1.Plasma-Derived Microparticles in Polycythaemia Vera
Madzlifah AHADON ; Suria Abdul AZIZ ; Chieh Lee WONG ; Chooi Fun LEONG
The Malaysian Journal of Pathology 2018;40(1):41-48
Introduction: Microparticles are membrane bound vesicles, measuring less than 1.0 um, which are released during cellular activation or during apoptosis. Studies have shown that these circulating microparticles play a role in coagulation, cell signaling and cellular interactions. Increased levels of circulating microparticles have been observed in a number of conditions where there is vascular dysfunction, thrombosis and inflammation. The objective of this study was to determine the various plasma-derived microparticles in patients with polycythaemia vera (PV) in Universiti Kebangsaan Malaysia Medical Centre and to compare them with normal control. Methods: A total of 15 patients with PV and 15 healthy volunteers were included in this cross-sectional descriptive study. Plasma samples from both patients and healthy volunteers were prepared and further processed for isolation of microparticles. Flow cytometry analyses were then carried out in all samples to determine the cellular origin of the microparticles. Full blood count parameters for both groups were also collected. Data collected were analyzed using SPSS version 12.0. Results: Patients with PV had a significantly higher percentage of platelet derived microparticles compared to healthy controls (P <0.05). The control group had a higher level of endothelial derived microparticles but the differences were not statistically significant (P > 0.05). Conclusion: The median percentage of positive events for platelet derived microparticles was higher in patients with PV compared to normal healthy controls.
2.Hb Lepore/β0-Thalassaemia With α+-Thalassaemia Interactions, A Potential Diagnostic Pitfall
Hafiza Alauddin ; Suziana Mohamad Nasir ; Madzlifah Ahadon ; Raja Zahratul Azma Raja Sabudin ; Azlin Ithnin ; Noor Hamidah Hussin ; Hamidah Alias ; Loh C-Khai ; Zarina Abdul Latiff ; Nor Azian Abdul Murad ; Ainoon Othman
The Malaysian Journal of Pathology 2015;37(3):287-292
Haemoglobin (Hb) Lepore is a variant Hb consisting of two α-globin and two δβ-globin chains. In a
heterozygote, it is associated with clinical findings of thalassaemia minor, but interactions with other
haemoglobinopathies can lead to various clinical phenotypes and pose diagnostic challenges. We
reported a pair of siblings from a Malay family, who presented with pallor and hepatosplenomegaly
at the ages of 21 months and 14 months old. The red cell indices and peripheral blood smears of
both patients showed features of thalassaemia intermedia. Other laboratory investigations of the
patients showed conflicting results. However, laboratory investigation results of the parents had led
to a presumptive diagnosis of compound heterozygote Hb Lepore/β-thalassaemia and co-inheritance
α+-thalassaemia (-α3.7). Hb Lepore has rarely been detected in Southeast Asian countries, particularly
in Malaysia. These two cases highlight the importance of family studies for accurate diagnosis,
hence appropriate clinical management and genetic counseling.
3.Hb lepore/β0-thalassaemia with α+-thalassaemia interactions, a potential diagnostic pitfall.
Alauddin, Hafiza ; Mohamad Nasir, Suziana ; Ahadon, Madzlifah ; Raja Sabudin, Raja Zahratul Azma ; Ithnin, Azlin ; Hussin, Noor Hamidah ; Alias, Hamidah ; Loh, C-Khai ; Abdul Latiff, Zarina ; Abdul Murad, Nor Azian ; Othman, Ainoon
The Malaysian Journal of Pathology 2015;37(3):287-92
Haemoglobin (Hb) Lepore is a variant Hb consisting of two α-globin and two δβ-globin chains. In a heterozygote, it is associated with clinical findings of thalassaemia minor, but interactions with other haemoglobinopathies can lead to various clinical phenotypes and pose diagnostic challenges. We reported a pair of siblings from a Malay family, who presented with pallor and hepatosplenomegaly at the ages of 21 months and 14 months old. The red cell indices and peripheral blood smears of both patients showed features of thalassaemia intermedia. Other laboratory investigations of the patients showed conflicting results. However, laboratory investigation results of the parents had led to a presumptive diagnosis of compound heterozygote Hb Lepore/β-thalassaemia and co-inheritance α+-thalassaemia (-α3.7). Hb Lepore has rarely been detected in Southeast Asian countries, particularly in Malaysia. These two cases highlight the importance of family studies for accurate diagnosis, hence appropriate clinical management and genetic counseling.