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  1. Abada E
    Malays J Pathol, 2022 Dec;44(3):533-538.
    PMID: 36591722
    Adequate sampling of gross pathology specimens and attention to microscopic details is crucial in establishing the correct diagnosis in a patient, regardless of a benign pre-surgical diagnosis. Adenoma malignum is a rare HPV-negative variant of well-differentiated adenocarcinoma of the endocervix. It is difficult to diagnose in surgical pathology specimens due to its deceptively-benign appearance. This was a case of a 43-year-old woman with a history of menorrhagia and metrorrhagia and radiology interpretation of degenerating uterine fibroids. Pre-operative Pap testing and hysteroscopic dilatation and curettage were non-contributory. Following surgery, microscopic examination of the cervix revealed well-spaced, deeply-invasive, variably-sized glands with irregular outlines/haphazard arrangements, lined by cells showing mild to moderate cytologic atypia. These cells were positive for p53 and CK7 on Immunohistochemistry and Ki-67 showed a high proliferative index. Monoclonal CEA, calretinin, CD10, and P16 were all negative. A diagnosis of adenoma malignum stage pT1b2 was made. The patient was subsequently placed on platinum-based chemotherapy and is doing well 2 years after her diagnosis. Cases such as this, underscore the importance of adequately sampling surgical resection specimens, with careful attention to microscopic details as incidental pathologies may be unearthed which could have significant implications on a patient's clinical outcome.
  2. Md Akhir MKA, Hussin H, Veerakumarasivam A, Choy CS, Abdullah MA, Abd Ghani F
    Malays J Pathol, 2017 Dec;39(3):227-234.
    PMID: 29279584 MyJurnal
    Urothelial carcinoma is a common malignant neoplasm that has a poor prognosis and a high frequency of recurrence and metastasis. Constant disease surveillance with periodic and long term cystoscopy examination is necessary for management of the disease. However, the monitoring and therapy regimen is expensive, incurring a massive burden to patients and the government. Therefore, the development of specific biomarkers for urothelial carcinoma at an early stage and recurrence detection becomes a priority. Homeobox genes are a family of genes that are involved in tumourigenesis. They might be potential prognostic markers for urothelial carcinoma. The study investigated the expression pattern of NANOG which is one of a homeobox gene in different stages and grades of urothelial carcinoma. NANOG expressions were also correlated with patient demographic factors and clinicopathological parameters. The expression of NANOG in 100 formalin-fixed paraffin-embedded urothelial carcinoma tissues was determined by immunohistochemistry. Immunohistochemistry showed positive expression of NANOG in all specimens with detection in the cytoplasm, nuclei and the nuclear membrane of the cancer cells. The immunohistochemical expression of NANOG increased across stages and grades of the tumour. The expression of NANOG was not significantly associated with demographic factors; gender (p = 0.376), race (p = 0.718) and age (p = 0.058) as well as with most of the clinicopathological parameters; pathological stage (p = 0.144), grade (p = 0.625), lymph node involvement (p = 0.174) and distant metastasis (p = 0.228). However, NANOG expression showed significant correlation with tumour invasion (p = 0.019). We concluded that NANOG might be a potential biomarker for early diagnosis of urothelial carcinoma of the bladder.
  3. Wan Ahmad Kammal WS, Azman M, Salleh AA, Md Pauzi SH, Abd Shukor N
    Malays J Pathol, 2020 Aug;42(2):283-286.
    PMID: 32860383
    Oncocytic carcinoma of the salivary gland is an uncommon tumour in the head and neck region. Owing to its rarity, identifying the histopathological features of a malignant tumour can be difficult and challenging. We report a case of a 70-year-old man who presented with a left facial weakness for six months in a background history of left parotid swelling over the past 10 years. Clinical examination revealed a 3x3cm left parotid mass and grade 4 facial nerve palsy. Fine needle aspiration of the mass showed scattered cohesive, monolayered sheets of uniform oncocytic cells. Subsequently, a left total parotidectomy and selective neck dissection were performed. Histological examination showed sheets of small oncocytes with minimal nuclear atypia. Evidence of nerve entrapment, capsular invasion and perivascular permeation were identified in focal areas. Thus, a final diagnosis of oncocytic carcinoma was rendered.
  4. Jalaludin ND, Mohd Dusa N, Hassan MR, Abd Shukor N
    Malays J Pathol, 2017 Dec;39(3):209-216.
    PMID: 29279582 MyJurnal
    Soft tissue tumours are a group of remarkably diverse neoplasms that frequently pose significant diagnostic challenges to general pathologists. This study aimed to compare the agreement of histopathological diagnoses between general pathologists from various referral institutes and the referred soft tissue pathologist in a tertiary centre. The common discrepancies and their causes are also presented here. A retrospective study was conducted on 243 cases of potential soft tissue tumours referred to Hospital Kuala Lumpur, Malaysia over a period of 5 years. Reports by the referring pathologists and the soft tissue pathologist were compared based on tumour classification and tumour behaviour. Overall, there was moderate agreement in soft tissue tumour diagnoses in both tumour classification (weighted κ = 0.423) and tumour behavior (weighted κ = 0.548). The highest agreement of tumour classification was seen in the adipocytic tumours (21/28 cases), Ewing sarcoma (5/7 cases) and smooth-muscle tumours (3/5 cases). The highest rates of discrepancies were the so-called fibrohistiocytic tumours (7/11 cases), vascular tumours (9/15 cases) and undifferentiated/ unclassified sarcomas (19/32 cases). Full agreement for tumour behaviour was seen in 178 cases and there were 21 cases of zero agreement. Liposarcoma, alveolar soft part sarcoma and benign fibrous histiocytoma were the most frequent benign/malignant diagnostic discrepancies. The most common causes of discrepancy were wrong morphological interpretation followed by insufficient immunohistochemical stains performed. In conclusion, review of diagnosis by a pathologist specialized in soft tissue improves the quality of diagnosis in these heterogenous and rare tumours. A good panel of immunohistochemical stains with additional molecular study is crucial in the general hospital laboratories practice.
  5. Hanim BS, Rahim RA, Mohd Salleh MF, Zakaria H, Abd Shukor N
    Malays J Pathol, 2023 Dec;45(3):463-471.
    PMID: 38155387
    INTRODUCTION: Chorioamnionitis is the inflammation of the placenta and is histologically defined as the presence of neutrophilic infiltration into the chorio-amnion membrane with and without involvement of the umbilical cord. Currently, the inflammatory mediators involved in the eliciting of inflammatory response is still largely under investigation. CD47 and CD36 are pro-inflammatory molecules that are still under investigation. The aim of this study was to determine the expressions of CD47 and CD36 in the placenta of mothers with chorioamnionitis.

