Background: Primary idiopathic myelofibrosis (PMF) is a clonal Philadelphia chromosome-negative myeloproliferative neoplasm characterized by extramedullary hematopoiesis and marrow fibrosis. It is an uncommon hematopoietic malignancy which primarily affects elderly individuals. The rational of this study was to determine its clinico-epidemiological profile along with risk stratification in Pakistani patients. Materials and Methods: In this retrospective cross sectional study, 20 patients with idiopathic myelofibrosis were enrolled from January 2011 to December 2014. Data were analyzed with SPSS version 22. Results: The mean age was $57.9{\pm}16.5years$ with 70% of patients aged above 50. The male to female ratio was 3:1. Overall only 10% of patients were asymptomatic and the remainder presented with constitutional symptoms. In symptomatic patients, major complaints were weakness (80%), weight loss (75%), abdominal discomfort (60%), night sweats (13%), pruritus (5%) and cardiovascular accidents (5%). Physical examination revealed splenomegaly as a predominant finding detected in 17 patients (85%) with the mean splenic span of $22.2{\pm}2.04cm$. The mean hemoglobin was $9.16{\pm}2.52g/dl$ with the mean MCV of $88.2{\pm}19.7fl$. The total leukocyte count of $17.6{\pm}19.2{\times}10^9/l$ and platelets count were $346.5{\pm}321.9{\times}10^9/l$. Serum lactate dehydrogenase, serum creatinine and uric acid were $731.0{\pm}154.1$, $0.82{\pm}0.22$ and $4.76{\pm}1.33$ respectively. According to risk stratification, 35% were in high risk, 40% in intermediate risk and 25% in low risk groups. Conclusions: The majority of PMF patients were male and presented with constitutional symptoms in our setting. Risk stratification revealed predominance of advanced disease in our series.
Background: Hyaluronan (HA) is an unbranched glycosaminoglycan. It has been proposed that HA acts as a vehicle for cytokines due to the strong negative charge on its surface. We hypothesized that HA would function like a cytokine scavenger and reduce the inflammatory signaling cascade and this would lead to improved survival in rats suffering with endotoxemia. Methods: Endotoxin (Salmonella, 10 mg/kg) or an equal amount of 0.9% NaCl (NS) was injected into the jugular vein of rats. HA (1,600 kDa, 0.35%) or NS was given at 0.1 mL/kg/h for 3 hours. HA or NS infusion was started at 4 hour after endotoxin injection. The rats were divided into the control and HA groups (n=16 for each group). The mean arterial pressure (MAP) was monitored during HA or normal saline infusion. Survival was assessed every 12 hours for 3 days throughout the experiment. Results: The survival rate (%) of the rats treated with HA was higher (60%) than that of the controls (20%) when HA was infused 4 hours after lipopolysaccharide (LPS) injection. The bronchoalveolar lavage (BAL) fluid of the animals surviving HA or NS infusion 4 hours after LPS showed that the total cell counts and number of neutrophils were significantly (p < 0.01) reduced in the HA treated groups compared with that of the controls (total cell count, $9.2{\times}10^4$/mL vs. $61{\times}10^4$/mL; neutrophils, $21{\times}10^4$/mL vs. $0.2{\times}10^4$/mL, respectively). There was no significant MAP difference between the HA or control groups either with or without endotoxin. Conclusion: Infusion of hyaluronan (1,600 kDa) reduced the BAL total cell count and the number of neutrophils and it improved the survival rate of the endotoxemic rats.
