1.Bullous Pemphigoid in an Elderly Patient with Myelodysplastic Syndrome and Refractory Anemia Coupled with Excess of Blast.
Yin Yin LEE ; Ping Chong BEE ; Chew Kek LEE ; Manimalar NAIKER ; Rokiah ISMAIL
Annals of Dermatology 2011;23(Suppl 3):S390-S392
Bullous pemphigoid (BP) has a recognized association with solid organ tumors, but is relatively rare in hematological malignancies. We report a 67-year-old male who developed BP after being diagnosed with myelodysplastic syndrome and refractory anemia with excess of blast (RAEB). Skin biopsy elucidated sub-epidermal bulla using direct immunofluorescence, revealing linear C3 and IgG deposits along the basement membrane. His BP was recalcitrant to the conventional treatment and only responded to a combination of high dose oral prednisolone and azathioprine. The relative refractory nature of his condition and concurrent RAEB supports a paraneoplastic nature.
Aged
;
Anemia
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Anemia, Refractory
;
Anemia, Refractory, with Excess of Blasts
;
Azathioprine
;
Basement Membrane
;
Biopsy
;
Blister
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Fluorescent Antibody Technique, Direct
;
Hematologic Neoplasms
;
Humans
;
Immunoglobulin G
;
Male
;
Myelodysplastic Syndromes
;
Paraneoplastic Syndromes
;
Pemphigoid, Bullous
;
Prednisolone
;
Skin
2.A Patient with Beta-Propeller Protein-Associated Neurodegeneration: Treatment with Iron Chelation Therapy
Shen Yang LIM ; Ai Huey TAN ; Azlina AHMAD-ANNUAR ; Susanne A SCHNEIDER ; Ping Chong BEE ; Jia Lun LIM ; Norlisah RAMLI ; Mohamad Imran IDRIS
Journal of Movement Disorders 2018;11(2):89-92
We present a case of beta-propeller protein-associated neurodegeneration, a form of neurodegeneration with brain iron accumulation. The patient harbored a novel mutation in the WDR45 gene. A detailed video and description of her clinical condition are provided. Her movement disorder phenomenology was characterized primarily by limb stereotypies and gait dyspraxia. The patient's disability was advanced by the time iron-chelating therapy with deferiprone was initiated, and no clinical response in terms of cognitive function, behavior, speech, or movements were observed after one year of treatment.
Brain
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Chelation Therapy
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Cognition
;
Extremities
;
Gait Apraxia
;
Humans
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Iron
;
Movement Disorders
3.Lack of association between the LRRK2 A419V variant and Asian Parkinson's disease.
Aroma Agape GOPALAI ; Shen Yang LIM ; Zariah Abdul AZIZ ; Soo Kun LIM ; Li Ping TAN ; Yip Boon CHONG ; Chong Tin TAN ; Santhi PUVANARAJAH ; Shanti VISWANATHAN ; Rishikesan KUPPUSAMY ; Ai Huey TAN ; Thien Thien LIM ; Gaik Bee EOW ; Mohamed Ibrahim NORLINAH ; Hui Hua LI ; Yi ZHAO ; Azlina AHMAD-ANNUAR
Annals of the Academy of Medicine, Singapore 2013;42(5):237-240
INTRODUCTIONThe G2385R and R1628P LRRK2 gene variants have been associated with an increased risk of Parkinson's disease (PD) in the Asian population. Recently, a new LRRK2 gene variant, A419V, was reported to be a third risk variant for PD in Asian patients. Our objective was to investigate this finding in our cohort of Asian subjects.
MATERIALS AND METHODSEight hundred and twenty-eight subjects (404 PD patients, and 424 age and gender-matched control subjects without neurological disorders) were recruited. Genotyping was done by Taqman® allelic discrimination assay on an Applied Biosystems 7500 Fast Real-Time PCR machine.
RESULTSThe heterozygous A419V genotype was found in only 1 patient with PD, compared to 3 in the control group (0.4% vs 1.3%), giving an odds ratio of 0.35 (95% confidence interval (CI), 0.01 to 3.79; P = 0.624).
