Prothrombotic markers in Thalassemia major patients: A

Background: It is being increasingly recognised thatthalassemia major patients, like intermedia, have increasedpropensity for thromboembolism. Deficiency of naturalanticoagulants is more recently defined finding contributingto the hypercoagulable state. The aim this study is todetermine natural anticoagulants levels and their correlationwith maternal characteristics, haematological andbiochemical markers.

Multiplex real-time RT-PCR assay for transfusion transmitted viruses in sero-negative allogeneic blood donors: an experience from Southern Pakistan

Background: Blood transfusion safety commences with healthy donor recruitment. The threat of transfusion transmitted infections is greatly minimized by serological tools but not entirely eliminated. Recently, nucleic-acid testing for blood donor screening has virtually eliminated this jeopardy. Methods: This prospective study was conducted from February 2015 to February 2016. Samples from seronegative donors were run on multiplex assay (Cobas, S-201 system platform, Roche) in a batch of six [MP-NAT]. In case of reactive pool, tests were run on every individual sample [IDNAT]. Results: Of 16957 donors, 16836 (99.2%) were replacement donors and the remaining 121 (0.7%) were voluntary donors, with a mean age of 29.09 ± 7.04 years. After serologic screening of all 16957 donors, 955 (5.6%) were found to be reactive; 291(1.71%) were reactive for hepatitis-B surface antigen, 361 (2.12%) for antibody to hepatitis C virus (anti-HCV), 14 (0.08%) for antibody to human immunodeficiency virus, 287 (1.69%) for syphilis and 2 (0.01%) for malaria. 14 (0.08%) NAT reactive donors were identified after testing the 16002 seronegative donors, with an overall NAT yield of one reactivity out of 1143 blood donations; 10 donors for HBV-DNA (HBV NAT yield-1:1600) and remaining 4 for HCV-RNA (HCV-NAT yield-1:4000). None were HIV positive. Conclusion: NAT has improved the safety attributes in blood products. Although the positivity rate for NAT testing is low but in view of the high prevalence of transfusion transmitted infections in our country, we recommend the parallel use of both serology and NAT screening of all donated blood.

Pattern of karyotypic aberrations in pakistani patients with de novo acute myeloid leukemia

Background: Acute myeloid leukemia [AMI] is a malignant disease of the bone marrow in which karyotypic analysis is the most important diagnostic and prognostic tool for predicting remission rate, relapse and overall survival. This study was carried out to determine the frequency and type of cytogenetic aberrations in de novo acute myeloid leukemia in adults at a tertiary care hospital

Materials and methods: This descriptive cross-sectional study was carried out in the Hematology Department, Liaquat National Hospital from November 2014 to April 2016.A total of 51 cases were diagnosed with AMI during the study period. Cytogenetic analysis was carried out by banding technique on bone marrow aspirate samples

Results: The mean age of the study subject was 42.03+/-17.70 years. Frequency of karyotyping abnormalities was observed in 47% of cases, in which most frequently occurring cytogenetic abnormalities were those of good cytogenetics including t[15;17] and t[8;21], seen in 23.5% and 9.8% of cases respectively. Intermediate risk cytogenetics including Del 9q was seen in 1.96% of cases. However, poor risk cytogenetics including complex cytogenetics, t[11;q23] and del [13] were seen in 7.8%, 1.96% and 1.96% of cases respectively. Normal cytogenetics was seen in 27 [52.9%] patients

Conclusion: Karyotyping is one of the most important diagnostic and prognostic tools and a maximum benefit could be attained through cytogenetic analysis. Cytogenetic aberrations in our series are more or less similar as reported at national level with preponderance of good risk cytogenetics in our setting

Primary versus secondary immune thrombocytopenia in adults; a comparative analysis of clinical and laboratory attributes in newly diagnosed patients in Southern Pakistan