    MATERIALS AND METHODS: This was a cross-sectional study, involving a total of 100 cases that comprised of acute subchorionitis (stage I, n=20), acute chorioamnionitis (stage II, n=20), acute necrotising chorioamnionitis (stage III, n=20) and non-chorioamnionitis placenta as control (n=40). All tissue blocks were retrieved from the archived pathology record over a period of 4 years. CD36 and CD47 immunohistochemistry were performed on all cases and their expression in various cell types on the placenta were analysed.

    RESULTS: CD36 was expressed only on the foetal vascular endothelial cells. Interestingly, CD47 showed positive staining on the neutrophils and its expression was significantly different between maternal inflammatory response stage II chorioamnionitis (n=13/20, p<0.001) with stage I and stage III chorioamnionitis.

    DISCUSSION: Our study showed CD47 was expressed in the neutrophils and it was associated with poorer perinatal outcomes and it may have a role in the pathogenesis of chorioamnionitis.

  6. Khoo JJ, Alwi RI, Abd-Rahman I
    Malays J Pathol, 2009 Jun;31(1):77-80.
    PMID: 19694319 MyJurnal
    Breast hamartoma is an uncommon poorly recognised benign breast neoplasm. Hamartoma displaying marked smooth muscle components known as myoid hamartoma of the breast is a much rarer entity. We present a case of myoid hamartoma of breast with chondroid differentiation in a 46-year-old woman. The painless breast lump was circumscribed and mammography showed a well-encapsulated large, dense mass with no calcification. Core needle biopsy was reported as fibroadenoma. The lesion was excised. Microscopically, it composed of many groups of mammary glandular components with dense fibrous stroma, adipose tissue and marked groups of smooth muscle fibres. Foci of chondroid differentiation were noted in the lesion. The smooth muscle cells showed strong and diffuse immunoreactivity for vimentin, myogloblin, alpha-smooth muscle actin, desmin and CD34 and failed to express pan-cytokeratin or S100 protein. The ducts lined by epithelial cells were reactive to pan-cytokeratin while the myoepithelial cells were reactive to S100 protein. The various immuno-histochemical staining as well as the cyto-histological changes encountered in myoid hamartomas are discussed with clinical, radiological and pathological correlation to differentiate it from other benign and malignant breast lesions.
  7. Yong WL, Yusof N, Ithnin A, Shuib S, Tumian R, Yousuf R, et al.
    Malays J Pathol, 2020 Dec;42(3):469-476.
    PMID: 33361731
    INTRODUCTION: Mixed phenotype acute leukaemia (MPAL) is a rare entity of acute leukaemia.

    CASE REPORT: Here we report a case of a 39-year-old lady, with an incidental finding of hyperleukocytosis (white blood cells count: 139.2 x 109/L). Her peripheral blood film revealed 36% of blasts and a bone marrow aspiration showed 53% of blasts. Immunophenotyping showed a population of blasts exhibiting positivity of two lineages, myeloid lineage and B-lymphoid lineage with strong positivity of CD34 and terminal deoxynucleotidyl transferase (Tdt). A conventional karyotyping revealed the presence of Philadelphia chromosome. She was diagnosed with MPAL with t(9,22), BCR ABL1, which carried a poor prognosis. She was treated with acute lymphoblastic leukaemia (ALL) chemotherapy protocol coupled with a tyrosine kinase inhibitor and was planned for an allogeneic stem cells transplant.

