AIM： TO describe the socio-demographic features, etiology, and risk factors for Budd-Chiari syndrome （BCS） in Egyptian patients. METHODS： Ninety-four Egyptian patients with confirmed primary Budd-Chiari syndrome w...AIM： TO describe the socio-demographic features, etiology, and risk factors for Budd-Chiari syndrome （BCS） in Egyptian patients. METHODS： Ninety-four Egyptian patients with confirmed primary Budd-Chiari syndrome were presented to the Budd-Chiari Study Group （BCSG） and admitted to the Tropical Medicine Department of Ain Shams University Hospital （Cairo, Egypt）. Complete clinical evaluation and laboratory investigations, including a thrombophilia workup and full radiological assessment, were performed to determine underlying disease etiologies.RESULTS： BCS was chronic in 79.8% of patients, acute or subacute in 19.1%, and fulminant in 1.1%. Factor V Leiden mutation （FVLM） was the most common etiological cause of disease （53.1%）, followed by mutation of the gene encoding methylene tetrahydrofolate reductase （MTHFR） （51.6%）. Current or recent hormonal treatment was documented in 15.5% of females, and BCS associated with pregnancy was present in 17.2% of females. Etiology could not be determined in 8.5% of patients. Males had significantly higher rates of MTHFR gene mutation and Behcet＇ s disease, and females had significantly higher rates of secondary antiphospholipid antibody syndrome. A highly significant positive relationship was evident between the presence of Behcet＇s disease and inferior vena caval occlusion, either alone or combined with occlusion of the hepatic veins （,0 〈 0.0001）. CONCLUSION： FVLM is the most common disease etiology and MTHFR the second most common in Egyptian BCS patients. BCS etiology tends to vary with geographic region.展开更多
Gastrointestinal (GI) bleeding refers to any hemorrhage ascribed to the pathologies of the gastrointestinal tract,extending from the mouth to the anal canal.Despite the recent improvements in the endoscopic,hemostatic...Gastrointestinal (GI) bleeding refers to any hemorrhage ascribed to the pathologies of the gastrointestinal tract,extending from the mouth to the anal canal.Despite the recent improvements in the endoscopic,hemostatic and adjuvant pharmacologic techniques,the reported mortality is still around 5%-10% for peptic ulcer bleeding and about 15%-20% for variceal hemorrhages.Although endoscopic management reduces the rates of re-bleeding,surgery,and mortality in active bleeding;early recurrence ratios still occur in around 20% of the cases even with effective initial hemostatic measures.In this quest for an alternative pro-hemostatic agent for the management of GI bleedings,Ankaferd blood stopper (ABS) offers a successful candidate,specifically for "difficult-to-manage" situations as evidenced by data presented in several studies.ABS is a standardized mixture of the plants Thymus vulgaris,Glycyrrhiza glabra,Vitis vinifera,Alpinia officinarum,and Urtica dioica.It is effective in both bleeding individuals with normal hemostatic parameters and in patients with deficient primary and/or secondary hemostasis.ABS also modulates the cellular apoptotic responses to hemorrhagic stress,as well as hemostatic hemodynamic activity.Through its effects on the endothelium,blood cells,angiogenesis,cellular proliferation,vascular dynamics,and wound healing,ABS is now becoming an effective alternative hemostatic medicine for gastrointestinal bleedings that are resistant to conventional anti-hemorrhagic measurements.The aim of this review is to outline current literature experience suggesting the place of ABS in the management of GI bleeding,and potential future controlled trials in this complicated field.展开更多
Hemorrhagic cystitis is a common and in its severe form potentially life threatening complication of hematopoietic stem cell transplantation. Hemorrhagic cystitis is defined as a diffuse inflammatory condition of the ...Hemorrhagic cystitis is a common and in its severe form potentially life threatening complication of hematopoietic stem cell transplantation. Hemorrhagic cystitis is defined as a diffuse inflammatory condition of the urinary bladder due to an infectious or noninfectious etiology resulting in bleeding from the bladder mucosa. Hemorrhagic cystitis is characterized by lower urinary tract symptoms including dysuria, hematuria and hemorrhage. The most common cause is a bacterial infection that usually responds promptly to treatment. But chronic