Back ground: Immune thrombocytopenic purpura (ITP) is a hemorrhagic diathesis, characterized by platelets destruction alongside impaired production. Patients from Asian regions often exhibit distinctive characteristics in comparison to the western patients. We accomplished this study to evaluate the prevalence of primary versus secondary ITP along with the comparative analysis between them. The secondary objective was to determine the etiological spectrum of secondary ITP. Methods: We illustrate the results of a large cohort of newly diagnosed adults ITP from southern Pakistan. The study extended from January 2009-December 2013. Complete blood counts, HbsAg, Anti-HCV, ANA, stool for Helicobacterpylori were done on all. HIV, TSH, anti-dsDNA, RA factor, APLA and direct coombs test were evaluated in cases where indicated. Results: A total of 417 patients were included with a mean age of 40.95±14.82 years. Primarily disease was observed in the 3rd decade of life. Male to female ratio was 1:1.5. Mean platelets count was 46.21±27.45x109 /l. At diagnosis 43.16% (n=180) patients had hemorrhagic manifestations whilst 56.8% (n=237) were asymptomatic. None of the patient presented with visceral, retropharyngeal or intracranial bleed. The prevalence of secondary ITP was substantially higher (64.8%) as compared to primary ITP (35.2%). Secondary ITP was predominantly seen in HCV reactive patients (24.4%) followed by helicobacter-pylori infection (11%). Nevertheless 16.4% patients had underlying autoimmune disorders. Providentially no study subject was found to be HIV reactive. Conclusions: Our study revealed predominance of secondary ITP. However bleeding manifestations and degree of thrombocytopenia were high in primary-ITP. Infectious etiology followed by autoimmune disorders is mainly implicated for secondary ITP in our setting.

Efficacy of helicobacter pylori eradication as an upfront treatment of secondary immune thrombocytopenia: an experience from Pakistan

Background: The effect of Helicobacter-pylori eradication therapy on the platelet counts in patients with immune thrombocytopenia is still debatable. The aim of this study was to assess the response rates of standard triple eradication therapy in secondary immune thrombocytopenia with Helicobacter pylori infection. Methods: From January 2012 to December 2013, 197 patients were diagnosed to have immune thrombocytopenia, out of which 22(11.1%) patients infected with HelicobacterPylorus were enrolled in this study. Helicobacter-Pylori infection was documented by Helicobacter-pylori stool antigen enzyme immunoassay method. All positive patients were put on triple eradication therapy. The responses rates to treatment were defined as per International Working Group on ITP. Results: Mean age of patients was 43.18±12.5 years. There were 10(45.5%) males and 12 (54.5%) females. Of the 22 patients, 7(31.8%) exhibited a complete response (CR) to Hpylori eradication therapy; 10(45.4%) attained a response; and 5(22.7%) had no response. Mean base line platelet counts were 53.36±24.5x109 /l, while platelet counts at 4 week following eradication was 80.86±51.0x109 /l (P=0.003). The predictive factor of response following eradication therapy was baseline platelet counts. Virtually all responders had baseline platelet counts >30x109 /l and all non-responders had <30x109 /l of platelet counts. Conclusions: Though the prevalence of H-pylori is low, this study confirmed the efficacy of eradication in increasing the platelet counts in H-pylori positive patients with ITP. It is an important measure in short time, safe and very cost effective to achieve platelets increment. We endorse the routine detection and eradication treatment of H-pylori infective ITP patients.

Adverse reactions in allogeneic blood donors: a tertiary care experience from a developing country

Objectives: Fragmented blood transfusion services along with an unmotivated blood donation culture often leads to blood shortage. Donor retention is crucial to meet the increasing blood demand, and adverse donor reactions have a negative impact on donor return. The aim of this study was to estimate adverse donor reactions and identify any demographic association

Methods: We conducted a prospective study between January 2011 and December 2013. A total of 41,759 healthy donors were enrolled. Professionally trained donor attendants drew blood and all donors were observed during and following donation for possible adverse events for 20 minutes. Blood donors were asked to report if they suffered from any delayed adverse consequences