    CONCLUSION: This MPAL case was diagnosed incidentally in an asymptomatic patient during medical check-up. We highlight this rare case report to raise the awareness about this rare disease. Understanding the pathogenesis of the disease with the underlying genes responsible for triggering the disease, uniform protocols for diagnosis and targeted treatment will help for proper management of these patients.

  8. Shuib S, Saaid NN, Zakaria Z, Ismail J, Abdul Latiff Z
    Malays J Pathol, 2017 Apr;39(1):77-81.
    PMID: 28413209 MyJurnal
    Potocki-Lupski syndrome (PTLS), also known as duplication 17p11.2 syndrome, trisomy 17p11.2 or dup(17)(p11.2p11.2) syndrome, is a developmental disorder and a rare contiguous gene syndrome affecting 1 in 20,000 live births. Among the key features of such patients are autism spectrum disorder, learning disabilities, developmental delay, attention-deficit disorder, infantile hypotonia and cardiovascular abnormalities. Previous studies using microarray identified variations in the size and extent of the duplicated region of chromosome 17p11.2. However, there are a few genes which are considered as candidates for PTLS which include RAI1, SREBF1, DRG2, LLGL1, SHMT1 and ZFP179. In this report, we investigated a case of a 3-year-old girl who has developmental delay. Her chromosome analysis showed a normal karyotype (46,XX). Analysis using array CGH (4X44 K, Agilent USA) identified an ~4.2 Mb de novo duplication in chromosome 17p11.2. The result was confirmed by fluorescence in situ hybridization (FISH) using probes in the critical PTLS region. This report demonstrates the importance of microarray and FISH in the diagnosis of PTLS.
  9. Arumugam K, Abdul Majeed N
    Malays J Pathol, 2011 Jun;33(1):21-4.
    PMID: 21874747 MyJurnal
    We investigated the usefulness of a single value of maternal HbA1c in late pregnancy as a predictor for neonatal hypoglycaemia and secondly, to find the appropriate threshold value. A prospective analysis of the HbA1c concentration between 36 to 38 weeks of gestation in 150 pregnant mothers with either pre-existing or gestational diabetes was performed. At delivery, glucose levels in the cord blood were analysed. Neonatal hypoglycaemia was defined as a blood sugar level of < 2.6 mmol/l. Receiver operator characteristic curve was constructed to evaluate the value of HbA1c concentration in predicting hypoglycaemia. There were 16 foetuses who were hypoglycaemic at delivery. The area under the ROC curve for predicting neonatal hypoglycaemia was 0.997 with a 95% confidence interval of 0.992 to 1, a very good prediction rate. The optimal threshold value for HbA1c in predicting hypoglycaemia in the foetus was 6.8% (51 mmol/mol). HbA1c level in late pregnancy is a good predictor for hypoglycaemia in the newborn.
  10. Zyoud TYT, Abdul Rashid SN, Suppiah S, Mahmud R, Kabeer A, Abd Manaf R, et al.
    Malays J Pathol, 2020 Dec;42(3):423-431.
    PMID: 33361724
    INTRODUCTION: Post-mortem computed tomography (PMCT) provides information that helps in the determination of the cause of death and corpse identification of disaster victims. One of the methods for corpse identification includes assessment of the body stature. There is a lack of post-mortem imaging studies that focus on the anthropometric assessment of corpses. Our aim was to identify the relationship between cadaveric spine length and autopsy length (AL) among and autopsy length (AL) among a Malaysian population and derive a regression formula for the estimation of corpse body height using PMCT.