and recurrent hemorrhagic cystitis often arises from anticancer chemotherapy or radiotherapy for the treatment of pelvic malignancies. Infectious etiologies are less common causes of chronic hemorrhagic cystitis except in immunocompromised hosts like bone marrow transplant recipients. Hemorrhagic cystitis is a significant complication of bone marrow transplantation which influences economic and survival outcome. Hemorrhagic cystitis can be divided into two classes according to onset time;early and late onset time. Earlyonset hemorrhagic cystitis is commonly associated used with chemo-radiotherapy protocols in some of the preparatory regimens. More than one factor is accused in the etiology of late onset hemorrhagic cystitis. Here, we present a patient whose hematuria started after 54 days from allogeneic stem cell transplantation.展开更多
The chronic myeloproliferative neoplasms (CMPN) are a group of clonal hematopoietic stem cell disorders in which large numbers of red blood cells, white blood cells, or platelets grow and spread excess in the bone mar...The chronic myeloproliferative neoplasms (CMPN) are a group of clonal hematopoietic stem cell disorders in which large numbers of red blood cells, white blood cells, or platelets grow and spread excess in the bone marrow and the pe- ripheral blood. Cytogenetic analysis of the t (9:22) and molecular detection of BCR/ABL is the main diagnostic criteria in Philadelphia positive CMPN (CML). The identification of non-receptor tyrosine kinase JAK2 mutations (exon 14 JAK2 V617F and exon 12) have significantly contributed to our understanding of the molecular mechanisms in the pathogenesis of Philadelphia negative CMPN such as polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) patients. According to the revised WHO classification, JAK2 mutation is considered as a major diagnostic and clonal marker in Philadelphia negative CMPN which will play a major role in designing personal- ized treatments for the disease. JAK2 V617F mutation frequency is unknown in Saudi Arabia. Therefore, investigation of the JAK2 V617F mutation was carried out in DNA samples from 78 peripheral blood specimens corresponding to patients with polycythemia vera (PV) (n = 11), Chronic myeloid leukemia (CML) (n = 45), essential thrombocythemia (ET) (n = 10), idiopathic myelofibrosis (MF) (n = 12). We used polymerase chain reaction and direct DNA sequencing to detect the JAK2 mutation. Overall, the incidence of the JAK2 V617F mutation was 91% in PV, 40% in ET, and 25% in MF. This approach proved to be reliable and more sensitive in detecting the mutation. Two essential findings arose from our study. First, this technique could be carried out with DNA samples, even partially degraded, from routinely processed BM or peripheral blood specimens. Second, after correlation with morphological features, it turned out that the characteristics of the megakaryocytes were more specific than the mutational status of JAK2 in characterizing ET and PMF. Concerning PV, as expected, the incidence of the JAK2 mutation was higher, but the morphological criteria were misleading in some cases, strongly suggesting that the combination of both morphology and molecular data would enable the characterization of virtually all cases. JAK2 V617F mutation frequency along with accurate morphological characterization is very reliable tool in diagnosing and classifying CMPN in Saudi patients.展开更多
Objective: To explore whether its antiplasmodium effect of andrographolide is attributed to its plausible effect on the plasma membrane of both Plasmodium falciparum infected and noninfected RBCs. Methods: Anti-plasmo...Objective: To explore whether its antiplasmodium effect of andrographolide is attributed to its plausible effect on the plasma membrane of both Plasmodium falciparum infected and noninfected RBCs. Methods: Anti-plasmodium effect of andrographolide against Plasmodium falciparum strains was screened using the conventional malaria drug sensitivity assay. The drug was incubated with uninfected RBCs to monitor its effect on their morphology, integrity and osmotic fragility. It was incubated with the plasmodium infected RBCs to monitor its effect on the parasite induced permeation pathways. Its effect on the potential of merozoites to invade new RBCs was tested using merozoite invasion assay. Results: It showed that at andrographolide was innocuous to RBCs at concentrations approach its therapeutic level against plasmodia. Nevertheless, this inertness was dwindled at higher concentrations. Conclusions: In spite of its success to inhibit plasmodium induced permeation pathway and the potential of merozoites to invade new RBCs, its anti-plasmodium effect can't be attributed to these functions as they were attained at concentrations higher than what is required to eradicate the parasite. Consequently, other mechanisms may be associated with its claimed actions.展开更多
