BACKGROUND: Sickle cell nephropathy, a heterogeneous group of renal abnormalities resulting from complex interactions of sickle cell disease (SCD)-related factors and non-SCD phenotype characteristics, is associated with an increased risk for morbidity and mortality. AIMS: The aims of this study were to determine the frequency of microalbuminuria (MA) among pediatric patients with SCD and to determine risk factors for MA among those patients. SUBJECTS AND METHODS: A case–control study was carried out on 120 patients with SCD, 2–18 years old, registered at Basrah Center for Hereditary Blood Diseases, and 132 age- and sex-matched healthy children were included as a control group. Investigations included complete blood panel, blood urea, serum creatinine (Cr), urinalysis, and urinary albumin-to-Cr ratio (ACR). Logistic regression analysis was used to assess the predictors of MA. RESULTS: Among SCD patients, 39 (32.5%) had MA compared to 6 (4.5%) in the control group. The mean levels of blood urea, serum Cr, and ACR were significantly higher, and the urine-specific gravity was significantly lower in SCD patients than in the control group (P < 0.05). Logistic regression analysis revealed that frequent painful crisis (odds ratio [OR]: 12.146, confidence interval [CI]: 3.439–42.952), high serum ferritin (OR: 8.146, CI: 1.802–36.827), deferoxamine therapy (OR: 23.423, CI: 3.961–60.509), and female sex (OR: 4.590, CI: 1.225–17.202) are independent risk factors for MA (P < 0.05). CONCLUSION: The frequency of MA was high among our pediatric SCD patients. Risk factors for MA include female sex, nutritional factors, painful episodes, and iron overload. This is important for planning for future follow-up and management of this common disease in our locality.
{"title":"Microalbuminuria among children and adolescents with sickle cell disease","authors":"Meaad K Hassan, L. Al-Naama, Sammer Jawad","doi":"10.4103/ijh.ijh_17_22","DOIUrl":"https://doi.org/10.4103/ijh.ijh_17_22","url":null,"abstract":"BACKGROUND: Sickle cell nephropathy, a heterogeneous group of renal abnormalities resulting from complex interactions of sickle cell disease (SCD)-related factors and non-SCD phenotype characteristics, is associated with an increased risk for morbidity and mortality. AIMS: The aims of this study were to determine the frequency of microalbuminuria (MA) among pediatric patients with SCD and to determine risk factors for MA among those patients. SUBJECTS AND METHODS: A case–control study was carried out on 120 patients with SCD, 2–18 years old, registered at Basrah Center for Hereditary Blood Diseases, and 132 age- and sex-matched healthy children were included as a control group. Investigations included complete blood panel, blood urea, serum creatinine (Cr), urinalysis, and urinary albumin-to-Cr ratio (ACR). Logistic regression analysis was used to assess the predictors of MA. RESULTS: Among SCD patients, 39 (32.5%) had MA compared to 6 (4.5%) in the control group. The mean levels of blood urea, serum Cr, and ACR were significantly higher, and the urine-specific gravity was significantly lower in SCD patients than in the control group (P < 0.05). Logistic regression analysis revealed that frequent painful crisis (odds ratio [OR]: 12.146, confidence interval [CI]: 3.439–42.952), high serum ferritin (OR: 8.146, CI: 1.802–36.827), deferoxamine therapy (OR: 23.423, CI: 3.961–60.509), and female sex (OR: 4.590, CI: 1.225–17.202) are independent risk factors for MA (P < 0.05). CONCLUSION: The frequency of MA was high among our pediatric SCD patients. Risk factors for MA include female sex, nutritional factors, painful episodes, and iron overload. This is important for planning for future follow-up and management of this common disease in our locality.","PeriodicalId":53847,"journal":{"name":"Iraqi Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":0.1,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47996822","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: Congo–Crimean hemorrhagic fever is a tick-borne zoonotic viral disease caused by Crimean–Congo hemorrhagic fever virus (CCHFV). The outbreak in Pakistan is increased during Eid-ul-Adha. We describe a cluster of cases that presented to our hospital. AIMS: The aim of this study was to determine the outcome of Crimean–Congo hemorrhagic fever-positive cases from January 2011 to August 2019. MATERIALS AND METHODS: Retrospective data were retrieved using the International Classification of Diseases version 9. We received 2101 samples for testing. Polymerase chain reaction (PCR)-positive cases were included in the study. History of bleeding and animal contact was recorded. Variables analyzed were age, gender, profession, and coinfection with other viral illnesses. RESULTS: A total of 70 PCR-positive cases were included in the study (frequency 3.3%). Sixty-one were males and nine were females. Fever was present in all cases. Epistaxis was noted in 54% of patients. Thrombocytopenia was present in all patients. Eighteen patients were butchers, six were shopkeepers, nine students, and few laborers. All females were housewives. Twenty-two patients had a history of contact with animals. Seven patients were coinfected with viral hepatitis. There were 23 (33%) deaths. CONCLUSION: Overall mortality was 33%. Twenty-two patients had a history of contact with animals. There is a strong need for public education, especially during the month of Eid-ul-Adha.
