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Severe thalassaemia intermedia: clinical problems in the absence of hypertransfusion

Mohamed N, Jackson N

Blood Rev, 1998 Sep;12(3):163-70.
PMID: 9745886

In many of the parts of the world where thalassaemia is common, the blood supply is inadequate or unsafe, and desferrioxamine is too expensive for routine use. We classify some patients as having 'severe thalassaemia intermedia', i.e. those with moderately severe thalassaemia who can survive without regular transfusions, but who are at risk of many complications which are reviewed here. These include bone deformity and fractures, extramedullary haemopoietic tumours, leg ulcers, autoimmune haemolysis and, especially after splenectomy, thromboembolism and infection. An increase in the quality and safety of the blood supply, and a cheaper and/or oral iron chelator, would enable more of these patients to be treated as thalassaemia major and have improved survival and quality of life.
Sideroblastic anemia recurring during two pregnancies

Jackson N, Hamizah I

Int J Hematol, 1996 Dec;65(1):85-8.
PMID: 8990629

A 25-year-old lady presented with a severe normocytic anemia (Hb 5.3 g/dl) and a sideroblastic marrow at the end of her first pregnancy. Six months into the puerperium, after the transfusion of a total of 8 units of red cells, there was apparent spontaneous improvement and then she was lost to follow-up. After a second pregnancy without clinical problems, she presented during a third pregnancy, at the age of 30 years, with similar hematological findings. Twenty-two months later she was well with a normal blood count. One possible reason for relapse in pregnancy is the increased demand for pyridoxine that occurs, but only one other case of sideroblastic anemia relapsing during pregnancies has been reported.
Markers of ineffective erythropoiesis in non-transfusion dependent β-thalassaemia

Khairullah S, Jackson N

Med J Malaysia, 2021 Jan;76(1):41-45.
PMID: 33510107

Non-transfused β-thalassaemia patients develop complications related to unsuppressed ineffective erythropoiesis (IE). Serum markers of IE would be useful for risk stratification and monitoring treatment. We studied β- thalassaemia trait (β-TT) and non-transfusion-dependent β- thalassaemia (β-NTDT) patients. Serum erythropoietin (EPO) and soluble transferrin receptor (sTfR) were correlated against markers of clinical severity (haemoglobin, LDH, retics, bilirubin, spleen size) and iron overload (ferritin, hepcidin, and MRI-T2* in NTDT patients). Eleven β-NTDT and nine β-TT subjects were studied. β- NTDT patients had significantly higher markers of haemolysis and iron overload. In β-NTDT, liver iron ranged from mild to severe, but no cardiac loading was seen. EPO and sTfR were higher in patients with β-NTDT than β-TT, and correlated significantly with each other (ρ=0.630, p=0.003). Both markers were negatively correlated with haemoglobin (sTfR ρ=-0.540, p=0.014; EPO ρ=-0.807, p<0.001, and positively correlated with spleen size (sTfR ρ=0.783, p<0.001; EPO ρ=0.654, p=0.002) and markers of iron overload. There was a strong correlation between ferritin and hepcidin (ρ=0.720, p<0.001), and a relatively lower increment of hepcidin for the degree of iron overload in β- NTDT compared to β-TT. EPO and sTfR appear to be reliable markers of erythropoiesis in non-transfused β-thalassaemia and correlate well with markers of disease severity. Their role in managing patients, predicting complications, and monitoring response to treatments aimed at reducing IE should be explored.
Fulltext M3-variant of acute promyelocytic leukaemia. A case report in a Malay boy

Jazilah W, Ariffin W, Jackson N

Med J Malaysia, 1995 Mar;50(1):105-7.
PMID: 7752961

The variant form of acute promyelocytic leukaemia (AML-M3) possesses its own characteristic morphology, although usually a few of the cells may have cytoplasmic features of typical AML-M3. In contrast to typical AML-M3, this M3-variant form commonly presents with hyperleucocytosis. As in typical AML-M3, disseminated intravascular coagulopathy (DIVC) occurs in the M3-variant. A boy with this morphological variant of AML-M3 is described. Uncharacteristically, his presenting white blood count was low, and DIVC was present before treatment was started. Six days into intensive chemotherapy his coagulopathy worsened and he subsequently died of intracranial haemorrhage. An alternative approach to the treatment of AML-M3, with the use of retinoids, is discussed.
Retinopathy in acute leukaemia at initial diagnosis: correlation of fundus lesions and haematological parameters

Reddy SC, Jackson N

Acta Ophthalmol Scand, 2004 Feb;82(1):81-5.
PMID: 14738490

PURPOSE: To determine the prevalence of retinal changes in newly diagnosed acute leukaemia patients, and to establish the relationship between retinal lesions and haematological parameters in these patients.
METHODS: A total of 127 patients with acute leukaemia (myeloid and lymphoid), of both genders, aged between 13 and 77 years, were examined by an ophthalmologist for retinal changes using direct/indirect ophthalmoscopy within 2 days of diagnosis before starting chemotherapy.
RESULTS: Retinal lesions were seen in 62 cases (49%), with intraretinal haemorrhages being the most common lesion (42%). A high white blood cell count was significantly associated with intraretinal haemorrhages (p = 0.04) and white-centred haemorrhages (p = 0.001), while a low platelet count was significantly associated with intraretinal haemorrhages (p = 0.03) in acute myeloid leukaemia patients.
CONCLUSIONS: A high white blood cell count may be considered as important as a low platelet count in the pathogenesis of leukaemic retinopathy.
Hydroxyurea treatment for chronic myeloid leukaemia during pregnancy