    MATERIALS AND METHODS: We retrospectively assessed 107 cadavers that had undergone conventional autopsy and PMCT. We made 5 measurements from the PMCT that included cervical length (CL), thoracic length (TL), lumbosacral length (LS), total column length of the spine, excluding the sacrum and coccyx (TCL), and ellipse line measurement of the whole spine, excluding the sacrum and coccyx (EL). We compared these anthropometric PMCT measurements with AL and correlated them using linear regression analysis.

    RESULTS: The results showed a significant linear relationship existed between TL and LS with AL, which was higher in comparison with the other parameters than the rest of the spine parameters. The linear regression formula derived was: 48.163 + 2.458 (TL) + 2.246 (LS).

    CONCLUSIONS: The linear regression formula derived from PMCT spine length parameters particularly thoracic and lumbar spine gave a finer correlation with autopsy body length and can be used for accurate estimation of cadaveric height. To the best of our knowledge, this is the first ever linear regression formula for cadaveric height assessment using only post mortem CT spine length measurements.

  11. Mohamed NA, Mansur FAF, Abdul Rahman N
    Malays J Pathol, 2020 Apr;42(1):107-110.
    PMID: 32342938
    INTRODUCTION: Malaysia declared its intent to eliminate malaria by 2020, with a phased goal of achieving zero local transmission. Nonetheless, Malaysia is highl susceptible to malaria importation due to geographical proximity to high-burden countries e.g. Thailand, Myanmar and high influx of foreign workers and students from Asia and Africa.

    CASE SERIES: We accumulated all malaria cases diagnosed in a tertiary hospital within a period of two years. Three cases were reported, where all of the patients were foreigners with recent travel history to African countries. All of them were infected by P. falciparum, responded to treatment and discharged well.

    DISCUSSION: This case series highlighted the importance of acquiring recent travel history during history taking and having a high index of suspicions on malaria when dealing with feverish patients originated particularly from African countries.

