Blood management strategies in congenital Glanzmann thrombasthenia at a hematology referral center

Ganesh Kasinathan,Jameela Sathar 대한혈액학회 2021 Blood Research Vol.56 No.4

Background Glanzmann thrombasthenia is associated with abnormalities in the glycoprotein IIb/IIIa receptor. This study, conducted at Ampang Hospital, Malaysia, aimed to assess outcomes of blood management strategies for Glanzmann thrombasthenia. Methods Ten patients with Glanzmann thrombasthenia aged 9 years (2009‒2018) were examined. Data on clinical characteristics, transfusion practices, and patient blood management were obtained from medical records. Patient blood management methods included parenteral iron, erythropoietin, hormonal pills, intrauterine progesterone contraceptive devices, tranexamic acid, and recombinant factor VIIa. Primary outcomes were hemoglobin levels and the proportion of patients who received blood transfusion. Secondary outcomes were morbidity and mortality. Results The median age at diagnosis was 8.2 years (range, 1‒15 yr). The female-to-male ratio was 9:1. Eight patients had type 2 disease (5‒20% of normal GPIIb/IIIa), and two patients had type 1 disease (normal GPIIb/IIIa <5%). All patients had iron deficiency. All female patients presented with significant menorrhagia. Other bleeding symptoms included epistaxis, spontaneous skin bruising, hemoptysis, gingival bleeding, knee hemarthrosis, and pelvic hematoma. No patient experienced life-threatening bleeding. Our patients had a mean hemoglobin level of 5.6 g/dL at diagnosis. All patients were optimized using non-transfusion methods as described above. Our patient had a current mean hemoglobin level of 11 g/dL. Approximately 70% (7/10) of patients did not experience receiving blood transfusions in the last 5 years. No patient experienced non-transfusion-related morbidities such as sepsis, thromboembolism, or cardiorespiratory events. Conclusion High cost, transfusion-related adverse events, and immunomodulation could be effectively prevented by avoiding unnecessary blood transfusions.

Disrupting Escherichia coli : A Comparison of Methods

Benov, Ludmil,AlIbraheem, Jameela 한국생화학분자생물학회 2002 BMB Reports Vol.35 No.4

The often-encountered problem of disrupting bacteria for the purpose of extracting soluble protein has generated various methods. Many require specialized equipment. Very often, especially during preliminary studies, investigators need a simple, fast, and inexpensive method for cell disruption that preserves biological activity. This paper compares some simple and inexpensive methods for cell disruption, such as bead-vortexing, freezing-thawing, French pressing, and sonication. It also provides some tips to increase protein yield and preserve biological activity. If performed under optimal conditions, bead-vortexing gives protein yields that are comparable to French pressing and sonication. It also preserves the activities of labile enzymes and releases periplasmic enzymes. Vortexing with glass beads appears to be the simplest method for cell disruption.

Various Aspects, Patterns and Risk Factors in Breast Cancer Patients of Balochistan

Baloch, Abdul Hameed,Shuja, Jameela,Daud, Shakeela,Ahmed, Muneer,Ahmad, Adeel,Tareen, Mehrullah,Khan, Farah,Kakar, Muhammad Azam,Baloch, Dost Mohammad,Kakar, Naseebullah,Naseeb, Hafiz Khush,Ahmad, Jam Asian Pacific Journal of Cancer Prevention 2012 Asian Pacific journal of cancer prevention Vol.13 No.8