Jackson N, Shukri A, Ali K

Br J Haematol, 1993 Sep;85(1):203-4.
PMID: 8251394

A patient being treated for chronic myeloid leukaemia with hydroxyurea became pregnant. Despite an increase in the dose of hydroxyurea (to 3 g per day) during the pregnancy, her white blood cell count could only be controlled at about 150 x 10(9)/l. A healthy baby girl was born at 37 weeks with normal blood counts and no evidence of congenital abnormality. There are now five reports of the use of hydroxyurea in pregnancy, and where leukapheresis is not available it may be the treatment of choice.
Transfusion medicine knowledge among clinicians at a teaching hospital in Malaysia

Poh KY, Jackson N

Malays J Pathol, 2023 Aug;45(2):187-194.
PMID: 37658528

INTRODUCTION: Inappropriate use of blood and blood products has been well reported from many countries including Malaysia and may be due to a deficit of transfusion medicine (TM) knowledge. This study is aimed to assess TM knowledge among clinicians in a tertiary hospital.
MATERIALS AND METHODS: The validated exam developed by the BEST collaborative group was used to assess TM knowledge of doctors, from junior residents up to senior specialists. Scores of 42%, 62%, and 82%, corresponding to basic, intermediate, and expert levels of knowledge, respectively. Convenience sampling was done from eight blood-using departments at University Malaya Medical Centre. The Kruskal-Wallis test was used to compare the candidates' exam scores between different variables.
RESULTS: A total of 184 doctors were assessed. The overall mean score was 40.1% (SD 12.7%). The most senior doctors had a significantly lower mean score compared with resident trainees and specialists. Doctors from haematology, anesthesiology, and internal medicine had significantly higher scores (51%, 47.4%, and 46.4% respectively, p<0.05). No correlations were found between the exam scores and the self-reported amount, or quality of prior TM teaching, nor with the year of postgraduate training. Participants did poorly on questions related to transfusion reactions, especially the question on transfusion-related acute lung injury.
CONCLUSION: Inadequate transfusion medicine knowledge was found across all the departments and levels of appointment. It is concerning that the most senior decision-making doctors had especially poor knowledge. TM training is needed by all residents, and regular updates should be given to established specialists.
Immunophenotyping pediatric leukemias in Kelantan, Malaysia

Menon BS, Dasgupta A, Jackson N

Pediatr Hematol Oncol, 1998 Mar-Apr;15(2):175-8.
PMID: 9592844

This study reviewed the immunophenotyping results of children with acute leukemia in Kelantan, Malaysia. In the 3.5-year period (January 1994 to June 1997), 45 cases were identified. All children were under the age of 12 years and the predominant ethnic group was Malay. Thirty-six cases (80%) were acute lymphoblastic leukemia (ALL) and 9 cases (20%) were acute myeloblastic leukemia (AML). Of the ALL cases, 3% were of B-cell and 22% of T-cell origin, and 96% of the B-lineage ALL were CD10 positive. All the AML cases expressed CD33 and 78% were positive for CD13. The incidence of mixed-lineage leukemias was 13.8% for My+ ALL and 11.1% for Ly+ AML.
Fulltext Autosomal dominant thrombocytopenia with microthrombocytes: a family study

Jackson N, Mohammad S, Zainal N, Jamaluddin N, Hishamuddin M

Med J Malaysia, 1995 Dec;50(4):421-4.
PMID: 8668069

A family demonstrating autosomal dominant thrombocytopenia is described. A 28-year-old Malay housewife was found to have a platelet count of 40 x 10(9)/l with a low mean platelet volume (6.8 fl) while being investigated prior to ovarian cystectomy. The bone marrow was consistent with immune thrombocytopenia but she failed to respond to appropriate therapy. Five siblings, one parent and one nephew have easy bruising and platelet counts of 39-82 x 10(9)/l. Platelet aggregation studies excluded a major functional defect. Survival of homologous platelets in the circulation was normal. Familial thrombocytopenias are rare but important to differentiate from the common acquired thrombocytopenias in order to spare the patient unnecessary treatments.
Fulltext Adult idiopathic thrombocytopenic purpura without the option of splenectomy

