A case series of acquired haemophilia in a Malaysian hospital: unpredictabty rare medical emergency

Acquired haemophilia (AH) is a rare bleeding disorder characterized by the presence of acquired inhibitors against Factor VIII causing disruption of coagulation cascade. It has no known genetic inheritance, and diagnosis remains a challenge. The peculiar presentations are later age of onset as acute pain in weight-bearing joints and spontaneous muscle haematoma with isolated prolonged activated partial thrombin time (APTT). Prevalence is 1 per million per year affecting both genders equally where blood product transfusion is seen in almost 87% of cases. The direct cause of AH is still unknown, and autoimmune dysregulation has been postulated, which predisposes to the development of the factor inhibitors. Being extremely rare, we are reporting two consecutive patients diagnosed by unusual bleeding episodes with isolated prolonged APTT due to Factor VIII inhibitors. AH deserves a special mention as high index of suspicion is required. More studies are required to provide better guidance in diagnosis and management of this condition

Thrombosis at unusual sites in reproductive age group-a case series

Thrombosis is one of the causes of morbidity and mortality in women of reproductive age group. Thrombosis at unusual sites may pose diagnostic and management dilemma for health care personnel. Teamwork and good communication provide the best modalities for maximum benefits to patients. Here with, we presented case a series of thrombosis at unusual sites seen and managed in our clinic. A 35 year-old Malay lady presented with left hemiparesis while she was on oestrogen based combined contraception pills (C-OCP). Imaging studies showed extensive venous thrombosis with bilateral acute cortical infarct. Thrombophilia screening of antiphospholipid syndrome were negative. She was put on anticoagulant and stopped 2 years after the incident. A 40 year-old Malay lady presented with abdominal discomfort, lethargy and massive splenomegaly. Bone marrow and trephine examination revealed primary myelofibrosis with positive JAK2617F. Imaging study showed chronic portal vein thrombosis with portal vein hypertension, complicated by gastro-oesophageal varices. She was put on hydroxyurea and later started on ruxolitinib with banding done over her gastro-oesophageal varices. A 26 year-old Malay lady presented with serositis, mouth ulcer and anaemia symptoms. Laboratory studies were positive for systemic lupus erythematosus and negative for antiphospholipid study. Imaging study showed long segment thrombosis of right internal jugular vein with surrounding subcutaneous oedema. She is currently stable on anticoagulants and steroid. Teamwork and holistic approach is practiced in the investigation and management to provide maximum benefits for patients

Diagnostic dilemma of reactive arthritis aided by multimodality imaging using MRI, CECT and 18F-FDG PET/CT scans

Reactive arthritis can be an elusive diagnosis especially in the elderly. A 77-year-old lady, presented with recent history of hip pain. She had been treated for urinary tract infection caused by Chlamydia sp. and had associated weight loss. She was also investigated for possible tuberculosis and occult malignancy. CT scan abdomen/pelvis and MRI revealed peri-articular muscle inflammation. Biopsy of her hip joint failed to find the causative factor. Whole-body 18F-FDG PET/CT scan revealed increased FDG uptake at bilateral hip and shoulder joints. She recovered after an intensive course of antibiotics. Thus, she was diagnosed with reactive arthritis. Reactive arthritis is usually a diagnosis of exclusion made by a high index of suspicion and positive serology test. Molecular imaging can be an alternative investigation for joint pains in the elderly, which enables excellent anatomical and functional information to exclude more sinister conditions such as malignancy

Construction of phosphomannose isomerase (PMI) transformation vectors and evaluation of the effectiveness of vectors in tobacco (Nicotiana tabacum L)

Phosphomannose isomerase (pmi) gene isolated from Escherichia coli allows transgenic plants carrying it to convert mannose-6- phosphate (from mannose), a carbon source that could not be naturally utilized by plants into fructose-6-phosphate which can be utilized by plants as a carbon source. This conversion ability provides energy source to allow the transformed cells to survive on the medium containing mannose. In this study, four transformation vectors carrying the pmi gene alone or in combination with the β-glucuronidase (gusA) gene were constructed and driven by either the maize ubiquitin (Ubi1) or the cauliflower mosaic virus (CaMV35S) promoter. Restriction digestion, PCR amplification and sequencing were carried out to ensure sequence integrity and orientation. Tobacco was used as a model system to study the effectiveness of the constructs and selection system. PMI11G and pMI3G, which carry gusA gene, were used to study the gene transient expression in tobacco. PMI3 construct, which only carries the pmi gene driven by CaMV35S promoter, was stably transformed into tobacco using biolistics after selection on 30 g 1-1 mannose without sucrose. Transgenic plants were verified using PCR analysis