Multiple primary cancers （MPC） are specific .malignant tumors type, manifesting as more than one primary tumor diagnosed in the same patient, either simultaneously or sequentially. The diagnostic criteria include： ...Multiple primary cancers （MPC） are specific .malignant tumors type, manifesting as more than one primary tumor diagnosed in the same patient, either simultaneously or sequentially. The diagnostic criteria include： the cancer must be clearly malignant as determined by histological evaluation; each cancer must be geographically separate and distinct;展开更多
Background Brucellosis can mimic various multisytem diseases, showing wide clinical polymorphism that frequently leads to misdiagnosis and treatment delay, further increasing the complication rates. In this study, we ...Background Brucellosis can mimic various multisytem diseases, showing wide clinical polymorphism that frequently leads to misdiagnosis and treatment delay, further increasing the complication rates. In this study, we aimed to examine bone marrow biopsy findings in brucellosis cases presenting with hematologic abnormalities. Methods Forty-eight brucellosis cases were prospectively investigated. Complaints and physical examination findings of patients were recorded. Patients＇ complete blood count, routine biochemical tests, erythrocyte sedimentation rate, C-reactive protein and serological screenings were performed. Bone marrow biopsy and aspiration was performed in patients with cytopenia, for bone marrow examination and brucella culture, in accordance with the standard procedures from spina iliaca posterior superior region of pelvic bone. Results Of the 48 patients, 35 （73%） were female and 13 （27%） were male. Mean age was （34.8±15.4） years （age range： 15-70 years）. Anemia, leukopenia, thrombocytopenia and pancytopenia were found in 39 （81%）, 28 （58%）, 22 （46%） and 10 patients （21%）, respectively. In the examination of bone marrow, hypercellularity was found in 35 （73%） patients. Increased megacariocytic, erythroid and granulocytic series were found in 28 （58%）, 15 （31%） and 5 （10%） patients, respectively. In addition, hemophagocytosis was observed in 15 （31%） patients, granuloma observed in 12 （25%） and increased eosinophil and plasma cells observed in 9 （19%） patients. Conclusion According to the results of our series, hemophagocytosis, microgranuloma formation and hypersplenism may be responsible for hematologic complications of brucellosis.展开更多
文摘AIM： TO describe the socio-demographic features, etiology, and risk factors for Budd-Chiari syndrome （BCS） in Egyptian patients. METHODS： Ninety-four Egyptian patients with confirmed primary Budd-Chiari syndrome were presented to the Budd-Chiari Study Group （BCSG） and admitted to the Tropical Medicine Department of Ain Shams University Hospital （Cairo, Egypt）. Complete clinical evaluation and laboratory investigations, including a thrombophilia workup and full radiological assessment, were performed to determine underlying disease etiologies.RESULTS： BCS was chronic in 79.8% of patients, acute or subacute in 19.1%, and fulminant in 1.1%. Factor V Leiden mutation （FVLM） was the most common etiological cause of disease （53.1%）, followed by mutation of the gene encoding methylene tetrahydrofolate reductase （MTHFR） （51.6%）. Current or recent hormonal treatment was documented in 15.5% of females, and BCS associated with pregnancy was present in 17.2% of females. Etiology could not be determined in 8.5% of patients. Males had significantly higher rates of MTHFR gene mutation and Behcet＇ s disease, and females had significantly higher rates of secondary antiphospholipid antibody syndrome. A highly significant positive relationship was evident between the presence of Behcet＇s disease and inferior vena caval occlusion, either alone or combined with occlusion of the hepatic veins （,0 〈 0.0001）. CONCLUSION： FVLM is the most common disease etiology and MTHFR the second most common in Egyptian BCS patients. BCS etiology tends to vary with geographic region.
