MyMedR

Detection of immunoglobulin gene rearrangement in B-cell malignancies

Ainoon O, Hamidah AB, Cheong SK, Hamidah HN

Malays J Pathol, 2000 Jun;22(1):5-11.

Rearrangement of the immunoglobulin heavy chain (IgH) gene has been used as a marker of lineage and clonality in the diagnosis of B lymphoproliferative disorders. A number of PCR-based techniques have been developed to overcome the disadvantages of Southern blotting, the standard technique in detecting IgH gene rearrangement. Using an established seminested PCR technique with consensus primers to the V and J regions of the IgH gene, we analysed DNA prepared from peripheral blood and/or bone marrow specimens from 30 cases of known B cell malignancies (16 chronic lymphocytic leukemia, 11 acute lymphoblastic leukemia and 3 Non-Hodgkin Lymphoma), 3 cases of T lymphoproliferative disease and 3 cases of reactive lymphocytosis diagnosed in Hospital UKM to detect rearranged IgH gene. We found that monoclonality as represented by the presence of rearranged IgH gene were demonstrated in all the 30 cases. The PCR findings showed 100% concordance with the Southern blot analysis results which also showed rearranged IgH bands in all the 30 cases. We also found that none of the cases of T lymphoproliferative diseases and reactive lymphocytosis showed presence of rearranged IgH band, suggesting that the amplification using the IgH primers is lineage-specific. In conclusion, we find the PCR a useful method to detect IgH gene rearrangement in peripheral blood and bone marrow specimen. Since the PCR results are comparable to that of the Southern blotting in demonstrating B cell monoclonality and owing to its many advantages we feel that it can replace the Southern blot technique for the diagnosis of B cell malignancies.

Fulltext Juvenile myelomonocytic leukaemia: a case series

Azma RZ, Zarina AL, Hamidah A, Jamal R, Sharifah NA, Ainoon O, et al.

Malays J Pathol, 2009 Dec;31(2):121-8.

PMID: 20514855 MyJurnal

Juvenile myelomonocytic leukaemia (JMML), previously known as juvenile chronic myeloid leukaemia (JCML) is a rare, myelodysplastic - myeloproliferative disease typically presenting in early childhood. This disorder is difficult to distinguish from other myeloproliferative syndrome such as chronic myeloid leukaemia (CML) because of the similarities in their clinical and bone marrow findings. However, because of its unique biological characteristics such as absolute monocytosis with dysplasia, absence of Philadelphia chromosome or BCR-ABL fusion protein, hypergammaglobulinaemia and raised fetal haemoglobin level, this disorder does not satisfy the criteria for inclusion in the CML or chronic myelomonocytic leukaemia (CMML) group, as seen in adult patients. We describe a series of three patients with JMML, who had almost similar clinical and laboratory findings, and discuss the difficulty in the classification and treatment of the disease.

Fulltext Minimal residual disease status in childhood acute lymphoblastic leukaemias by flow cytometry and their clinical and haematological features

Azma, R.Z., Zarina, A.L., Hamidah, A., Cheong, SK, Jamal, R., Hamidah, N.H.

Medicine & Health, 2010;5(1):22-33.

MyJurnal

Residual disease in patients with acute leukaemia indicates unfavorable prognosis. The evaluation of remission using flow cytometry allows a better estimation of minimal residual disease (MRD) after induction chemotherapy in childhood acute lymphoblastic leukaemia (ALL) cases. Patients in morphological marrow remission with presence of blast cells of less than 5%, may still have up to 1010 leukaemic cells. However with flow cytometric analysis, lower levels of the residual leukaemic cells (1 in 104 cells) can be detected and it can be used as a tool to predict relapse. This study compared the presenting clinical and haematological features of children with ALL and their residual disease status determined by flow cytometry. Analysis of their MRD status following remission-induction chemotherapy were done at day-28, week-12 and week-20. The cases were also followed up to five years, to determine their survival status. Their residual disease status by flow cytometric immunophenotyping was also compared with their bone marrow findings morphologically. Thirty-eight cases of precursor B-ALL in pediatric patients from UKM Medical Centre (UKMMC) were analyzed. There was no significant correlation between demographic, clinical and haematological features with MRD status at day-28. However, there was a significant correlation between MRD status by flow cytometry and by morphological marrow examination at week-12. Three cases showed persistent MRD findings until week-20 where two of the cases relapsed and died subsequently. Twenty four patients were still alive after five years of follow up.