{"title":"Clinico-hematological features and outcome of patients affected by Congo–Crimean hemorrhagic fever: An experience from a single center","authors":"M. Shaikh, N. Ali, A. Memon","doi":"10.4103/ijh.ijh_44_22","DOIUrl":"https://doi.org/10.4103/ijh.ijh_44_22","url":null,"abstract":"BACKGROUND: Congo–Crimean hemorrhagic fever is a tick-borne zoonotic viral disease caused by Crimean–Congo hemorrhagic fever virus (CCHFV). The outbreak in Pakistan is increased during Eid-ul-Adha. We describe a cluster of cases that presented to our hospital. AIMS: The aim of this study was to determine the outcome of Crimean–Congo hemorrhagic fever-positive cases from January 2011 to August 2019. MATERIALS AND METHODS: Retrospective data were retrieved using the International Classification of Diseases version 9. We received 2101 samples for testing. Polymerase chain reaction (PCR)-positive cases were included in the study. History of bleeding and animal contact was recorded. Variables analyzed were age, gender, profession, and coinfection with other viral illnesses. RESULTS: A total of 70 PCR-positive cases were included in the study (frequency 3.3%). Sixty-one were males and nine were females. Fever was present in all cases. Epistaxis was noted in 54% of patients. Thrombocytopenia was present in all patients. Eighteen patients were butchers, six were shopkeepers, nine students, and few laborers. All females were housewives. Twenty-two patients had a history of contact with animals. Seven patients were coinfected with viral hepatitis. There were 23 (33%) deaths. CONCLUSION: Overall mortality was 33%. Twenty-two patients had a history of contact with animals. There is a strong need for public education, especially during the month of Eid-ul-Adha.","PeriodicalId":53847,"journal":{"name":"Iraqi Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":0.1,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43382673","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}
Etoposide is a chemotherapeutic agent that belongs to the podophyllotoxin drug class. Etoposide is used in treating many types of cancers including blood cancers. However, hypersensitivity reactions to etoposide and other chemotherapeutic agents are common. A 29-year-old female was admitted to the bone marrow transplant center for autologous hematopoietic stem cell transplantation. She was previously diagnosed with Hodgkin's lymphoma. The Lomustine, Etoposide, Cytarabine and Melphalan, (LEAM) protocol has been prescribed as a conditioning regimen before stem cell transplantation for this patient. On the 4th day of LEAM protocol, after the last day of the etoposide dose, the patient develops a severe allergic reaction to etoposide. After investigation, we found that the patient was allergic to penicillin which also appeared when the patient takes piperacillin + tazobactam. The possibility of cross-allergic reactions between etoposide and penicillin is unknown. The cross-allergic reactions between etoposide and penicillin are not reported in previous studies.