Background: Polycythaemia rubra vera (PV) is a Philadelphia chromosome negative myeloproliferative neoplasm characterized by increased red cell production, independent of the mechanisms that regulate normal erythropoiesis. The aim of this study was to analyze the clinico-epidemiological profile of Pakistani patients with PV. Materials and Methods: In this retrospective cross sectional study, 26 patients with PV were enrolled from January 2010 to December 2014. They were diagnosed based on WHO criteria. Results: The mean age was $53.4{\pm}9.31years$ (range 36-72) and the male to female ratio was 2:1. Overall 30.7% of patients were asymptomatic. In symptomatic patients, major complaints were headache (30.8%), abdominal discomfort (23.1%), blurred vision (15.3%), pruritus (11.5%) and vascular incidents (11.5%). Physical examination revealed plethoric face and splenomegaly as predominant findings, detected in 34.6% and 30.7%, respectively, with the mean splenic span of $15.9{\pm}2.04cm$. The mean hemoglobin was $18.1{\pm}1.9g/dl$ with the mean hematocrit of $55.6{\pm}8.3%$. The mean total leukocyte count was $12.8{\pm}7.1{\times}10^9/l$ and the platelet count $511{\pm}341.9{\times}10^9/l$. Mean erythrocyte sedimentation rate was $3.5{\pm}1.22mm/hr$. Serum lactate dehydrogenase, serum creatinine and uric acid were $552.7{\pm}309.2$, $0.8{\pm}0.17$ and $6.60{\pm}1.89$ respectively. Conclusions: PV in Pakistani patients, unlike in the West, is seen in a moderately young population. The disease is frequently seen in male gender and primarily patients present with symptoms related to hyperviscosity.
Background: Acute promyelocytic leukemia (APL) is a distinctive clinical, biological and molecular subtype of acute myeloid leukemia. However, data from Pakistan are scarce. Therefore we reviewed the demographic and clinical profile along with risk stratification of APL patients at our center. Materials and Methods: In this descriptive cross sectional study, 26 patients with acute promyelocytic leukemia were enrolled from January 2011 to June 2015. Data were analyzed with SPSS version 22. Results: The mean age was $31.8{\pm}1.68years$ with a median of 32 years. The female to male ratio was 2:1.2. The majority of our patients had hypergranular variant (65.4%) rather than the microgranular type. The major complaints were bleeding (80.7%), fever (76.9%), generalized weakness (30.7%) and dyspnea (15.38%). Physical examination revealed petechial rashes as a predominant finding detected in 61.5% followed by pallor in 30.8%. The mean hemoglobin was $8.04{\pm}2.29g/dl$ with the mean MCV of $84.7{\pm}7.72fl$. The mean total leukocyte count of $5.44{\pm}7.62{\times}10^9/l$; ANC of $1.08{\pm}2.98{\times}10^9/l$ and mean platelets count were $38.84{\pm}5.38{\times}10^9/l$. According to risk stratification, 15.3% were in high, 65.4% in intermediate and 19.2% in low risk groups. Conclusions: Clinico-epidemiological features of APL in Pakistani patients appear comparable to published data. Haemorrhagic diathesis is the commonest presentation. Risk stratification revealed predominance of intermediate risk disease.
Background: Non-conversion of sputum smear and culture prolongs the infectivity of the patient and has been associated with unfavorable outcomes. We aimed to evaluate factors associated with persistent sputum positivity at the end of two months of treatment of new case pulmonary tuberculosis (TB). Methods: Data of 87 human immunodeficiency virus-negative patients with culture-positive drug-susceptible pulmonary TB admitted to local university hospital between September 2015 and September 2016 were reviewed. Factors associated with sputum smear and/or culture positivity at the end of the second month of treatment were analyzed. Results: Twenty-two patients (25.3%) remained smear and/or culture-positive. Male sex, lower body mass index (BMI), unemployment, alcohol abuse, higher number of lobes involved and cavities on chest X-rays, shorter time to detection (TTD) on liquid cultures, higher respiratory sample smear grading and colony count in solid cultures, higher C-reactive protein, erythrocyte sedimentation rate, leukocytosis, thrombocytosis, and anemia were all significantly associated with persistent sputum positivity. However, in the logistic regression analysis only male sex, lower BMI, alcohol abuse, higher radiological involvement, cavitation, higher smear grading, higher colony count in solid cultures and shorter TTD were determined as independent factors associated with persistent sputum positivity at the end of 2 months of treatment. Conclusion: In conclusion, higher sputum smear and culture grading at diagnosis, shorter TTD, higher number of lobes involved, cavitation, male sex, alcohol abuse, and lower BMI were independently associated with persistent sputum positivity. These factors should be sought when distinguishing which patients will remain infectious longer and possibly have worse outcomes.