Exploring the beliefs of caregivers about the caregiving experiences of children with acute lymphoblastic leukemia in Malaysia

Chong ASS, Mahadir A, Hamidah A, Rizuana IH, Afifi L, Chan CMH

Belitung Nurs J, 2022;8(3):204-212.

PMID: 37547110 DOI: 10.33546/bnj.2083

BACKGROUND: Cancer is one of the major leading causes of childhood death, and the most common type is acute lymphoblastic leukemia. The survival rate has increased in recent years; however, the long patient trajectory may trigger psychological distress among caregivers as they play an active role in ensuring that the child's basic needs are met. Being in a patient-focused system, the needs of caregivers may be neglected.

OBJECTIVE: This study aimed to explore the caregivers' beliefs on children with acute lymphoblastic leukemia caregiving experiences in order to promote their well-being.

METHODS: Caregivers from thirteen families of children with acute lymphoblastic leukemia participated in this phenomenological study. NVivo 12 was used for the thematic analysis of the data. Consolidated criteria for reporting qualitative research (COREQ) were used in this study.

RESULTS: Overall, five main themes for caregivers' beliefs were identified from their responses: 1) dietary intake with emphasis on its importance in aiding recovery and its potential influence on cancer complications, 2) childcare which emphasized the need to be strong and self-sacrifice, 3) treatment which reflected the use of home or natural remedies and caregivers' negative perception towards chemotherapy, 4) causes of cancer believed to be linked to early childcare choices on food, beverage, and stress imposed on the child, and 5) source of beliefs that included doctors, online platforms, personal encounters and information on food labels.

CONCLUSION: Caregivers' beliefs are varied and nuanced, formed in a multicultural social background of Malaysia. These findings provide knowledge for future supportive cancer care for patients, their caregivers, and the treatment outcome in the Malaysian context. Nurses, who play an essential role between healthcare professionals and patients and/or their caregivers, can be empowered to provide psychological support, early detection of psychological distress, and exploration of caregiver beliefs, given that the number of clinical psychologists in Malaysia is lacking, and there is greater preference for care to be provided by doctors or nurses.

Fulltext Cold agglutinins in low-grade B-cell lymphoma

Fadilah SA, Hamidah AB, Cheong SK

Med J Malaysia, 1999 Sep;54(3):383-5.

PMID: 11045070

The presence of serum cold agglutinin can be the initial presentation of lymphoproliferative diseases. Conditions with persistent cold agglutinins are a spectrum of diseases that vary from benign lymphoproliferation of the "autoimmune-like chronic cold agglutinin disease" to malignant lymphoma. We report a case of a 72-year-old woman who presented with severe anaemia, hepatosplenomegaly and episodes of peripheral haemagglutination precipitated by cold exposure. The haemoglobin was 5.6 g/dL with a cold agglutinin titer of 1:256 at 4 degrees C and 1:8 at room temperature (30 degrees C). The cold agglutinin showed anti-I specificity and kappa light chain restriction. Peripheral blood showed atypical lymphoid cells with a B-cell immunophenotype. Immunoglobulin gene rearrangement study by polymerase chain reaction (PCR) showed an amplified band at 100 bp, consistent with a clonal proliferation of B-lymphocytes. We believe that our patient had cold antibody haemolytic anaemia as the initial presentation of a low-grade non-Hodgkin's lymphoma. The association of cold antibody haemolytic anaemia with low-grade B-cell lymphoma is unusual.