{"title":"Cross-allergic reactions between etoposide and penicillin in autologous bone marrow transplant patient","authors":"A. Alsajri, M. Shubber, W. Al-Qerem","doi":"10.4103/ijh.ijh_25_22","DOIUrl":"https://doi.org/10.4103/ijh.ijh_25_22","url":null,"abstract":"Etoposide is a chemotherapeutic agent that belongs to the podophyllotoxin drug class. Etoposide is used in treating many types of cancers including blood cancers. However, hypersensitivity reactions to etoposide and other chemotherapeutic agents are common. A 29-year-old female was admitted to the bone marrow transplant center for autologous hematopoietic stem cell transplantation. She was previously diagnosed with Hodgkin's lymphoma. The Lomustine, Etoposide, Cytarabine and Melphalan, (LEAM) protocol has been prescribed as a conditioning regimen before stem cell transplantation for this patient. On the 4th day of LEAM protocol, after the last day of the etoposide dose, the patient develops a severe allergic reaction to etoposide. After investigation, we found that the patient was allergic to penicillin which also appeared when the patient takes piperacillin + tazobactam. The possibility of cross-allergic reactions between etoposide and penicillin is unknown. The cross-allergic reactions between etoposide and penicillin are not reported in previous studies.","PeriodicalId":53847,"journal":{"name":"Iraqi Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":0.1,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45480494","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: Endothelial dysfunction is a likely pathogenic mechanism in hypertensive disorders of pregnancy leading to a hypercoagulable state. OBJECTIVE: The present study aims to measure thrombomodulin (TM) in patients with preeclampsia (PE) and gestational hypertension (GH) and compare them with healthy pregnant control and its relation to disease severity and associated hematological parameters. MATERIALS AND METHODS: This cross-sectional study was done for 80 participants, 30 preeclamptic, 30 GH patients, and 20 healthy age-matched pregnant from all TM assays were done in by an enzyme-linked immunosorbent assay. Other hematological parameters including complete blood count, prothrombin time, and activated partial thromboplastin time where assessed in these patients. RESULTS: TM level was significantly higher in patients with PE when compared to both women with GH and normal pregnant women (P = 0.009) and (P < 0.001), respectively. Likewise, TM level was significantly higher in patients with GH when compared to healthy pregnant controls (P = 0.034). Plasma TM level was found to be 77% sensitive and 75% specific for the diagnosis of PE (the area under the curve was 0.835) at a 95% confidence interval. CONCLUSION: TM is significantly elevated in pregnant women with PE and GH and is associated with the severity of the disease.
{"title":"Increased thrombomodulin level in hypertensive disorders of pregnancy","authors":"Z. Hashim, Bassam M. Hameed","doi":"10.4103/ijh.ijh_46_22","DOIUrl":"https://doi.org/10.4103/ijh.ijh_46_22","url":null,"abstract":"BACKGROUND: Endothelial dysfunction is a likely pathogenic mechanism in hypertensive disorders of pregnancy leading to a hypercoagulable state. OBJECTIVE: The present study aims to measure thrombomodulin (TM) in patients with preeclampsia (PE) and gestational hypertension (GH) and compare them with healthy pregnant control and its relation to disease severity and associated hematological parameters. MATERIALS AND METHODS: This cross-sectional study was done for 80 participants, 30 preeclamptic, 30 GH patients, and 20 healthy age-matched pregnant from all TM assays were done in by an enzyme-linked immunosorbent assay. Other hematological parameters including complete blood count, prothrombin time, and activated partial thromboplastin time where assessed in these patients. RESULTS: TM level was significantly higher in patients with PE when compared to both women with GH and normal pregnant women (P = 0.009) and (P < 0.001), respectively. Likewise, TM level was significantly higher in patients with GH when compared to healthy pregnant controls (P = 0.034). Plasma TM level was found to be 77% sensitive and 75% specific for the diagnosis of PE (the area under the curve was 0.835) at a 95% confidence interval. CONCLUSION: TM is significantly elevated in pregnant women with PE and GH and is associated with the severity of the disease.","PeriodicalId":53847,"journal":{"name":"Iraqi Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":0.1,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46948051","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}
Ahmet Kaya, M. Erkurt, I. Kuku, E. Kaya, İ. Berber, S. Biçim, Emine Hidayet, F. Yağın, A. Sarici
BACKGROUND: In allogeneic stem cell transplantation, donor and recipient HLA tissue compatibility is essential for the success of stem cell transplantation. HLA tissue compatibility percentage is the most important parameter that increases the success of transplantation in donor selection. Pretransplant HLA tissue typing can be looked at in low and high resolution according to the facilities of the center and the urgency of transplantation. Many centers evaluate HLA HLA-A, B, C, DP, DQ, DR tissue types before allogeneic bone marrow transplantation. HLA tissue types differ in many races and even between unrelated individuals of the same race. AIMS: This study aimed to show the common human leukocyte antigen (HLA) rates and differences in Syrian and Turkish ethnicity patients who underwent allogeneic stem cell transplantation in our center. MATERIALS AND METHODS: HLA tissue similarities between Turkish and Syrian patients were revealed by examining the HLA tissue records of Turkish and Syrian patients who applied to the bone marrow transplant unit of Inonu University Turgut Ozal Medical Center between December 2009 and November 2021 for allogeneic stem cell transplantation. RESULTS: As a result of our study, it has been observed that there are similarities in terms of HLA A*02, HLA B*35, HLA C 04,07,12, HLA DP*02,04,11 HLA DQ*02,03,05,06, HLA DR*01,03,11,13 in Turkish and Syrian patients. High resolution HLA subgroups of the patients are shown in Tables three and four. CONCLUSION: In allogeneic stem cell transplantation, there may be similar HLA tissue types among ethnic groups.
