, endothelial adhesion molecules and angiogenic factors, in the severity of pruritus associated to CTCL and mastocytosis. CTCL - Mycosis Fungoides (MF, n=17) and Sézary syndrome (SS, n=10) and mastocytosis patients (n=17) were evaluated. Interleukin (IL)-8, IL-31, Vascular Endothelial Growth Factor (VEGF), E-selectin, serotonin and C-reactive protein (CRP) levels, were assessed; tryptase was measured in mastocytosis. Pruritus severity was assessed, using a Visual Analogue Scale (VAS). Compared to controls (n=29), CTCL patients presented higher CRP and IL-31. SS patients had higher IL-31, E-selectin and CRP than MF patients and controls. Itch correlated with IL- 31 and E-selectin, when considering all CTCL patients; in SS, itch correlated with E-selectin. Advanced CTCL stages revealed higher IL-31, E-selectin and CRP than early stages, and controls; itch intensity correlated with IL-31 and E-selectin, in advanced stages. Mastocytosis showed higher serotonin and VEGF, compared to controls, and itch intensity correlated with tryptase. Data suggest that in mastocytosis, serotonin is an important biomarker and that tryptase levels reflect itch intensity; IL-31 and E-selectin appear to be more important mediators in CTCL and strongly correlated with itch severity. The different involvement of studied mediators, probably due to different immune responses, suggests that different mechanisms underlie these diseases and may lead to different itch mechanisms.
{"title":"Soluble Mediators Potentially Involved in Pruritus Associated to Cutaneous T-Cell Lymphomas and Mastocytosis: A Cross-Sectional Study","authors":"S. Coimbra, Porto Portugal Ucibio Requimte, M. Mirand, M. Abreu, M. Lima, A. Santos-Silva","doi":"10.26420/jblooddisordl.2021.1062","DOIUrl":"https://doi.org/10.26420/jblooddisordl.2021.1062","url":null,"abstract":"Pruritus is a major distressing symptom, common in inflammatory diseases, like Cutaneous T-Cell Lymphoma (CTCL) and mastocytosis. We aimed to study the involvement of some molecules, namely, cytokines, neuromediators, endothelial adhesion molecules and angiogenic factors, in the severity of pruritus associated to CTCL and mastocytosis. CTCL - Mycosis Fungoides (MF, n=17) and Sézary syndrome (SS, n=10) and mastocytosis patients (n=17) were evaluated. Interleukin (IL)-8, IL-31, Vascular Endothelial Growth Factor (VEGF), E-selectin, serotonin and C-reactive protein (CRP) levels, were assessed; tryptase was measured in mastocytosis. Pruritus severity was assessed, using a Visual Analogue Scale (VAS). Compared to controls (n=29), CTCL patients presented higher CRP and IL-31. SS patients had higher IL-31, E-selectin and CRP than MF patients and controls. Itch correlated with IL- 31 and E-selectin, when considering all CTCL patients; in SS, itch correlated with E-selectin. Advanced CTCL stages revealed higher IL-31, E-selectin and CRP than early stages, and controls; itch intensity correlated with IL-31 and E-selectin, in advanced stages. Mastocytosis showed higher serotonin and VEGF, compared to controls, and itch intensity correlated with tryptase. Data suggest that in mastocytosis, serotonin is an important biomarker and that tryptase levels reflect itch intensity; IL-31 and E-selectin appear to be more important mediators in CTCL and strongly correlated with itch severity. The different involvement of studied mediators, probably due to different immune responses, suggests that different mechanisms underlie these diseases and may lead to different itch mechanisms.","PeriodicalId":73627,"journal":{"name":"Journal of blood disorders & transfusion","volume":"45 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90165109","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-04-23DOI: 10.26420/JBLOODDISORDL.2021.1061
Moreb Js, Transplantation Hematologic Malignancies, K. Elliott, M. Verenes
We describe the case of a patient with high grade, triple hit, Non-Hodgkin’s Lymphoma (NHL) who underwent high-dose chemotherapy and Autologous Stem Cell Transplantation (ASCT) as a consolidation. Patient received BEAM conditioning regimen. She engrafted after usual post ASCT course. However, 2 months post ASCT she developed atypical neurologic symptoms and findings leading to general weakness mainly in the lower extremities with multiple falls, mental status changes and high CSF protein with severe sensorimotor neuropathy. She initially failed treatment with IVIg but responded to high dose steroids. More than one year after transplant, she has maintained her neurological improvement, but unable to walk, while her NHL continues to be in remission.
{"title":"Atypical Neurologic Complications Post Autologous Stem Cell Transplantation","authors":"Moreb Js, Transplantation Hematologic Malignancies, K. Elliott, M. Verenes","doi":"10.26420/JBLOODDISORDL.2021.1061","DOIUrl":"https://doi.org/10.26420/JBLOODDISORDL.2021.1061","url":null,"abstract":"We describe the case of a patient with high grade, triple hit, Non-Hodgkin’s Lymphoma (NHL) who underwent high-dose chemotherapy and Autologous Stem Cell Transplantation (ASCT) as a consolidation. Patient received BEAM conditioning regimen. She engrafted after usual post ASCT course. However, 2 months post ASCT she developed atypical neurologic symptoms and findings leading to general weakness mainly in the lower extremities with multiple falls, mental status changes and high CSF protein with severe sensorimotor neuropathy. She initially failed treatment with IVIg but responded to high dose steroids. More than one year after transplant, she has maintained her neurological improvement, but unable to walk, while her NHL continues to be in remission.","PeriodicalId":73627,"journal":{"name":"Journal of blood disorders & transfusion","volume":"8 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79501097","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-04-04DOI: 10.4172/2155-9864-C1-039
pIgor Krizajp
{"title":"The first intrinsic tenase complex inhibitor with serine protease structure: A new perspective in anticoagulant therapy","authors":"pIgor Krizajp","doi":"10.4172/2155-9864-C1-039","DOIUrl":"https://doi.org/10.4172/2155-9864-C1-039","url":null,"abstract":"","PeriodicalId":73627,"journal":{"name":"Journal of blood disorders & transfusion","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-04-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46520279","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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