Sultan, Sadia;Zaheer, Hasan Abbas;Irfan, Syed Mohammed;Ashar, Sana
Asian Pacific Journal of Cancer Prevention
/
v.17
no.1
/
pp.357-360
/
2016
Background: Acute myeloid leukemia (AML) is an acquired clonal frequent malignant disorder of myeloid progenitor cells. Our aim was to study demographical and clinicopathological features of adult Pakistani AML patients at presentation. Materials and Methods: In this single centre study extending from January 2010 to December 2014, data were retrieved from the patient records with a predetermined performa and analyzed with SPSS version 22. Results: Overall 125 patients were diagnosed at our institution with de novo AML during the study period. There were 76 males and 49 females (ratio 1.5:1), with an age range between 15 and 85 years and a mean age of $38.8{\pm}20.1years$. The major complaints were fever (72.8%), generalized weakness (60%), bleeding (37.6%) and dyspnea (12%). Physical examination revealed pallor in 56.8%, splenomegaly and hepatomegaly in 16% and 12.8%, respectively, and lymphodenopathy in 10.4%. The mean hemoglobin was $8.19{\pm}2.12g/dl$ with a mean MCV of $86.0{\pm}9.83fl$, a mean total leukocyte count of $43.1{\pm}68.5{\times}10^9/l$, an ANC of $3.09{\pm}6.66{\times}10^9/l$ and a mean platelet count of $62.3{\pm}78.6{\times}10^9/l$. Conclusions: AML in Pakistani patients is seen in a relatively very young population with male preponderance, compared with the west. However, clinico-pathological features appear comparable to published data.
Mozaheb, Zahra;NazarAbadi, Mohamad Hasan Hasanzadeh;Aghaee, Monavar Afzal
Asian Pacific Journal of Cancer Prevention
/
v.13
no.7
/
pp.3009-3013
/
2012
Background: The clinical course of individual chronic lymphocytic leukemia (CLL) is highly variable and clinical staging systems do not help us to predict if and at what rate there will be disease progression in an individual patient diagnosed with early stage disease. Recently, several important observations related to other prognostic factors including lymphocyte doubling time (LDT), ${\beta}_2$-microglobulin (${\beta}_2$-MG), and percent of smudge cell in peripheral blood smears, cytogenetic and molecular analysis have been made. The aim of this study was to evaluate a range of prognostic factors in our CLL patients. Design and methods: Seventy patients with CLL were enrolled. Prognostic factors of disease including Binet staging, LDT, ${\beta}_2$-MG, ESR, LDH, percent of smudge cell in peripheral blood smear, absolute lymphocyte count, and conventional cytogenetic (CC) analysis were evaluated at diagnosis, and the patients were followed up to determine their outcome. We compared factors with each other and with Binet staging and prognosis. Results: Enrolled patients aged 37-85 years at diagnosis or during follow up. There was no relationship between serum LDH level (P=0.3), ESR (P=0.11), percent of smudge cells in peripheral blood smear (P=0.94), and absolute lymphocyte count (P=0.18) with the stage of disease and prognosis, but the ${\beta}_2$ macroglobulin level (p<0.0001), LDT (p<0.001) had direct and significant relation with staging and outcome. In 19% of patients cytogenetic alteration were seen. Conclusion: The detection of cytogenetic alteration only using the CC method is not sufficient and we need to use FISH, but because FISH study is an expensive method not available in all areas, instead we believe that ${\beta}_2$ MG can be applied in its place as a good prognostic factor for CLL at diagnosis and during follow up. We suggest to add it to Binet staging for prognostic subgrouping of CLL.