Assessment of P-gp and MRP1 activities using MultiDrugQuant Assay Kit: a preliminary study of correlation between protein expressions and its functional activities in newly diagnosed acute leukaemia patients

Fazlina N, Maha A, Zarina AL, Hamidah A, Zulkifli SZ, Cheong SK, et al.

Malays J Pathol, 2008 Dec;30(2):87-93.

PMID: 19291917

Multidrug resistance (MDR) is believed to be responsible for poor response of patients towards chemotherapy particularly patients with acute myeloid leukemia (AML) and acute lymphoblastic leukemia (ALL). The best-characterized resistance mechanism is the one mediated by permeability-glycoprotein (P-gp) encoded by MDR1 gene, which is responsible for drug efflux. We studied P-gp and multidrug resistance-associated protein 1 (MRP1) expression and functional activities in 43 newly diagnosed acute leukemia cases (19 paediatric ALL cases and 24 adult AML cases). The expression and functional activities were examined using flow cytometry and MultiDrugQuant assay kit (involving calcein AM uptake and efflux). P-gp and MRP1 expression and its functional activities were observed in 68.4% of paediatric ALL. In adult AML cases, all cases expressed MRP1 and its functional activities but only 58.3% were positive for P-gp and its functional activities. We were able to show a significant correlation between the expression of the multidrug resistant protein (P-gp and MRP1) and their functional activity in adult AML and paediatric ALL samples.

Successful treatment of very large congenital infantile fibrosarcoma

Hamidah A, Reena M, Halim ARA, Ibrahim S, Eguchi M, Zarina AL, et al.

Pediatr Int, 2011 Oct;53(5):768-770.

PMID: 21955012 DOI: 10.1111/j.1442-200X.2011.03358.x

Hamidah A, Wong CY, Tamil AM, Zarina LA, Zulkifli ZS, Jamal R

Pediatr Blood Cancer, 2011 Jul 15;57(1):105-9.

PMID: 21465639 DOI: 10.1002/pbc.23125

Health-Related Quality of Life (HRQOL) in pediatric leukemia patients in Malaysia has not been studied before. This was mainly due to a lack of databases on patients in the past. Many patients abandoned treatment or were lost to follow up. With more children now fully compliant and completing treatment nowadays, with higher cure rate, HRQOL has become important for our patients. The purpose of the current study was to determine the HRQOL scores in children with acute leukemia and to compare the scores for those on maintenance chemotherapy with those off-treatment as well as to determine factors which might affect HRQOL.

Prevalence and parental perceptions of complementary and alternative medicine use by children with cancer in a multi-ethnic Southeast Asian population

Hamidah A, Rustam ZA, Tamil AM, Zarina LA, Zulkifli ZS, Jamal R

Pediatr Blood Cancer, 2009 Jan;52(1):70-4.

PMID: 18937312 DOI: 10.1002/pbc.21798

The purpose of the current study was to determine the prevalence of use of complementary and alternative medicine (CAM) by children with cancer and to compare the characteristics of CAM users and CAM nonusers.

Fulltext Growth velocity in transfusion dependent prepubertal thalassemia patients: results from a thalassemia center in Malaysia

Hamidah A, Arini MI, Zarina AL, Zulkifli SZ, Jamal R

Southeast Asian J. Trop. Med. Public Health, 2008 Sep;39(5):900-5.