背景:在同种异体干细胞移植中，供体和受体HLA组织相容性是干细胞移植成功的关键。在供体选择中，HLA组织相容性百分比是提高移植成功率的最重要参数。移植前HLA组织分型可根据中心的设施和移植的紧急程度进行高低分辨率的观察。许多中心在异体骨髓移植前评估HLA- a、B、C、DP、DQ、DR组织类型。HLA组织类型在许多种族中是不同的，甚至在同一种族的不相关个体之间也是不同的。目的:本研究旨在显示在我们中心接受同种异体干细胞移植的叙利亚和土耳其种族患者的共同人类白细胞抗原(HLA)率和差异。材料与方法:通过对2009年12月至2021年11月在Inonu大学Turgut Ozal医学中心骨髓移植单元申请同种异体干细胞移植的土耳其和叙利亚患者的HLA组织记录进行分析，揭示土耳其和叙利亚患者HLA组织的相似性。结果:我们的研究发现，土耳其和叙利亚患者HLA a *02、HLA B*35、HLA C 04、07、12、HLA DP*02、04、11、HLA DQ*02、03、05、06、HLA DR*01、03、11、13具有相似性。患者高分辨率HLA亚群见表3和表4。结论:在同种异体干细胞移植中，不同民族间可能存在相似的HLA组织类型。
{"title":"The frequency of HLA A, B, C, DP, DQ, DR allele in patients of Turkish and Syrian nationals with allogeneic stem cell transplantation","authors":"Ahmet Kaya, M. Erkurt, I. Kuku, E. Kaya, İ. Berber, S. Biçim, Emine Hidayet, F. Yağın, A. Sarici","doi":"10.4103/ijh.ijh_21_22","DOIUrl":"https://doi.org/10.4103/ijh.ijh_21_22","url":null,"abstract":"BACKGROUND: In allogeneic stem cell transplantation, donor and recipient HLA tissue compatibility is essential for the success of stem cell transplantation. HLA tissue compatibility percentage is the most important parameter that increases the success of transplantation in donor selection. Pretransplant HLA tissue typing can be looked at in low and high resolution according to the facilities of the center and the urgency of transplantation. Many centers evaluate HLA HLA-A, B, C, DP, DQ, DR tissue types before allogeneic bone marrow transplantation. HLA tissue types differ in many races and even between unrelated individuals of the same race. AIMS: This study aimed to show the common human leukocyte antigen (HLA) rates and differences in Syrian and Turkish ethnicity patients who underwent allogeneic stem cell transplantation in our center. MATERIALS AND METHODS: HLA tissue similarities between Turkish and Syrian patients were revealed by examining the HLA tissue records of Turkish and Syrian patients who applied to the bone marrow transplant unit of Inonu University Turgut Ozal Medical Center between December 2009 and November 2021 for allogeneic stem cell transplantation. RESULTS: As a result of our study, it has been observed that there are similarities in terms of HLA A*02, HLA B*35, HLA C 04,07,12, HLA DP*02,04,11 HLA DQ*02,03,05,06, HLA DR*01,03,11,13 in Turkish and Syrian patients. High resolution HLA subgroups of the patients are shown in Tables three and four. CONCLUSION: In allogeneic stem cell transplantation, there may be similar HLA tissue types among ethnic groups.","PeriodicalId":53847,"journal":{"name":"Iraqi Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":0.1,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46956753","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: Acute lymphoblastic leukemia (ALL) is a heterogeneous disorder that is caused by the clonal expansion of immature lymphoid cells with a high rate among children more than adults. FMS-like tyrosine kinase 3 (FLT3) is a cellular receptor belongs to the Class III receptor tyrosine kinase family. The main expression of FLT3 on bone marrow (BM) cells especially CD34+ hematopoietic stem cells, early progenitor cells, dendritic progenitor cells, and other cells of organs (brain, placenta, and testis). Activation of FLT3 results in increased cell proliferation, decreased cell apoptosis, and inhibition of differentiation of cells. This study aims to detect the expression of the FLT3 cluster of differentiation antigen 135 (CD135) in childhood B-ALL patients. Moreover, to correlate this expression with hematological parameters include a complete blood count and BM examination findings and clinical parameters. PATIENTS, MATERIALS AND METHODS: This study was conducted on 30 newly diagnosed pediatric ALL patients. Diagnosis of the disease was based on the blood film, BM examination findings, cytochemistry, and flowcytometry of peripheral blood (PB) and/or BM sample, 1 ml of PB and/or BM sample was collected in EDTA tubes for flowcytometry for detection of CD135. RESULTS: This study found that male patients were more than females with a male-to-female ratio (1.14:1) and a median age of 5 years. Most of the patients had a positive expression of the FLT3 receptor and according to NCI risk groups, 60% of patients fall in the standard risk and 40% in the high-risk group. There was a significant correlation between the level of FLT3 (CD135) and age but no significant correlation with hemoglobin, white blood count, platelets, and peripheral or BM blast percentage. CONCLUSION: In this study, the patients with positive FLT3 blast cells (which is a bad prognostic factor) were associated with good prognostic factors. This proves that FLT3 is an independent prognostic factor.