  12. Chua KB, Mustafa B, Abdul Wahab AH, Chem YK, Khairul AH, Kumarasamy V, et al.
    Malays J Pathol, 2011 Jun;33(1):13-20.
    PMID: 21874746
    A prospective study was carried out to evaluate the sensitivity of dengue NS1 antigen-capture ELISA in comparison with dengue virus isolation, conventional RT-PCR and real-time RT-PCR for laboratory confirmation of acute dengue based on single-acute serum samples. Four primary healthcare centres were involved to recruit patients with clinical diagnosis of dengue illness. Patient's demographic, epidemiological and clinical information were collected on a standardized data entry form and 5 ml of venous blood was collected upon consent. In the laboratory, six types of laboratory tests were performed on each of the collected acute serum sample. Of the 558 acute serum samples collected from 558 patients with clinical diagnosis of dengue from mid-August 2006 to March 2009, 174 serum samples were tested positive by the dengue NS1 antigen-capture ELISA, 77 by virus isolation, 92 by RT-PCR and 112 by real-time RT-PCR. A total of 190 serum samples were tested positive by either one or a combination of the four methods whereas, only 59 serum samples were tested positive by all four methods. Thus, based on single-acute serum samples, 190 of the 558 patients (34.1%) were laboratory-confirmed acute dengue. The overall test sensitivity was 91.6%, 40.5%, 48.4% and 58.9% for dengue NS1 antigen-capture ELISA, virus isolation, conventional RT-PCR and real-time RT-PCR respectively. Statistically, dengue NS1 antigen-capture ELISA was the most sensitive and virus isolation was the least sensitive test for the laboratory confirmation of acute dengue based on single-acute serum specimens. Real-time RT-PCR was significantly more sensitive than the conventional RT-PCR.
  13. Chiew SF, Looi LM, Cheah PL, Teoh KH, Chang SW, Abdul Sani SF
    Malays J Pathol, 2023 Dec;45(3):363-374.
    PMID: 38155378
    Epithelial-mesenchymal transition (EMT) is increasingly explored in cancer progression. Considering that triple negative (TN) breast cancer has the poorest survival among molecular subtypes, we investigated 49 TN, 45 luminal and 25 HER2-enriched female breast carcinomas for EMT expression (using E-cadherin and vimentin immunohistochemistry) against lymphovascular and/or lymph node invasion. E-cadherin and vimentin expressions were semi-quantitated for positive- cancer cells (0=0-<1%, 1=1-10%, 2 =11-50%, 3=>50%) and staining intensity (0=negative, 1=weak, 2=moderate, 3=strong), with final score (low=0-4 and high=6-9) derived by multiplying percentage and intensity scores for each marker. Low E-cadherin and/or high vimentin scores defined EMT positivity. Low E-cadherin co-existing with high vimentin defined "complete" (EMT-CV), while low E-cadherin (EMT-C) or high vimentin (EMT-V) occurring independently defined "partial" subsets. 38 (31.9%) cancers expressed EMT, while 59.2 % TN, 13.3% luminal and 12% HER2-enriched cancers expressed EMT (p<0.05). Among the cancers with lymphovascular and/or lymph node invasion, EMT positivity by molecular types were 66.7% TN, 7.4% luminal and 11.8% HER2-enriched (p<0.05). Although EMT-V, associated with stem-cell properties was the dominant TN EMT profile, EMT-CV, a profile linked to vascular metastases, was encountered only in TN. EMT appears important in TN cancer and different EMT profiles may be associated with its aggressive nature.
  14. Arumugam M, Jamil A, Amiseno RA, Rosli N, Abdul Shukor N
    Malays J Pathol, 2020 Aug;42(2):277-281.
    PMID: 32860382
    INTRODUCTION: Merkel cell carcinoma (MCC) is a rare and aggressive malignancy of the skin, with poor clinical outcomes. Typical conditions include a rapidly growing, solitary dome-shaped, violaceous nodule. Several root causes have been identified - sun exposure, age, lighter skin, immunocompromised state, and polyomavirus infection. Wide local excision is the best treatment. The tumour is radiotherapy-responsive. However, the success rate of the treatment with chemotherapy is rather limited. Immunotherapy has shown promising results. Early detection is important to prevent morbidity and mortality.

    CASE REPORT: In this literature work, we reported on a particular case of MCC, as exhibited by an 84-year-old Chinese woman, and discussed the clinical features and management of MCC.

    DISCUSSION: We highlighted that MCC cases have a link to the polyomavirus 5. Patients who were identified with the Polyomavirus 5, and underwent immunotherapy, were seen to depict much better prognosis.