CONCLUSIONA419V is not an important LRRK2 risk variant in our Asian cohort of patients with PD. Our data are further supported by a literature review which showed that 4 out of 6 published studies reported a negative association of this variant in PD.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Alanine ; genetics ; Case-Control Studies ; China ; ethnology ; Cohort Studies ; Cytosine ; Female ; Gene Frequency ; Genetic Variation ; genetics ; Genotype ; Heterozygote ; Humans ; India ; ethnology ; Leucine-Rich Repeat Serine-Threonine Protein Kinase-2 ; Malaysia ; ethnology ; Male ; Middle Aged ; Parkinson Disease ; genetics ; Polymorphism, Genetic ; genetics ; Protein-Serine-Threonine Kinases ; genetics ; Risk Factors ; Singapore ; Thymine ; Valine ; genetics ; Young Adult
4.The predictive value of early molecular response in chronic myeloid leukaemia patients treated with imatinib in a single real-world medical centre in a developing country.
Ping Chong BEE ; Veera SEKARAN ; Richard Rui Jie NG ; Ting Yi KWEH ; Gin Gin GAN
Singapore medical journal 2017;58(3):150-154
INTRODUCTIONThe prognosis of patients with chronic myeloid leukaemia (CML) has improved since the introduction of imatinib. However, patients who do not achieve complete cytogenetic response (CCyR) and major molecular response (MMR) have poorer prognosis. Recent clinical trials have demonstrated that early and deeper cytogenetic and molecular responses predict a better long-term outcome. This study aimed to analyse the relationship between early molecular response and clinical outcome in a real-life setting.
METHODSThis retrospective study included all patients with CML, in chronic or accelerated phase, who were treated with imatinib at University of Malaya Medical Centre, Malaysia.
RESULTSA total of 70 patients were analysed. The median follow-up duration was 74 months, and the cumulative percentages of patients with CCyR and MMR were 80.0% and 65.7%, respectively. Overall survival (OS) and event-free survival (EFS) at ten years were 94.3% and 92.9%, respectively. Patients who achieved CCyR and MMR had significantly better OS and EFS than those who did not. At six months, patients who had a BCR-ABL level ≤ 10% had significantly better OS and EFS than those who had a BCR-ABL level > 10%. The target milestone of CCyR at 12 months and MMR at 18 months showed no survival advantage in our patients.
CONCLUSIONOur data showed that imatinib is still useful as first-line therapy. However, vigilant monitoring of patients who have a BCR-ABL level > 10% at six months of treatment should be implemented so that prompt action can be taken to provide the best outcome for these patients.
Academic Medical Centers ; Adult ; Antineoplastic Agents ; therapeutic use ; Cytogenetics ; Disease-Free Survival ; Female ; Follow-Up Studies ; Fusion Proteins, bcr-abl ; metabolism ; Humans ; Imatinib Mesylate ; therapeutic use ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; diagnosis ; drug therapy ; genetics ; Malaysia ; Male ; Middle Aged ; Predictive Value of Tests ; Prognosis ; Retrospective Studies ; Treatment Outcome ; Universities
5.Anxiety and depression in patients with haematological neoplasms in Malaysia
Gin Gin Gan ; Diana Leh Ching Ng ; Yeh Chwan Leong ; Ping Chong Bee ; Edmund Fui Min Chin ; Habibah Abdul Halim ; Tengku Ahmad Hidayat Tengku Aziz ; Henning Loo ; Alan KH Teh
The Medical Journal of Malaysia 2019;74(3):191-197
Background: It is not uncommon that anxiety and
depression occur in patients with cancers, and past
researches have shown that the quality of life of patients is
negatively affected. This study aims to determine the
prevalence of anxiety and depression of patients with
haematological cancers in Malaysia and to investigate the
possible association of these psychological symptoms with
their quality of life.
Methods: This is a cross-sectional study where patients with
haematological cancers attending two major hospitals were
recruited. Anxiety and depression symptoms were assessed
using the Hospital Anxiety and Depression scale (HADS).
Quality of life (QoL) of these patients was measured using
the European Organisation for Research and Treatment of
Cancer quality of life questionnaire (EORTC QLQ C30). An
overall summary QoL score in combination with financial
difficulty score and global health score were used for
analysis.
Results: A total of 319 patients were recruited. Thirty-three
percent of patients had anxiety symptoms, 23.5% had
depression symptoms. In summary the overall score of QoL
is significantly lower in patients with higher scores for
depression and anxiety, (p<0.05). Patients who exhibit
anxiety symptoms were more frequently female, still
undergoing treatment whereas patients who had higher
depression scores were older and had acute leukemias or
myeloproliferative neoplasms. Patients who have
depression are significantly associated with a higher
financial difficulty score, p<0.05.
Conclusion: The poor quality of life in patients who have
anxiety and depression should raise awareness amongst the
health professions treating them so that additional support
can be provided.