{"title":"Relation between FMS-like tyrosine kinase 3 factor and hematological parameter in acute lymphoblastic leukemia patients by flow cytometry","authors":"Zainab Al-Ali, B. Mohammed","doi":"10.4103/ijh.ijh_49_22","DOIUrl":"https://doi.org/10.4103/ijh.ijh_49_22","url":null,"abstract":"BACKGROUND: Acute lymphoblastic leukemia (ALL) is a heterogeneous disorder that is caused by the clonal expansion of immature lymphoid cells with a high rate among children more than adults. FMS-like tyrosine kinase 3 (FLT3) is a cellular receptor belongs to the Class III receptor tyrosine kinase family. The main expression of FLT3 on bone marrow (BM) cells especially CD34+ hematopoietic stem cells, early progenitor cells, dendritic progenitor cells, and other cells of organs (brain, placenta, and testis). Activation of FLT3 results in increased cell proliferation, decreased cell apoptosis, and inhibition of differentiation of cells. This study aims to detect the expression of the FLT3 cluster of differentiation antigen 135 (CD135) in childhood B-ALL patients. Moreover, to correlate this expression with hematological parameters include a complete blood count and BM examination findings and clinical parameters. PATIENTS, MATERIALS AND METHODS: This study was conducted on 30 newly diagnosed pediatric ALL patients. Diagnosis of the disease was based on the blood film, BM examination findings, cytochemistry, and flowcytometry of peripheral blood (PB) and/or BM sample, 1 ml of PB and/or BM sample was collected in EDTA tubes for flowcytometry for detection of CD135. RESULTS: This study found that male patients were more than females with a male-to-female ratio (1.14:1) and a median age of 5 years. Most of the patients had a positive expression of the FLT3 receptor and according to NCI risk groups, 60% of patients fall in the standard risk and 40% in the high-risk group. There was a significant correlation between the level of FLT3 (CD135) and age but no significant correlation with hemoglobin, white blood count, platelets, and peripheral or BM blast percentage. CONCLUSION: In this study, the patients with positive FLT3 blast cells (which is a bad prognostic factor) were associated with good prognostic factors. This proves that FLT3 is an independent prognostic factor.","PeriodicalId":53847,"journal":{"name":"Iraqi Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":0.1,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45943715","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}
N. Al-Rahal, AhmedShemran Alwataify, S. Shawkat, Israa Almusawi, AzeezahMohammed Mohsin
BACKGROUND: Recombinant activated factor VII (FVII) is a product onetime evolved to stop bleeding occurring in hemophilia A and B patients with inhibitor, congenital FVII deficiency, Glanzmann disease, and in life-threatening bleeding. AIM: The aim was to evaluate the safety and efficacy of the coagulation factor VIIa, recombinant (AryoSeven RT) among congenital FVII deficiency patients at different centers in Iraq. METHODOLOGY: This is a prospective, observational, noninterventional study done at 5 medical centers in Iraq and it included 22 patients with FVII deficiency (congenital form) older than 14 years of age. Patients are recorded and followed for 6 months and they are subjected to AryoSeven RT depending on each patient individually. There were 3 main visits and 3 unscheduled visits for each patient during the study. Effectiveness evaluation was performed 6 h after each intervention. Adverse drug reactions related to the administration of AryoSeven RT were reported for each patient during each visit. RESULTS: A total of 22 participants were enrolled, classified into 18 (82%) were