PMID: 19058587

Growth impairment is commonly seen in children with thalassemia despite regular blood transfusions and desferrioxamine treatments. We investigated the growth velocity of 26 prepubertal patients with beta-thalassemia or HbE-beta thalassemia who were transfusion dependent aged between 2 and 13 years. The prevalence of impaired growth velocity (ie, growth velocity less than the third percentile) amongst the transfusion dependent prepubertal thalassemics was 57.7% compared to 19.2% in the control group. The mean height velocity of the thalassemics was 11.1% less than controls but this difference was not statistically significant (4.23cm/year vs 4.76cm/year, p = 0.08). The mean serum ferritin level of the thalassemics with a height < 3rd percentile was higher compared to those with a height > 3rd percentile (4,567.0 vs 2,271.0, p = 0.01). Our study showed that there was a high prevalence of impaired growth velocity amongst our transfusion dependent prepubertal thalassemics. This highlights the problem of inadequate chelation therapy, and compliance with chelation therapy amongst our patients. This study emphasizes the importance of monitoring growth parameters and optimal iron chelation therapy in these patients.

X-linked dyskeratosis congenita in Malaysia

Hamidah A, Rashid RA, Jamal R, Zhao M, Kanegane H

Pediatr Blood Cancer, 2008 Feb;50(2):432.

PMID: 17417794

Recurrent gastric inflammatory myofibroblastic tumour in a 13-year-old male

Hamidah A, Khu SY, Thambidorai CR, Muhaizan WM, Zarina AL, Jamal R

Pediatr Surg Int, 2007 Jun;23(6):601-3.

PMID: 17063336

A 13-year-old boy who had epigastric pain and pallor for 2 months and found to have an ulcerative mass in the stomach and underwent partial gastrectomy. A diagnosis of inflammatory myofibroblastic tumour (IMT) of the stomach was made on histological examination. Three years later, recurrence in the stomach, with invasion into the pancreas and hilum of the spleen was noted and was managed by wide wedge resection of the stomach, distal pancreatectomy and splenectomy. The patient has been recurrence-free for the past 2 years. Gastric IMT is an uncommon tumour in children with unpredictable prognosis.

Fulltext Peginterferon alfa-2b and ribavirin in thalassaemia/chronic hepatitis C virus-co-infected non-responder to standard interferon-based

Hamidah A, Thambidorai CR, Jamal R

Med J Malaysia, 2005 Oct;60(4):517-9.

PMID: 16570722

We describe a patient with HbE-beta thalassaemia and chronic hepatitis C virus infection (genotype 1a) who was treated successfully with peginterferon alfa-2b and ribavirin, following failure to respond to standard interferon and ribavirin therapy. She had sustained virological response for nearly 24 months after completing peginterferon alfa-2b and ribavirin therapy. Transfusion requirements were significantly increased during combination therapy due to ribavirin-induced haemolysis. The adverse effects of interferon were well tolerated. Combination therapy with peginterferon alfa-2b and ribavirin maybe a feasible treatment option for a subset of thalassaemia/HCV infected non-responders to standard interferon-based therapy.

Prolonged remission after splenectomy for refractory Evans syndrome--a case report and literature review

Hamidah A, Thambidorai CR, Jamal R

Southeast Asian J. Trop. Med. Public Health, 2005 May;36(3):762-4.

PMID: 16124452

We describe a patient with Evans syndrome (autoimmune hemolytic anemia and autoimmune thrombocytopenia) who was refractory to steroids and intravenous immunoglobulin. She responded to splenectomy and has remained in clinical remission for 3 years. In the majority of cases, splenectomy rarely induces a durable remission but it may be beneficial in a small group of patients, hence should be considered as alternative therapy in the management of these patients.

Fulltext Short stature and truncal shortening in transfusion dependent thalassemia patients: results from a thalassemia center in Malaysia

Hamidah A, Rahmah R, Azmi T, Aziz J, Jamal R

Southeast Asian J. Trop. Med. Public Health, 2001 Sep;32(3):625-30.

