1.Rare bleeding disorders: spectrum of disease and clinical manifestations in the Pakistani population
Rafia MAHMOOD ; Asad MAHMOOD ; Maria KHAN ; Sadia ALI ; Saleem Ahmed KHAN ; Syed Raza JAFFAR
Blood Research 2020;55(3):146-150
Background:
Rare inherited coagulation factor deficiencies constitute an important group of bleeding disorders. A higher frequency of these disorders is seen in areas of high consanguinity.Our aim was to study the prevalence and spectrum of rare inherited bleeding disorders, characterize the severity of the deficiencies, identify different clinical manifestations, and evaluate different treatments provided.
Methods:
This cross-sectional study was conducted in the Department of Haematology, Armed Forces Institute of Pathology Rawalpindi, between January 2014 and December 2018.A detailed history was taken, and an examination was performed. The signs and symptoms were noted, and the patients were diagnosed on the basis of a coagulation profile. The disease severity was assessed using factor assays.
Results:
Among 2,516 patients with suspected coagulation disorders, 774 (30.8%) had an inherited bleeding disorder. Of the 774 patients, 165 (21.3%) had a rare bleeding disorder;91 (55.2%) of them were males, and 74 (44.9%) were females, with a male-to-female ratio of 1.2:1. The median patient age was 9 years 3 months. The most common disorder was factor VII deficiency (46 patients, 27.9%). The most common clinical presentation was bruising in 102 (61.8%) and gum bleeding in 91 (55.2%) patients.
Conclusion
The most common rare bleeding disorder in our population is factor VII deficiency. The prevalence of these bleeding disorders is high in our population due to a high number of consanguineous marriages.
2.Clinicohematological parameters and outcomes in a cohort of chronic lymphocytic leukemia patients with Deletion 17p from Pakistan.
Rafia MAHMOOD ; Saleem Ahmed KHAN ; Chaudhry ALTAF ; Hamid Saeed MALIK ; Muhammad Tahir KHADIM
Blood Research 2018;53(4):276-280
BACKGROUND: Chronic lymphocytic leukemia (CLL) exhibits profound heterogeneity in its clinical course. Its clinicohematological and cytogenetic features play a significant role in determining the clinical course and in predicting the treatment response and prognosis. In this context, 17p deletion is known to predict a poor prognosis, as these cases are refractory to conventional therapy. This study aimed to evaluate the clinicohematological characteristics, outcomes, and prognostic factors among CLL patients with and without del 17p in Pakistan. METHODS: This prospective observational study was conducted at the Department of Haematology, Armed Forces Institute of Pathology (Rawalpindi, Pakistan) between January 2013 and December 2017. Patients were diagnosed based on the International Workshop on Chronic Lymphocytic Leukaemia IWCLL criteria, their clinicohematological parameters were recorded, and cytogenetic analyses were performed. The time from diagnosis to treatment and the 2-year overall survival rate were also evaluated. RESULTS: We evaluated 130 CLL cases, including 24 patients (18.5%) with del 17p, who included 18 men (75%) and 6 women (25%). The median age was 68 years. Binet stage C was detected at the presentation in 16 patients (67%). Treatment was administered to 14 patients (70%) at a median interval of 11 months (range, 0–28 mo) after diagnosis. The overall response rate was 64.3%, the median event-free survival was 9 months (range, 1–23 mo), and the 2-year overall survival rate was 65%. CONCLUSION: Del 17p is relatively common in Pakistan, and patients harboring this deletion had poor treatment response and survival outcomes.
Arm
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Cohort Studies*
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Cytogenetic Analysis
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Cytogenetics
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Diagnosis
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Disease-Free Survival
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Education
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Female
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Humans
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In Situ Hybridization, Fluorescence
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Leukemia, Lymphocytic, Chronic, B-Cell*
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Male
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Observational Study
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Pakistan*
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Pathology
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Population Characteristics
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Prognosis
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Prospective Studies
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Survival Rate
3.Updates on Knowledge, Attitude and Preventive Practices on Tuberculosis among Healthcare Workers
Farhanah Abd Wahab ; Sarimah Abdullah ; Jafri Malin Abdullah ; Hasnan Jaafar ; Siti Suraiya Md Noor ; Wan Mohd Zahiruddin Wan Mohammad ; Abdul Aziz Mohamed Yusoff ; John Tharakan ; Shalini Bhaskar ; Muthuraju Sangu ; Mohd Shah Mahmood ; Fauziah Kassim ; Md. Hanip Rafia ; Mohammed Safari Mohammed Haspani ; Azmi Alias ; Rogelio Hernández Pando
Malaysian Journal of Medical Sciences 2016;23(6):25-34
Ranking as the most communicable disease killer worldwide, tuberculosis, has accounted
with a total of 9.6 million new tuberculosis cases with 1.5 million tuberculosis-related deaths
reported globally in 2014. Tuberculosis has remain as an occupational hazard for healthcare
workers since 1920s and due to several tuberculosis outbreaks in healthcare settings in the early
1990s, the concern about the transmission to both patients and healthcare workers has been
raised. Healthcare workers have two to three folds greater the risk of active tuberculosis than the
general population. Several studies on knowledge, attitude and practices on tuberculosis among
healthcare workers worldwide have revealed that majority of the participated healthcare workers
had good knowledge on tuberculosis. Most of the healthcare workers from South India and South
Africa also reported to have positive attitude whereas a study in Thailand reported that most of
the healthcare providers have negative attitude towards tuberculosis patients. Nevertheless,
majority of the healthcare workers have low level of practice on tuberculosis prevention. An
improved communication between healthcare workers and the patients as well as their families
is the key to better therapeutic outcomes with good knowledge, attitude and preventive practice
towards tuberculosis.