文摘Gastrointestinal (GI) bleeding refers to any hemorrhage ascribed to the pathologies of the gastrointestinal tract,extending from the mouth to the anal canal.Despite the recent improvements in the endoscopic,hemostatic and adjuvant pharmacologic techniques,the reported mortality is still around 5%-10% for peptic ulcer bleeding and about 15%-20% for variceal hemorrhages.Although endoscopic management reduces the rates of re-bleeding,surgery,and mortality in active bleeding;early recurrence ratios still occur in around 20% of the cases even with effective initial hemostatic measures.In this quest for an alternative pro-hemostatic agent for the management of GI bleedings,Ankaferd blood stopper (ABS) offers a successful candidate,specifically for "difficult-to-manage" situations as evidenced by data presented in several studies.ABS is a standardized mixture of the plants Thymus vulgaris,Glycyrrhiza glabra,Vitis vinifera,Alpinia officinarum,and Urtica dioica.It is effective in both bleeding individuals with normal hemostatic parameters and in patients with deficient primary and/or secondary hemostasis.ABS also modulates the cellular apoptotic responses to hemorrhagic stress,as well as hemostatic hemodynamic activity.Through its effects on the endothelium,blood cells,angiogenesis,cellular proliferation,vascular dynamics,and wound healing,ABS is now becoming an effective alternative hemostatic medicine for gastrointestinal bleedings that are resistant to conventional anti-hemorrhagic measurements.The aim of this review is to outline current literature experience suggesting the place of ABS in the management of GI bleeding,and potential future controlled trials in this complicated field.
文摘Hemorrhagic cystitis is a common and in its severe form potentially life threatening complication of hematopoietic stem cell transplantation. Hemorrhagic cystitis is defined as a diffuse inflammatory condition of the urinary bladder due to an infectious or noninfectious etiology resulting in bleeding from the bladder mucosa. Hemorrhagic cystitis is characterized by lower urinary tract symptoms including dysuria, hematuria and hemorrhage. The most common cause is a bacterial infection that usually responds promptly to treatment. But chronic and recurrent hemorrhagic cystitis often arises from anticancer chemotherapy or radiotherapy for the treatment of pelvic malignancies. Infectious etiologies are less common causes of chronic hemorrhagic cystitis except in immunocompromised hosts like bone marrow transplant recipients. Hemorrhagic cystitis is a significant complication of bone marrow transplantation which influences economic and survival outcome. Hemorrhagic cystitis can be divided into two classes according to onset time;early and late onset time. Earlyonset hemorrhagic cystitis is commonly associated used with chemo-radiotherapy protocols in some of the preparatory regimens. More than one factor is accused in the etiology of late onset hemorrhagic cystitis. Here, we present a patient whose hematuria started after 54 days from allogeneic stem cell transplantation.