female and 4 (18%) were male. The mean age was 27.5 ± 14.0 years. Among 91 bleeding events, AryoSeven RT efficacy was effective in 89 events, excellent in 1 event, and partially effective in also 1 event. There was a reduction of PT from baseline (57.3 ± 15.2 s) to (13.9 ± 6.2 s) after 1st dose of AryoSeven RT and more reduction after 2nd dose of therapy (13.4 ± 4.4 s) and these were statistically significant (P = 0.001). Regarding FVII activity, there was a significant increase from baseline (8.4% ± 8.0%) to (95.8% ± 46.6%) after 1st dose and (131.8% ± 40.1%) after 2nd dose of AryoSeven RT with P = 0.001 for both. No major adverse events were reported except for headache in one participant (4.5%), and injection site reactions in three participants (13.6%).) CONCLUSION: AryoSeven RT is safe and effective clinically and by laboratory data in stopping bleeding in patients older than 14 years with inherited FVII deficiency.
{"title":"AryoSeven RT (Coagulation factor VIIa, recombinant) safety and efficacy study among congenial factor VII deficient patients in Iraq","authors":"N. Al-Rahal, AhmedShemran Alwataify, S. Shawkat, Israa Almusawi, AzeezahMohammed Mohsin","doi":"10.4103/ijh.ijh_50_22","DOIUrl":"https://doi.org/10.4103/ijh.ijh_50_22","url":null,"abstract":"BACKGROUND: Recombinant activated factor VII (FVII) is a product onetime evolved to stop bleeding occurring in hemophilia A and B patients with inhibitor, congenital FVII deficiency, Glanzmann disease, and in life-threatening bleeding. AIM: The aim was to evaluate the safety and efficacy of the coagulation factor VIIa, recombinant (AryoSeven RT) among congenital FVII deficiency patients at different centers in Iraq. METHODOLOGY: This is a prospective, observational, noninterventional study done at 5 medical centers in Iraq and it included 22 patients with FVII deficiency (congenital form) older than 14 years of age. Patients are recorded and followed for 6 months and they are subjected to AryoSeven RT depending on each patient individually. There were 3 main visits and 3 unscheduled visits for each patient during the study. Effectiveness evaluation was performed 6 h after each intervention. Adverse drug reactions related to the administration of AryoSeven RT were reported for each patient during each visit. RESULTS: A total of 22 participants were enrolled, classified into 18 (82%) were female and 4 (18%) were male. The mean age was 27.5 ± 14.0 years. Among 91 bleeding events, AryoSeven RT efficacy was effective in 89 events, excellent in 1 event, and partially effective in also 1 event. There was a reduction of PT from baseline (57.3 ± 15.2 s) to (13.9 ± 6.2 s) after 1st dose of AryoSeven RT and more reduction after 2nd dose of therapy (13.4 ± 4.4 s) and these were statistically significant (P = 0.001). Regarding FVII activity, there was a significant increase from baseline (8.4% ± 8.0%) to (95.8% ± 46.6%) after 1st dose and (131.8% ± 40.1%) after 2nd dose of AryoSeven RT with P = 0.001 for both. No major adverse events were reported except for headache in one participant (4.5%), and injection site reactions in three participants (13.6%).) CONCLUSION: AryoSeven RT is safe and effective clinically and by laboratory data in stopping bleeding in patients older than 14 years with inherited FVII deficiency.","PeriodicalId":53847,"journal":{"name":"Iraqi Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":0.1,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44791774","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: Chronic lymphocytic leukemia (CLL) is a malignancy of mature appearing clonal B lymphocytes where there is a progressive accumulation of leukemic cells in peripheral blood, bone marrow, and secondary lymphoid tissues as a consequence of defective apoptosis and survival signals derived from the microenvironment. The soluble CD23 (sCD23) is a 25 kDa fragment that can be found in serum, plasma, and urine in patients with CLL. It is a B-cell growth factor. B-cell maturation antigen (BCMA) is a member of the tumor necrosis factor superfamily, it enhances the survival and proliferation of mature B cells and plasma cells through signal transduction of the