  15. Yap E, Wan Jamaluddin WF, Tumian NR, Mashuri F, Mohammed F, Tan GC, et al.
    Malays J Pathol, 2014 Dec;36(3):201-5.
    PMID: 25500520 MyJurnal
    NK/T cell lymphoma, nasal type is an aggressive and uncommon malignancy. Disease that occurs outside of the aerodigestive tract exhibits an even more aggressive clinical behaviour and does not respond as well to conventional therapy compared to its nasal counterpart. We report such a case of NK/T cell lymphoma, nasal type, that presented as an anterior chest wall mass, arising from the left pectoralis muscle. An interesting feature we wish to highlight is the associated eosinophilia that corresponded to disease activity, exhibiting fluctuations with surgical resection and chemotherapy. To the best of our knowledge this is the third reported case of NK/T cell lymphoma that is associated with peripheral eosinophilia. Our case highlights the role of certain NK cell subsets that play a major role in eosinophilic activation in NK/T lymphomas and calls for more research into further classification of this disease by virtue of its NK cell subsets.
  16. Nyunt WW, Wong YP, Wan Jamaludin WF, Abdul Wahid SF
    Malays J Pathol, 2016 Apr;38(1):55-9.
    PMID: 27126666 MyJurnal
    Non-necrotic epithelioid granulomas have been reported in association with neoplasms including Hodgkin and non-Hodgkin lymphoma. We report a case of diffuse large B cell lymphoma with chronic granulomatous inflammation to highlight awareness of obscure tumour cells within the granuloma, to avoid delay in diagnosis and management of lymphoma. A 39-year-old Malay lady with no past medical history, presented with a 2-month history of progressive worsening of difficulty in breathing, cough, low-grade fever, loss of weight and loss of appetite. Chest X-ray showed an anterior mediastinal mass and computed tomography (CT)-guided biopsy was reported as chronic granulomatous inflammation suggestive of tuberculosis. After 2 months of anti-TB treatment, her symptoms were not relieved. The patient underwent another CT-guided biopsy of the anterior mediastinal mass in another hospital and the histopathology revealed diffuse large B cell lymphoma. The patient was referred for treatment. On histopathological review, the first sample showed noncaseating granulomas engulfing tumour cells and large abnormal lymphoid cells which were CD20 positive and with high Ki-67 proliferative index. The patient was diagnosed with diffuse large B cell lymphoma stage IV B IPSS score 3. She underwent chemotherapy (R-EPOCH) and responded well to treatment.
  17. Wan Jamaludin WF, Mohamad Yusoff F, Ismail NA, Mohd Idris MR, Palaniappan S, Ng CKK, et al.
    Malays J Pathol, 2018 Apr;40(1):61-67.
    PMID: 29704386 MyJurnal
    INTRODUCTION: Immunosuppressive state due to haematological malignancies and chemotherapy may cause disruption to wound healing despite optimum conventional treatment and standard wound dressing. Non-healing wounds are predisposed to infection whereas chemotherapy dose reductions or interruptions are associated with poor survival.

    BACKGROUND: Mononuclear cells contain progenitor cells including haematopoietic and mesenchymal stem cells, endothelial progenitor cells and fibroblasts which facilitate wound healing through cytokines, growth factor secretions, cell-cell interactions and provision of extracellular matrix scaffolding. Clinical applications of autologous mononuclear cells therapy in wound healing in non-malignant patients with critical limb ischaemia have been reported with remarkable outcome.

    METHODS: We report three patients with haematological malignancies undergoing chemotherapy, who received autologous mononuclear cells implantation to treat non-healing wound after optimum conventional wound care. The sources of mononuclear cells (MNC) were from bone marrow (BM), peripheral blood (PB) and mobilised PB cells (mPB-MNC) using granulocyte colony stimulating factor (G-CSF). The cells were directly implanted into wound and below epidermis. Wound sizes and adverse effects from implantation were assessed at regular intervals.

    RESULTS: All patients achieved wound healing within three months following autologous mononuclear cells implantation. No implantation adverse effects were observed.

    CONCLUSIONS: Autologous mononuclear cells therapy is a feasible alternative to conventional wound care to promote complete healing in non-healing wounds compounded by morbid factors such as haematological malignancies, chemotherapy, diabetes mellitus (DM), infections and prolonged immobility.

  18. Nyunt WWT, Abdul Jalil D, Zakariah NA, Abdul Karim N, Mohd Idris MR, Nasuruddin DN, et al.
    Malays J Pathol, 2020 Apr;42(1):143-150.
    PMID: 32342945
    INTRODUCTION: Plasma cell leukaemia (PCL) is a rare variant of multiple myeloma. We report a case of PCL to demonstrate the clonal evolution, resulting in disease relapse after achieving complete remission, and its aggressive nature of the disease, leading to poor clinical outcome.