Purpose: Breast cancer is the commonest malignancy of females throughout the world with one million new cases each year. In Pakistan, the burden of breast cancer disease is high with late stage presentation being a common feature, more than half being stage III or stage IV. The objective of this study was to study various aspects, patterns and risk factors in breast cancer patients of Balochistan. Method: Present study was performed on 134 patients of breast cancer who were registered in CENAR. The patients were interviewed by providing a questionnaire. Informed consent was taken from all the patients who took part in this study after explanation of the study aims. Body mass index (BMI) was calculated andbiopsy reports were obtained from patients files. All the cases were classified with respect to age, gender, ethnic group (Baloch, Pashtoon, Punjabi, Afghani, Hazara) BMI, cancer type, cancer grade, hormonal status, side of the cancer, fertility and marital status. Results: Out of 134 patients, the most common ethnic group was Pashtoon with a total of 42 and the common age group was 41-50 years with a total of 51. Invasive ductal carcinoma (IDC) was the most common type, accounting for in 128 patients (95.5%) followed by invasive lobular carcinoma (ILC). Conclusion: Pashtoon was the most common ethnic group, IDC was common type and most of the patients had an ER/PR positive hormonal status.

Spondylodiscitis Complicated by the Ingestion of a Button Battery: a Case Report

Praharaju Janaki Sudhakar,Jameela Al-Dossary,Neelam Malik 대한영상의학회 2008 Korean Journal of Radiology Vol.9 No.6

A one-and-a-half year old boy who presented with a cough, irritability, and refusal of food was found to have an impacted foreign body in his upper esophagus. We present the radiologic findings of the foreign body (a button battery) impacted in the upper esophagus and its complications; namely, mediastinitis and spondylodiscitis after endoscopic removal. Further, plain radiograph and MR imaging findings are discussed along with literature review. A one-and-a-half year old boy who presented with a cough, irritability, and refusal of food was found to have an impacted foreign body in his upper esophagus. We present the radiologic findings of the foreign body (a button battery) impacted in the upper esophagus and its complications; namely, mediastinitis and spondylodiscitis after endoscopic removal. Further, plain radiograph and MR imaging findings are discussed along with literature review.

Clinical characteristics and outcomes of thrombotic microangiopathy in Malaysia

Yee Yee Yap,Jameela Sathar,Kian Boon Law,Putri Astina Binti Zulkurnain,Syed Carlo Edmund,Kian Meng Chang,Ross Baker 대한혈액학회 2018 Blood Research Vol.53 No.2

Background Thrombotic microangiopathy (TMA) with non-deficient ADAMTS-13 (a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13) outcome is unknown hence the survival analysis correlating with ADAMTS-13 activity is conducted in Malaysia/ Methods This was a retrospective epidemiological study involving all cases of TMA from 2012‒2016. Results We evaluated 243 patients with a median age of 34.2 years; 57.6% were female. Majority of the patients were Malay (62.5%), followed by Chinese (23.5%) and Indian (8.6%). The proportion of patients with thrombotic thrombocytopenic purpura (TTP) was 20.9%, 72.2% of which were acquired while 27.8% were congenital. Patients with ADAMTS-13 activity ≥5% had a four-fold higher odds of mortality compared to those with ADAMTS-13 activity <5% (odds ratio: 4.133, P=0.0425). The mortality rate was 22.6% (N=55). Most cases had secondary etiologies (42.5%), followed by acquired TTP (16.6%), atypical hemolytic uremic syndrome (HUS) or HUS (12.8%) and congenital TTP (6.4%). Patients with secondary TMA had inferior overall survival (P=0.0387). The secondary causes comprised systemic lupus erythematosus (30%), infection (29%), pregnancy (10%), transplant (8%), malignancy (6%), and drugs (3%). Transplant-associated TMA had the worst OS (P=0.0016) among the secondary causes. Plasma exchange, methyl-prednisolone and intravenous immunoglobulin were recorded as first-line treatments in 162 patients, while rituximab, bortezomib, vincristine, azathioprine, cyclo-phosphamide, cyclosporine, and tacrolimus were described in 78 patients as second-line treatment. Conclusion This study showed that TMA without ADAMTS-13 deficiency yielded inferior outcomes compared to TMA with severeADAMTS-13 deficiency, although this difference was not statistically significant.