B-cell activating factor and a proliferation-inducing ligand. AIMS: The aims of this study were to assess the serum levels of sCD23 and BCMA in newly diagnosed CLL patients and to correlate them with clinical Binet staging and other hematological and clinical parameters. PATIENTS, MATERIALS AND METHODS: This study was conducted on 54 newly diagnosed CLL patients and 27 healthy controls. Diagnosis of CLL patients was based on lymphocyte count of >5 × 109/L and immunophenotyping. The serum levels of sCD23 and BCMA were measured in both groups using an enzyme-linked immunosorbent assay. RESULTS: Serum levels of sCD23 and BCMA were significantly higher in CLL patients in comparison with control group (P < 0.001 for both). There was a significant direct association between serum levels of sCD23 and BCMA with the clinical Binet stage of the disease (P < 0.001 for both). sCD23 showed significant correlation with hemoglobin (Hb) level (P < 0.001), total white blood cell (WBC) count (P = 0.001), lymphocyte count (P < 0.001), platelet count (P = 0.017), B-symptoms (P = 0.001), and splenomegaly (P = 0.019), whereas BCMA has significant correlations with Hb level, total WBC count, lymphocyte count (P < 0.001 for each one), B-symptoms (P < 0.001), lymphadenopathy (P = 0.001), splenomegaly (P = 0.024), and hepatomegaly (P = 0.04). CONCLUSIONS: The levels of serum sCD23 and serum BCMA increase with advancing Binet stages of the disease indicating their possible usefulness as good and reliable parameters for prognostic evaluation in CLL patients. The significant correlation of serum sCD23 and serum BCMA with hematological parameters and clinical features render them as reliable tumor burden markers in CLL patients.
{"title":"Clinical significance of serum sCD23 and B-cell maturation antigen levels in patients with chronic lymphocytic leukemia","authors":"T. Ameen, Haithem A Al-Rubaie","doi":"10.4103/ijh.ijh_31_22","DOIUrl":"https://doi.org/10.4103/ijh.ijh_31_22","url":null,"abstract":"BACKGROUND: Chronic lymphocytic leukemia (CLL) is a malignancy of mature appearing clonal B lymphocytes where there is a progressive accumulation of leukemic cells in peripheral blood, bone marrow, and secondary lymphoid tissues as a consequence of defective apoptosis and survival signals derived from the microenvironment. The soluble CD23 (sCD23) is a 25 kDa fragment that can be found in serum, plasma, and urine in patients with CLL. It is a B-cell growth factor. B-cell maturation antigen (BCMA) is a member of the tumor necrosis factor superfamily, it enhances the survival and proliferation of mature B cells and plasma cells through signal transduction of the B-cell activating factor and a proliferation-inducing ligand. AIMS: The aims of this study were to assess the serum levels of sCD23 and BCMA in newly diagnosed CLL patients and to correlate them with clinical Binet staging and other hematological and clinical parameters. PATIENTS, MATERIALS AND METHODS: This study was conducted on 54 newly diagnosed CLL patients and 27 healthy controls. Diagnosis of CLL patients was based on lymphocyte count of >5 × 109/L and immunophenotyping. The serum levels of sCD23 and BCMA were measured in both groups using an enzyme-linked immunosorbent assay. RESULTS: Serum levels of sCD23 and BCMA were significantly higher in CLL patients in comparison with control group (P < 0.001 for both). There was a significant direct association between serum levels of sCD23 and BCMA with the clinical Binet stage of the disease (P < 0.001 for both). sCD23 showed significant correlation with hemoglobin (Hb) level (P < 0.001), total white blood cell (WBC) count (P = 0.001), lymphocyte count (P < 0.001), platelet count (P = 0.017), B-symptoms (P = 0.001), and splenomegaly (P = 0.019), whereas BCMA has significant correlations with Hb level, total WBC count, lymphocyte count (P < 0.001 for each one), B-symptoms (P < 0.001), lymphadenopathy (P = 0.001), splenomegaly (P = 0.024), and hepatomegaly (P = 0.04). CONCLUSIONS: The levels of serum sCD23 and serum BCMA increase with advancing Binet stages of the disease indicating their possible usefulness as good and reliable parameters for prognostic evaluation in CLL patients. The significant correlation of serum sCD23 and serum BCMA with hematological parameters and clinical features render them as reliable tumor burden markers in CLL patients.","PeriodicalId":53847,"journal":{"name":"Iraqi Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":0.1,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43638463","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}