    CASE REPORT: A 69-year-old man presented with a three-day-history of worsening generalized body weakness, poor oral intake, nausea, significant loss of weight and lower back pain. He was diagnosed as primary PCL, based on hypercalcaemia, renal insufficiency, anaemia, thrombocytopenia, lytic bone lesions, 24% abnormal plasma cells in peripheral blood, immunophenotype of clonal plasma cells which were positive for CD38, CD138 and CD56 markers with kappa light chain restriction, 49% abnormal plasma cells in bone marrow, monoclonal paraprotein (IgG kappa) in serum and urine, and positive IGH rearrangement (Fluorescence in-situ hybridisation, FISH). He achieved complete remission after four cycles of Bortezomib-based therapy. There was a plan for high-dose therapy plus autologous haematopoietic cell transplantation. A month later, the disease relapsed, as evidenced by 94% abnormal plasma cells in his bone marrow aspirate, complex karyotype and abnormal FISH results. He passed away a few days later, from severe septicaemia. Time-to-progression of disease was 1 month and overall survival was 5 months.

    DISCUSSION: This case report illustrates the clonal evolution and aggressive nature of primary PCL with older age at presentation, leading to a shorter duration of remission and overall survival.

  19. Nyunt WWT, Remli R, Abdul Muttlib FA, Leong CF, Masir N, Tumian NR, et al.
    Malays J Pathol, 2017 Dec;39(3):297-303.
    PMID: 29279594 MyJurnal
    POEMS syndrome is the syndrome of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and typical Skin changes. A 65-year-old lady presented with the 2-day-history of inability to walk, 4-month-history of progressive worsening of muscle weakness of both lower limbs and 1-year-history of progressive worsening of bilateral numbness of lower limbs. Nerve conduction study revealed generalized sensorimotor demyelinating polyneuropathy. She was initially treated as chronic inflammatory demyelinating polyradiculoneuropathy with intravenous immunoglobulin (IVIG) and high-dose prednisolone. However, she had no significant neurological improvement despite getting standard therapy. In addition to peripheral neuropathy, the presence of hepatosplenomegaly, skin changes, polycythaemia and thrombocytosis prompted for further investigations. She was diagnosed as POEMS syndrome based on the presence of two mandatory major criteria [polyneuropathy, monoclonal plasma cell proliferative disorder (lambda)], one major criterion (sclerotic bone lesions) and three minor criteria (organomegaly, skin changes and thrombocytosis/polycythaemia). She received treatment with melphalan and prednisolone. She achieved clinical improvement and partial response (haematologic and radiological) after six cycles of therapy. We highlight the awareness of this rare syndrome, for patients presenting with peripheral neuropathy and not responding to its standard therapy, by recognizing other associated clinical manifestations and proceeding further diagnostic work-up.
  20. Hassan N, Dhaliwal JS, Mohd Ibrahim H, Osman R, HIdris SZ, Lee le J, et al.
    Malays J Pathol, 2015 Aug;37(2):83-90.
    PMID: 26277663 MyJurnal
    Soluble HLA (sHLA) are potential tumour markers released in order to counter immune surveillance. sHLA-class II is less known especially in acute lymphoblastic leukaemia (ALL). This study aimed to investigate soluble, surface and allelic expression of HLA Class II (sHLA-DR) in B-cell ALL patients and compare with soluble expression in normal individuals. A sandwich enzyme-linked immunosorbent assay (ELISA) was developed to measure soluble HLA-DRB1 in plasma. Flow cytometric analysis was performed to determine median fluorescence intensity in HLA-DR surface expression. HLA-DNA typing by polymerase chain reaction, sequence specific oligonucleotides, PCRSSO was performed to determine HLA-DRB1 type in ALL samples. Results showed sHLA-DRB1 (mean±SEM) was significantly increased (p=0.001) in plasma of ALL patients (0.260 ±0.057 μg/mL; n=30) compared to healthy controls (0.051 ± 0.007µg/mL; n=31) of Malay ethnicity. However, these levels did not correlate with percentage or median fluorescence intensity of HLA-DR expressed on leukemia blasts (CD19+CD34 ± CD45(lo)HLA-DR+) or in the normal B cell population (CD19+CD34- CD45(hi)HLA-DR+) of patients. No significant difference was observed in gender (male/female) or age (paediatric/adult). Only a trend in reduced sHLA was observed in patients carrying HLA-DR04. These results have to be validated with a larger number of samples.
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