Results: Out of 41,759 blood donors, 537 [1.3%] experienced adverse reactions. The incidence was one in every 78 donations. The mean age of donors who experienced adverse events was 26.0 +/- 6.8 years, and all were male. Out of 537 donors, 429 [80%] developed vasovagal reaction [VVR], 133 [25%] had nausea, 63 [12%] fainted, 35 [6%] developed hyperventilation, 9 [2%] had delayed syncope, and 9 [2%] developed hematoma. Arterial prick, nerve injury, cardiac arrest, and seizures were not observed. Donors aged less than < 30 years and weighing <70 kg were significantly associated with VVR, hyperventilation, and nausea [p < 0.005]. Undergraduates and Urdu speaking donors also had a significant association with fainting and nausea, respectively [p < 0.05]

Conclusion: The prevalence of adverse events was low at our tertiary center. A VVR was the predominant adverse reaction and was associated with age and weight. Our study highlights the importance of these parameters in the donation process. A well-trained and experienced phlebotomist and pre-evaluation counseling of blood donors could further minimize the adverse reactions

Serum Fasting Lipid Profile In Children And Adolescents With -Thalassaemia Major In Southern Pakistan

Objective: Serum fasting lipid profile has been studied in various clinical spectrum of Beta (β)- thalassaemia syndrome. Premature cardiac impairment in thalassaemia major appears primarily due to iron accumulation and oxidative injury; however it might be a sequel of abnormal lipoprotein concentrations. The rational of this study is to analyse the serum fasting lipid profile in cardiovascular disease free β-thalassaemia major (β-TM) patients. Relationships with age, gender, haematological parameters, liver enzymes and serum ferritin were observed. Method: Fasting serum lipid levels, liver function test (LFT), complete blood count (CBC) and serum ferritin were measured in 36 patients with homozygous β-TM from March 2012 to March 2014. Patients were stratified into two groups, age ≤15 and >15 years, to determine the possible lipid profile distinction in relation to age. Results: 17 were males and 19 were females, with median age of 12.0 years. The mean total cholesterol (TC) and triglyceride (TG) were 5.01±1.32 and 8.36±5.28 mmol/L respectively. High TG was detected in 36.1%, while high density lipoprotein cholesterol (HDL) and low density lipoprotein cholesterol (LDL) were markedly low, 0.98±0.51 and 2.35±1.22 mmol/L respectively. No statistically significant difference was noted between the two age groups. The median TC to HDL ratio (TC:HDL) was elevated, 5.7 (4.0). We established significant correlation of total bilirubin with TC (r=-0.4), HDL(r=-0.5) and LDL (r=-0.4) (P<0.05). Conclusion: Dyslipidaemia in β-TM patients is irrespective of age and gender including low HDL and high TC:HDL, whilst high TC:HDL may contribute as a significant risk marker for future cardiac events in these patients.

Effect of iron chelator desferrioxamine on serum zinc levels in patients with beta thalassemia major

β-thalassemia is the most common genetic disorder worldwide with an increased prevalence around the Mediterranean, Indian subcontinent and in South-East Asia. Various siderotic and non-siderotic complications significantly impact the quality of life. Thalassemic patients are also at risk of zinc deficiency due to diverse causes including desferrioxamine chelation. This study sought to investigate the prevalence of zinc deficiency in beta thalassemia major patients on desferrioxamine for iron chelation. Study design: This was a descriptive, prospective, cross-sectional study over a 6-month period. 63 cases of beta thalassemia major within the age group of 5-15 years on desferrioxamine for at least 1 year, were included. Basic patient demographics such as age, gender and duration of disease were recorded. Serum zinc levels were determined by atomic absorption spectrophotometry. Results: The mean age of patients was 10.84±3.47 (5 to 15) years. There were 35 (55.6%) males and 28(44.4%) females. The prevalence of zinc deficiency (zinc levels < 50 μg / dl) was 22.2%. Proportions of deficiency were higher in males with a duration of disease beyond 10 years. Conclusions: Zinc deficiency is not uncommon in beta thalassemia patients on desferrioxamine. We suggest that zinc levels be regularly monitored in these patients.

Intraleukocytic hemozoin pigments in complicated Plasmodium falciparum cerebral malaria

No abstract available.

Visceral leishmaniasis in an Afghan woman with systemic lupus erythematosus

No abstract available.