PMID: 11944728

One of the major complications in patients with transfusion dependent thalassemia is growth impairment secondary to iron overload. We studied the growth status in 66 patients with beta-thalassemia major and HbE-beta thalassemia who were transfusion dependent, aged from 2 to 24 years, and 66 controls matched for sex and age. The prevalence of short stature in transfusion-dependent thalassemics was 54.5% compared to 4.5% in control group (p<0.001). Short stature was more prevalent in those above the age of 10 years in this study group (83.3% vs 16.7%). Transfusion dependent thalassemics with short stature were found to have significantly lower mean standing height standard deviation scores (SDS), sitting height SDS and subischial leg length SDS values (p<0.001). There was also a significant difference between the mean sitting height SDS and the mean subischial leg length SDS in our thalassemics with short stature, suggesting that the short stature was due to disproportionate truncal shortening. Serum ferritin levels were significantly higher in transfusion dependent thalassemics who were short compared to those who were of normal height (p = 0.002). However, the mean pre-transfusion hemoglobin levels did not differ significantly between patients with short stature and those with normal height (p = 0.216). The prevalence of short stature also did not differ significantly between those with beta-thalassemia major and those with HbE-beta thalassemia (p = 0.32). This study highlighted the importance of providing optimal treatment in these patients, including monitoring of growth parameters and optimizing iron chelation therapy.

Fulltext Treatment of chronic hepatitis C virus infection with interferon alfa and ribavirin: sustained response in two patients with transfusion dependent thalassaemia

Hamidah A, Yong JF, Zulkifli HI, Jamal R

Med J Malaysia, 2002 Sep;57(3):353-6.

PMID: 12440276

We describe two cases of transfusion dependent thalassaemics with chronic hepatitis C virus infection whom were treated successfully with interferon and ribavirin, following failure of response or relapse after an initial response to interferon monotherapy. They had sustained virological response for more than twelve months after completing therapy. Transfusion requirements were significantly increased during the combination therapy, probably due to ribavirin-induced haemolysis. Serum ferritin level decreased significantly during the treatment. Combination therapy with interferon alfa and ribavirin may be a feasible treatment option for some nonresponders to prior interferon monotherapy.

Fulltext Parental reports of behavioural outcome among paediatric leukaemia survivors in Malaysia: a single institution experience

Hamidah A, Sham Marina M, Tamil AM, Loh CK, Zarina LA, Jamal R, et al.

Trop Med Int Health, 2014 Oct;19(10):1177-84.

PMID: 25047756 DOI: 10.1111/tmi.12358

To determine the behavioural impact of chemotherapy in survivors of acute lymphoblastic leukaemia (ALL) treated with chemotherapy only and to identify treatment-related or sociodemography-related factors that might be associated with behavioural outcome.

Fulltext Cefepime plus amikacin as an initial empirical therapy of febrile neutropenia in paediatric cancer patients

Hamidah A, Lim YS, Zulkifli SZ, Zarina AL, Nordiah AJ, Jamal R

Singapore Med J, 2007 Jul;48(7):615-9.

PMID: 17609821

We evaluated the efficacy of cefepime in association with amikacin in the initial empirical therapy of febrile neutropenic children.

Fulltext Piperacillin-tazobactam plus amikacin as an initial empirical therapy of febrile neutropenia in paediatric cancer patients

Hamidah A, Rizal AM, Nordiah AJ, Jamal R

Singapore Med J, 2008 Jan;49(1):26-30.

PMID: 18204765

We evaluated piperacillin-tazobactam in association with amikacin in the initial empirical therapy of febrile neutropenic children.

Fulltext Thymoma: a case report and review of the literature

Hamidah, A., Poulsaeman, V., Suria, A.A., Zarina, A.L., Zulfiqar, M.A., Jamal, R.

Medicine & Health, 2010;5(1):49-54.

MyJurnal

Thymomas comprise about 1% of all mediastinal tumours and are rare in children. Typically, these tumours are aggressive, with a poor outcome. The current treatment of invasive thymoma is often multidisciplinary. We report a 16-year-old boy with invasive thymoma who was successfully treated with systemic chemotherapy, surgical resection and irradiation. The patient has been in continuous remission for 6 years without radiographic evidence of tumour recurrence.

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