Background: The prognostic significance of the circulating absolute monocyte count (AMC) in patients with locally advanced hepatocellular carcinoma (HCC) is uncertain. This study was designed to assess the association of circulating AMC with survival outcomes in patients diagnosed with locally advanced or metastatic HCC receiving systemic chemotherapy. Materials and Methods: Between January 1, 2005 and December 30, 2012, locally advanced or metastatic HCC patients who had Child-Pugh stage A or B disease and received systemic chemotherapy were retrospectively enrolled. Patient features including gender, age, extrahepatic metastasis, Child-Pugh stage, serum alpha-fetoprotein(AFP) level and AMC were collected to investigate their prognostic impact on overall survival(OS). Results: A total of 216 patients were eligible for the study. The optimal cut-off value of AMC for OS analysis was $0.38{\times}10^9/L$. Median OS was 5.84 months in low-AMC group (95% confidence interval [CI], 5.23 to 6.45), and 5.21 months in high-AMC group (95% CI, 4.37 to 6.04; p=0.003). In COX multivariate analysis, elevated AMC remained as an independent prognostic factor for worse OS (HR, 1.578; 95% CI, 1.120 to 2.223, p=0.009). Conclusions: Our results indiicate that circulating AMC is confirmed to be an independent prognostic factor for OS in patients with locally advanced or metastatic HCC receiving systemic chemotherapy.
Background: Hodgkin's lymphoma (formerly, Hodgkins disease) is a potentially curable malignancy with distinctive biological behavior and specific clinical characteristics. Limited information is available from developing countries for patients with classical Hodgkin's lymphoma (cHL). Therefore we reviewed the demographical and clinico-hematological profiles along with bone marrow infiltration patterns in adult patients presenting at Liaquat National Hospital and Medical College. Materials and Methods: In this cross sectional study, 62 adult (${\geq}15years$) patients with cHL were enrolled from January 2010 to December 2014. Results: The mean age was $29.7{\pm}13.8years$ with a median of 30 years. The male to female ratio was 2:1. B symptoms were present in 72.5% of patients and lymph node enlargement in 85.4%. The frequency of bone marrow infiltration in our cHL patients was found to be 27.4%, the pattern being predominantly focal followed by diffuse. The mean hemoglobin was $9.4{\pm}1.9g/dl$ with a mean MCV of $78.1{\pm}7.9fl$, a mean total leukocyte count of $10.9{\pm}20.6{\times}10^9/l$ and a mean platelet count of $241.6{\pm}150.1{\times}10^9/l$. Conclusions: Our analysis shows that clinico-pathological features of cHL in Pakistan are comparable to published data. Peripheral lymphodenopathy associated with B symptoms is the commonest presentation. Bone marrow involvement is more common in our setup as patients usually presented at an advanced stage of disease.
Background: Acute lymphoblastic leukemia (ALL) is a malignant disease in which early lymphoid precursors proliferate and replace the normal hematopoiesis. It has distinctive clinical and biological features. In respect to adult ALL, available data from Pakistan are limited. Therefore we reviewed the demographical and clinicohematological profiles along with FAB stratification of adult patients with ALL presented at our hospital. Materials and Methods: In this cross sectional study, 51 adults (${\geq}15years$) patients with ALL were enrolled from January 2010 to December 2014. Results: The mean age was $23.8{\pm}12.9years$ with the median age of 18.0 years. The male to female ratio was 2:1. The major complaints were fever (60.7%), generalized weakness (47.0%), overt bleeding (19.6%) and weight loss (13.7%). Physical examination revealed lymphodenopathy as a predominant finding detected in 43.1% followed by splenomegaly and hepatomegaly in 23.5% and 21.5%, respectively. The mean hemoglobin level was $9.0{\pm}2.75g/dl$ with a mean MCV of $82.2{\pm}15.4fl$, a mean total leukocyte count of $31.1{\pm}64.0{\times}10^9/l$, a mean ANC of $2.1{\pm}3.0{\times}10^9/l$ and a mean platelet count of $71.7{\pm}85.7{\times}10^9/l$. According to FAB classification, 47.1% were L1 type, 45.1% L2 and 7.8% L3 variant. Conclusions: Clinico-pathological features appeared comparable to published data. Febrile illness associated with lymphodenopathy was the commonest presentation. FAB classification revealed a predominance of ALL-L1 variant in Pakistani adult patients with ALL.
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