IL-8 as a potential in-vitro severity biomarker for dengue disease

Dengue is a common infection, caused by dengue virus. There are four different dengue serotypes, with different capacity to cause severe dengue infections. Besides, secondary infections with heterologous serotypes, concurrent infections of multiple dengue serotypes may alter the severity of dengue infection. This study aims to compare the severity of single infection and concurrent infections of different combinations of dengue serotypes in-vitro. Human mast cells (HMC)-1.1 were infected with single and concurrent infections of multiple dengue serotypes. The infected HMC-1.1 supernatant was then added to human umbilical cord vascular endothelial cells (HUVEC) and severity of dengue infections was measured by the percentage of transendothelial electrical resistance (TEER). Levels of IL- 10, CXCL10 and sTRAIL in HMC-1.1 and IL-8, IL-10 and CXCL10 in HUVEC culture supernatants were measured by the ELISA assays. The result showed that the percentage of TEER values were significantly lower in single infections (p0.4), indicating that IL-8 may be suitable as an in-vitro severity biomarker. In conclusion, this in-vitro model presented few similarities with regards to the conditions in dengue patients, suggesting that it could serve as a severity model to test for severity and levels of severity biomarkers upon different dengue virus infections

Assessment of depression and anxiety in hematological cancer patients and their relationship with quality of life.

OBJECTIVES:To determine the relationship between major depressive disorder, anxiety disorders and the quality of life of haematological cancer patients. METHODS: This cross-sectional study was conducted at Ampang Hospital Kuala Lumpur, Malaysia, a tertiary referral centre hospital for haematological cancer. The Mini-International Neuropsychiatric Interview was used for the diagnosis of major depressive disorder and anxiety disorders. The European Organization for Research and Treatment of Cancer Quality of Life Questionnaire was utilised to measure patients' quality of life. RESULTS: A total of 105 haematological cancer patients were included in the study with response rate of 100%. Major depressive disorder correlated with almost all domains of the quality of life, except the pain scores. Logistic regression showed that insomnia and financial difficulties were related to major depressive disorder. Different anxiety disorders also correlated with quality of life in specific domains. The leading anxiety disorders that correlated mostly with quality-of-life scales were generalised anxiety disorder, followed by obsessive-compulsive disorder, social anxiety disorder, as well as post-traumatic stress disorder and panic disorder with agoraphobia (p<0.05). CONCLUSIONS: Psychological treatment along with medication and intervention should be implemented to improve the overall quality of life and psychiatric disorder symptoms among the haematological cancer patients

The socio-demographic and clinical factors associated with quality of life among patients with haematological cancer in a large government hospital in Malaysia.

Background: The paper examined the quality of life of haematological cancer patients according to their socio-demographic profiles and clinical diagnoses. Methods: This cross-sectional study was conducted at the tertiary referral centre of Ampang Hospital, Kuala Lumpur, involving 105 patients. The European Organisation for Research and Treatment of Cancer Quality of Life (EORTC QLQ-C30) questionnaire was used to measure their quality of life. Results: The study involved patients diagnosed with all types of haematological cancer, including non-Hodgkin lymphoma (NHL), acute myelogenous leukaemia (AML), acute lymphoblastic leukaemia (ALL), Hodgkin lymphoma (HL), and multiple myeloma (MM), with a response rate of 83.3%. The patients with ALL, HL, without NHL, and without MM were younger than other patients. There were significant differences in quality of life scores in different socio-demographic groups and types of cancer diagnosis. The global quality of life of the female patients was much better than that of the male patients. Patients who were 40 years old or younger had a better global quality of life and physical functioning, as well as fewer symptoms of constipation, nausea, and vomiting. Employed patients were in less pain but showed greater impairments of cognitive function than did unemployed patients. Patients who earned a monthly wage of RM1000 or less had reduced physical function, more symptoms of pain, and more financial difficulties compared with patients who earned more. Patients with AML tended to have better physical functioning than did patients with MM, whose physical functioning was impaired. Comparatively, more symptoms of dyspnoea were found in ALL and HL patients than in other types of lymphoma. Compared with other patients, those with ALL had a greater loss of appetite, and other lymphoma patients had fewer symptoms of pain. Patients with NHL had impaired role functioning and more constipation compared with other patients. The results were all statistically significant (P < 0.05). Conclusion: The quality of life of haematological cancer patients is affected by sociodemographic factors and clinical diagnoses. Efforts should be made to improve the overall quality of life of these patients