Disrupting Escherichia coli: A Comparison of Methods

Benov, Ludmil,Al-Ibraheem, Jameela 생화학분자생물학회 2002 Journal of biochemistry and molecular biology Vol.35 No.4

The often-encountered problem of disrupting bacteria for the purpose of extracting soluble protein has generated various methods. Many require specialized equipment. Very often, especially during preliminary studies, investigators need a simple, fast, and inexpensive method for cell disruption that preserves biological activity. This paper compares some simple and inexpensive methods for cell disruption, such as bead-vortexing, freesing-thawing, French pressing, and sonication. It also provides some tips to increase protein yield and preserve biological activity. If performed under optimal conditions, bead-vortexing gives protein yields that are comparable to French pressing and sonication. It also preserves the activities of labile enzymes and releases periplasmic enzymes. Vortexing with glass beads appears to be the simplest method for cell disruption.

Clinical characteristics and outcomes of thrombotic microangiopathy in Malaysia

Yee Yee Yap,Jameela Sathar,Kian Boon Law,Putri Astina Binti Zulkurnain,Syed Carlo Edmund,Kian Meng Chang,Ross Baker 대한혈액학회 2018 Blood Research Vol.53 No.2

Background Thrombotic microangiopathy (TMA) with non-deficient ADAMTS-13 (a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13) outcome is unknown hence the survival analysis correlating with ADAMTS-13 activity is conducted in Malaysia/ Methods This was a retrospective epidemiological study involving all cases of TMA from 2012‒2016. Results We evaluated 243 patients with a median age of 34.2 years; 57.6% were female. Majority of the patients were Malay (62.5%), followed by Chinese (23.5%) and Indian (8.6%). The proportion of patients with thrombotic thrombocytopenic purpura (TTP) was 20.9%, 72.2% of which were acquired while 27.8% were congenital. Patients with ADAMTS-13 activity ≥5% had a four-fold higher odds of mortality compared to those with ADAMTS-13 activity <5% (odds ratio: 4.133, P=0.0425). The mortality rate was 22.6% (N=55). Most cases had secondary etiologies (42.5%), followed by acquired TTP (16.6%), atypical hemolytic uremic syndrome (HUS) or HUS (12.8%) and congenital TTP (6.4%). Patients with secondary TMA had inferior overall survival (P=0.0387). The secondary causes comprised systemic lupus erythematosus (30%), infection (29%), pregnancy (10%), transplant (8%), malignancy (6%), and drugs (3%). Transplant-associated TMA had the worst OS (P=0.0016) among the secondary causes. Plasma exchange, methyl-prednisolone and intravenous immunoglobulin were recorded as first-line treatments in 162 patients, while rituximab, bortezomib, vincristine, azathioprine, cyclo-phosphamide, cyclosporine, and tacrolimus were described in 78 patients as second-line treatment. Conclusion This study showed that TMA without ADAMTS-13 deficiency yielded inferior outcomes compared to TMA with severeADAMTS-13 deficiency, although this difference was not statistically significant.

Philadelphia-positive (PH+) acute lymphoblastic leukemia (ALL): developing strategies for curing this stubborn disease

Christopher Chin Keong Liam,Yang Liang Boo,Siew Lian Chong,Jameela Sathar,Tee Chuan Ong,Sen Mui Tan 대한혈액학회 2022 Blood Research Vol.57 No.2

Antimicrobial Management of Complicated Skin and Soft Tissue Infections in an Era of Emerging Multi-Drug Resistance: Perspectives from 5 Gulf Countries

Wadha Alfouzan,Zainab Al-Balushi,Muna Al-Maslamani,Asmaa Al-Rashed,Salman Al-Sabah,Jameela Al-Salman,Mohamed Baguneid,Faryal Khamis,Nervana Habashy,Ayman Kurdi,Christian Eckmann 대한외상중환자외과학회 2021 Journal of Acute Care Surgery Vol.11 No.3