文摘The chronic myeloproliferative neoplasms (CMPN) are a group of clonal hematopoietic stem cell disorders in which large numbers of red blood cells, white blood cells, or platelets grow and spread excess in the bone marrow and the pe- ripheral blood. Cytogenetic analysis of the t (9:22) and molecular detection of BCR/ABL is the main diagnostic criteria in Philadelphia positive CMPN (CML). The identification of non-receptor tyrosine kinase JAK2 mutations (exon 14 JAK2 V617F and exon 12) have significantly contributed to our understanding of the molecular mechanisms in the pathogenesis of Philadelphia negative CMPN such as polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) patients. According to the revised WHO classification, JAK2 mutation is considered as a major diagnostic and clonal marker in Philadelphia negative CMPN which will play a major role in designing personal- ized treatments for the disease. JAK2 V617F mutation frequency is unknown in Saudi Arabia. Therefore, investigation of the JAK2 V617F mutation was carried out in DNA samples from 78 peripheral blood specimens corresponding to patients with polycythemia vera (PV) (n = 11), Chronic myeloid leukemia (CML) (n = 45), essential thrombocythemia (ET) (n = 10), idiopathic myelofibrosis (MF) (n = 12). We used polymerase chain reaction and direct DNA sequencing to detect the JAK2 mutation. Overall, the incidence of the JAK2 V617F mutation was 91% in PV, 40% in ET, and 25% in MF. This approach proved to be reliable and more sensitive in detecting the mutation. Two essential findings arose from our study. First, this technique could be carried out with DNA samples, even partially degraded, from routinely processed BM or peripheral blood specimens. Second, after correlation with morphological features, it turned out that the characteristics of the megakaryocytes were more specific than the mutational status of JAK2 in characterizing ET and PMF. Concerning PV, as expected, the incidence of the JAK2 mutation was higher, but the morphological criteria were misleading in some cases, strongly suggesting that the combination of both morphology and molecular data would enable the characterization of virtually all cases. JAK2 V617F mutation frequency along with accurate morphological characterization is very reliable tool in diagnosing and classifying CMPN in Saudi patients.
文摘Objective: To explore whether its antiplasmodium effect of andrographolide is attributed to its plausible effect on the plasma membrane of both Plasmodium falciparum infected and noninfected RBCs. Methods: Anti-plasmodium effect of andrographolide against Plasmodium falciparum strains was screened using the conventional malaria drug sensitivity assay. The drug was incubated with uninfected RBCs to monitor its effect on their morphology, integrity and osmotic fragility. It was incubated with the plasmodium infected RBCs to monitor its effect on the parasite induced permeation pathways. Its effect on the potential of merozoites to invade new RBCs was tested using merozoite invasion assay. Results: It showed that at andrographolide was innocuous to RBCs at concentrations approach its therapeutic level against plasmodia. Nevertheless, this inertness was dwindled at higher concentrations. Conclusions: In spite of its success to inhibit plasmodium induced permeation pathway and the potential of merozoites to invade new RBCs, its anti-plasmodium effect can't be attributed to these functions as they were attained at concentrations higher than what is required to eradicate the parasite. Consequently, other mechanisms may be associated with its claimed actions.
文摘Multiple primary cancers （MPC） are specific .malignant tumors type, manifesting as more than one primary tumor diagnosed in the same patient, either simultaneously or sequentially. The diagnostic criteria include： the cancer must be clearly malignant as determined by histological evaluation; each cancer must be geographically separate and distinct;
文摘Background Brucellosis can mimic various multisytem diseases, showing wide clinical polymorphism that frequently leads to misdiagnosis and treatment delay, further increasing the complication rates. In this study, we aimed to examine bone marrow biopsy findings in brucellosis cases presenting with hematologic abnormalities. Methods Forty-eight brucellosis cases were prospectively investigated. Complaints and physical examination findings of patients were recorded. Patients＇ complete blood count, routine biochemical tests, erythrocyte sedimentation rate, C-reactive protein and serological screenings were performed. Bone marrow biopsy and aspiration was performed in patients with cytopenia, for bone marrow examination and brucella culture, in accordance with the standard procedures from spina iliaca posterior superior region of pelvic bone. Results Of the 48 patients, 35 （73%） were female and 13 （27%） were male. Mean age was （34.8±15.4） years （age range： 15-70 years）. Anemia, leukopenia, thrombocytopenia and pancytopenia were found in 39 （81%）, 28 （58%）, 22 （46%） and 10 patients （21%）, respectively. In the examination of bone marrow, hypercellularity was found in 35 （73%） patients. Increased megacariocytic, erythroid and granulocytic series were found in 28 （58%）, 15 （31%） and 5 （10%） patients, respectively. In addition, hemophagocytosis was observed in 15 （31%） patients, granuloma observed in 12 （25%） and increased eosinophil and plasma cells observed in 9 （19%） patients. Conclusion According to the results of our series, hemophagocytosis, microgranuloma formation and hypersplenism may be responsible for hematologic complications of brucellosis.