Hemoglobin H (HbH) disease is alpha (α)-thalassemia characterized by the inactivation of three of four α-globin genes due to deletions with or without nondeletional α-thalassemia. HbH disease is not necessarily a benign disorder as has been generally thought. Here, we report a 33-year-old female who has a lifelong history of anemia without blood transfusions. but when she got married, during pregnancies developed symptomatic moderate-to-severe anemia necessitating infrequent blood transfusions. Later due to symptomatic anemias and increasing the frequency of blood transfusions, she underwent splenectomy, 3 years from splenectomy she developed a gradual dark skin discoloration with frequent hypotension and was diagnosed with Addison's disease which is a rare endocrine complication of thalassemia-induced iron overload.
{"title":"Addison's disease in a lady with hemoglobin H disease","authors":"N. Rashid","doi":"10.4103/ijh.ijh_42_22","DOIUrl":"https://doi.org/10.4103/ijh.ijh_42_22","url":null,"abstract":"Hemoglobin H (HbH) disease is alpha (α)-thalassemia characterized by the inactivation of three of four α-globin genes due to deletions with or without nondeletional α-thalassemia. HbH disease is not necessarily a benign disorder as has been generally thought. Here, we report a 33-year-old female who has a lifelong history of anemia without blood transfusions. but when she got married, during pregnancies developed symptomatic moderate-to-severe anemia necessitating infrequent blood transfusions. Later due to symptomatic anemias and increasing the frequency of blood transfusions, she underwent splenectomy, 3 years from splenectomy she developed a gradual dark skin discoloration with frequent hypotension and was diagnosed with Addison's disease which is a rare endocrine complication of thalassemia-induced iron overload.","PeriodicalId":53847,"journal":{"name":"Iraqi Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":0.1,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41972548","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}
Central nervous system involvement in multiple myeloma (MM) is rare and carries a very poor prognosis. We report the case of a 62-year-old man diagnosed with MM who received induction chemotherapy with bortezomib, cyclophosphamide, and dexamethasone and achieved very good partial response. He developed seizures and altered sensorium while on maintenance chemotherapy. Magnetic resonance imaging of the brain showed leptomeningeal enhancement, and cerebrospinal fluid cytology was positive for plasma cells. His general condition worsened, and he died before starting any specific treatment.
{"title":"Central nervous system relapse in multiple myeloma: An unusual complication","authors":"A. Vasudevan, G. Narayanan, N. Nayak","doi":"10.4103/ijh.ijh_10_21","DOIUrl":"https://doi.org/10.4103/ijh.ijh_10_21","url":null,"abstract":"Central nervous system involvement in multiple myeloma (MM) is rare and carries a very poor prognosis. We report the case of a 62-year-old man diagnosed with MM who received induction chemotherapy with bortezomib, cyclophosphamide, and dexamethasone and achieved very good partial response. He developed seizures and altered sensorium while on maintenance chemotherapy. Magnetic resonance imaging of the brain showed leptomeningeal enhancement, and cerebrospinal fluid cytology was positive for plasma cells. His general condition worsened, and he died before starting any specific treatment.","PeriodicalId":53847,"journal":{"name":"Iraqi Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":0.1,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48217550","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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