Thrombosis at unusual sites in reproductive age group: a case series

Thrombosis is one of the causes of morbidity and mortality in women of reproductive age group. Thrombosis at unusual sites may pose diagnostic and management dilemma for health care personnel. Teamwork and good communication provide the best modalities for maximum benefits to patients. Here with, we presented case a series of thrombosis at unusual sites seen and managed in our clinic. A 35 year-old Malay lady presented with left hemiparesis while she was on oestrogen based combined contraception pills (C-OCP). Imaging studies showed extensive venous thrombosis with bilateral acute cortical infarct. Thrombophilia screening of antiphospholipid syndrome were negative. She was put on anticoagulant and stopped 2 years after the incident. A 40 year-old Malay lady presented with abdominal discomfort, lethargy and massive splenomegaly. Bone marrow and trephine examination revealed primary myelofibrosis with positive JAK2617F. Imaging study showed chronic portal vein thrombosis with portal vein hypertension, complicated by gastro-oesophageal varices. She was put on hydroxyurea and later started on ruxolitinib with banding done over her gastro-oesophageal varices. A 26 year-old Malay lady presented with serositis, mouth ulcer and anaemia symptoms. Laboratory studies were positive for systemic lupus erythematosus and negative for antiphospholipid study. Imaging study showed long segment thrombosis of right internal jugular vein with surrounding subcutaneous oedema. She is currently stable on anticoagulants and steroid. Teamwork and holistic approach is practiced in the investigation and management to provide maximum benefits for patients

Depression and quality of life among patients with hematological cancer in a Malaysian hospital

Objective: To determine the prevalence of major depressive disorder (MDD) in hematological cancer patients and to investigate MDD with quality of life. Methods: The research, which uses a cross sectional design, has been carried out at Ampang Hospital, Kuala Lumpur. The hospital is a tertiary referral center for cancer cases that include non-Hodgkin lymphoma, acute myelogenous leukemia, acute lymphoblastic leukemia, Hodgkin lymphoma and other hematological cancers. In total, 105 patients with hematological malignancies were included in the study. This study employed the MINI International Neuropsychiatric Interview for diagnosis of MDD, the Patient Health Questionnaire (PHQ-9) for symptom severity of depression and the European Organisation for Research and Treatment of Cancer Quality Of Life questionnaire (EORTC QLQ-C30) to assess the quality of life of the respondents. Result: The response rate was 83.3%. The prevalence of MDD was 24.8% (n=26) with the majority of cases classified as moderately severe depression (38.5%). About 92.3% (n=24) of depressed hematological cancer patients were diagnosed with a current episode of MDD. The depressed patients also had significantly reduced quality of life in physical, role, emotional, cognitive and social domains (p<0.05). They also had significantly more symptoms of fatigue, nausea and vomiting, dyspnea, insomnia, appetite loss, constipation and diarrhea in addition to having financial difficulties and poor global health statuses (p<0.05). Conclusion: The prevalence of depression among the hematological cancer patients was high and the depressed patients had reduced qualities of life. Special attention in psychotherapy would help to improve both depression level and the quality of life in these patients

Thrombosis at unusual sites in reproductive age group: a case series

Thrombosis is one of the causes of morbidity and mortality in women of reproductive age group. Thrombosis at unusual sites may pose diagnostic and management dilemma for health care personnel. Teamwork and good communication provide the best modalities for maximum benefits to patients. Here with, we presented case a series of thrombosis at unusual sites seen and managed in our clinic. A 35 year-old Malay lady presented with left hemiparesis while she was on oestrogen based combined contraception pills (C-OCP). Imaging studies showed extensive venous thrombosis with bilateral acute cortical infarct. Thrombophilia screening of antiphospholipid syndrome were negative. She was put on anticoagulant and stopped 2 years after the incident. A 40 year-old Malay lady presented with abdominal discomfort, lethargy and massive splenomegaly. Bone marrow and trephine examination revealed primary myelofibrosis with positive JAK2617F. Imaging study showed chronic portal vein thrombosis with portal vein hypertension, complicated by gastro-oesophageal varices. She was put on hydroxyurea and later started on ruxolitinib with banding done over her gastro-oesophageal varices. A 26 year-old Malay lady presented with serositis, mouth ulcer and anaemia symptoms. Laboratory studies were positive for systemic lupus erythematosus and negative for antiphospholipid study. Imaging study showed long segment thrombosis of right internal jugular vein with surrounding subcutaneous oedema. She is currently stable on anticoagulants and steroid. Teamwork and holistic approach is practiced in the investigation and management to provide maximum benefits for patients