The number of complicated skin and soft tissue infections (cSSTIs) in the Arabian Gulf region has risen in recent years, particularly those caused by multi-drug resistant (MDR) pathogens. The high prevalence of diabetes, obesity, and associated cardio-metabolic comorbidities in the region renders medical and surgical management of cSSTI patients with MDR infections challenging. An experienced panel of international and regional cSSTI experts (consensus group on cSSTIs) was convened to discuss clinical considerations for MDR infections from societal, antimicrobial stewardship, and cost perspectives, to develop best practice recommendations. This article discusses antibiotic therapies suitable for treating MDR cSSTIs in patients from the Gulf region and recommends that these should be tailored according to the local bacterial ecology by country and region. The article highlights the need for a comprehensive patient treatment pathway and defined roles of each of the multidisciplinary teams involved with managing patients with MDR cSSTIs. Aligned and inclusive definitions of cSSTIs for clinical and research purposes, thorough and updated epidemiological data on cSSTIs and methicillin-resistant Staphylococcus aureus in the region, clearcut indications of novel agents and comprehensive assessment of comparative data should be factored into decision-making are necessary. The number of complicated skin and soft tissue infections (cSSTIs) in the Arabian Gulf region has risen in recent years, particularly those caused by multi-drug resistant (MDR) pathogens. The high prevalence of diabetes, obesity, and associated cardio-metabolic comorbidities in the region renders medical and surgical management of cSSTI patients with MDR infections challenging. An experienced panel of international and regional cSSTI experts (consensus group on cSSTIs) was convened to discuss clinical considerations for MDR infections from societal, antimicrobial stewardship, and cost perspectives, to develop best practice recommendations. This article discusses antibiotic therapies suitable for treating MDR cSSTIs in patients from the Gulf region and recommends that these should be tailored according to the local bacterial ecology by country and region. The article highlights the need for a comprehensive patient treatment pathway and defined roles of each of the multidisciplinary teams involved with managing patients with MDR cSSTIs. Aligned and inclusive definitions of cSSTIs for clinical and research purposes, thorough and updated epidemiological data on cSSTIs and methicillin-resistant Staphylococcus aureus in the region, clear-cut indications of novel agents and comprehensive assessment of comparative data should be factored into decision-making are necessary.

Validating lactate dehydrogenase (LDH) as a component of the PLASMIC predictive tool (PLASMIC-LDH)

Christopher Chin Keong Liam,Jim Yu-Hsiang Tiao,Yee Yee Yap,Yi Lin Lee,Jameela Sathar,Simon McRae,Amanda Davis,Jennifer Curnow,Robert Bird,Philip Choi,Pantep Angchaisuksiri,Sim Leng Tien,Joyce Ching Me 대한혈액학회 2023 Blood Research Vol.58 No.1

Background The PLASMIC score is a convenient tool for predicting ADAMTS13 activity of <10%. Lactate dehydrogenase (LDH) is widely used as a marker of haemolysis in thrombotic thrombocytopenic purpura (TTP) monitoring, and could be used as a replacement marker for lysis. We aimed to validate the PLASMIC score in a multi-centre Asia Pacific region, and to explore whether LDH could be used as a replacement marker for lysis. Methods Records of patients with thrombotic microangiopathy (TMA) were reviewed. Patients’ ADAMTS13 activity levels were obtained, along with clinical/laboratory findings relevant to the PLASMIC score. Both PLASMIC scores and PLASMIC-LDH scores, in which LDH replaced traditional lysis markers, were calculated. We generated a receiver operator characteristics (ROC) curve and compared the area under the curve values (AUC) to determine the predictive ability of each score. Results 46 patients fulfilled the inclusion criteria, of which 34 had ADAMTS13 activity levels of <10%. When the patients were divided into intermediate-to-high risk (scores 5‒7) and low risk (scores 0‒4), the PLASMIC score showed a sensitivity of 97.1% and specificity of 58.3%, with a positive predictive value (PPV) of 86.8% and negative predictive value (NPV) of 87.5%. The PLASMIC-LDH score had a sensitivity of 97.1% and specificity of 33.3%, with a PPV of 80.5% and NPV of 80.0%. Conclusion Our study validated the utility of the PLASMIC score, and demonstrated PLASMIC-LDH as a reasonable alternative in the absence of traditional lysis markers, to help identify high-risk patients for treatment via plasma exchange.