Jackson N, Mustapha M, Baba AA

Med J Malaysia, 1995 Sep;50(3):250-5.
PMID: 8926904

In this part of Malaysia, consent of splenectomy is virtually unobtainable, so we studied the outcome of ITP without this treatment option. Thirty-two adult patients were seen, but 7 defaulted before therapy evaluation. Of the remaining 25, 17 achieved a complete remission with prednisolone, but in only 8 was this prolonged. Twelve patients, who failed to respond to prednisolone or who required > 15 mg/day as maintenance, were offered splenectomy, but all fused. Of these 12: one has died from an intracranial haemorrhage; three others have defaulted while on no treatment with platelet counts of < 16 x 10(9)/1; one has had a baby who died from intracranial bleeding. The other seven patients have platelet counts ranging from 4 - 202 x 10(9)/1 with moderate bleeding on doses of prednisolone of 0-60 mg/day: long-term corticosteroid side-effect are evident in all but one of them. This study demonstrates that ITP patients who refuse splenectomy have a high morbidity.
Reference ranges for D-dimer levels in Malaysian women in the three trimesters of pregnancy

Mohmad Sallih N, Subbiah I, Ali A, Jackson N

Malays J Pathol, 2019 Apr;41(1):7-13.
PMID: 31025632

INTRODUCTION: Plasma D-dimer levels rise progressively during pregnancy, so one cannot apply normal reference ranges, or the usual cut-off value (500ng/mL), for the exclusion of venous thromboembolism (VTE), in pregnant women. This study was carried out in pregnant Malaysian women in order to build applicable reference ranges for D-dimer.
MATERIALS AND METHODS: A cross-sectional study was conducted to measure D-dimer in healthy pregnant women, and a non-pregnant control group, using the quantitative HaemosIL D-dimer HS500 assay. Reference ranges were derived using CLSI 'Robust' methods, and differences between group medians were tested using the Kruskal-Wallis and Mann-Whitney U tests.
RESULTS: Plasma D-dimer levels were measured in 92 pregnant women (distributed across the three trimesters)and 31 control women. The medians (and reference ranges) in ng/mL were: control 265 (<799); first trimester 481 (<1070); second trimester 1073 (357-1748); 3rd trimester 1533 (771-2410). There were significant differences between the D-dimer levels of each group and each of the other groups (P<0.001).
CONCLUSIONS: Reference ranges for D-dimer in pregnant Malaysian women have been establised by this study. Whether these ranges can be used to determine cut-off levels for the exclusion of VTE at different stages of pregnancy is doubtful, as the levels rise continuously through pregnancy, and some very high outlying values occur in apparently normal near-term pregnancy.
Retinal findings in adult leukaemia: correlation with leukocytosis

Jackson N, Reddy SC, Hishamuddin M, Low HC

Clin Lab Haematol, 1996 Jun;18(2):105-9.
PMID: 8866143

The associations between retinal findings and haematological parameters in acute leukaemia are controversial. Sixty-three newly-diagnosed acute leukaemia patients, aged 12-77 years, were studied prospectively for the presence of intra-retinal haemorrhages (IRH), white-centred haemorrhages (WCH), cotton wool spots (CWS) and macular haemorrhages (MH), Thirty-three patients (52.4%) showed at least one retinal abnormality. The prevalence of individual findings was: IRH (30 cases), WCH (20 cases), CWS (5 cases), MH (11 cases). In contrast to previous studies, there was no association between any of these retinal findings and the haemoglobin level or the platelet count. There was a higher median WBC in patients with IRH (68 x 10(9)/l) than in those without IRH (15.4 x 10(9)/l), P = 0.037. When the acute myeloblastic leukaemia cases were considered separately, an association was also found between higher WBC and the presence of WCH and CWS. There was no association between retinal findings and FAB type in the AML cases. We conclude that a high WBC may be at least as important as anaemia and thrombocytopenia in the pathogenesis of the retinopathy of acute leukaemia.
Lymphoblastic lymphoma/leukaemia presenting as perichondritis of the pinna

Indudharan R, Arni T, Myint KK, Jackson N

J Laryngol Otol, 1998 Jun;112(6):592-4.
PMID: 9764308

Extra-nodal non-Hodgkin's lymphoma (NHL) of the pinna has only been reported once in a patient with immunodeficiency. We report an unusual case of lymphoblastic lymphoma in a patient without any immunodeficiency, presenting as an inflammatory lesion of the pinna, which illustrates the need to biopsy any non-healing lesion as soon as possible to ensure that such a treatable malignancy is diagnosed at an early stage.
Ocular involvement in leukemia--a study of 288 cases

Reddy SC, Jackson N, Menon BS

Ophthalmologica, 2003 Nov-Dec;217(6):441-5.
PMID: 14573980

Two hundred and eighty-eight newly diagnosed cases of leukemia (164 males and 124 females, 167 adults and 121 children, 245 acute and 43 chronic, 151 myeloid and 137 lymphoid), aged between 6 weeks and 78 years, were examined for eye changes in the oncology wards within 2 days of diagnosis before starting chemotherapy. Ocular lesions were present in 102 patients (35.4%)--retinal vascular changes in 91 (31.6%); infiltration of ocular tissues in 5 (1.7%), and neuro-ophthalmic signs in 6 (2.1%) cases. Some of the patients had more than one ocular lesion in one or both eyes. The eye changes were seen more often in adults (49.1%) than in children (16.5%), and in myeloid leukemia (41.0%) than in lymphoid leukemia (29.2%). Eye symptoms were present in 29 patients (10%) at initial diagnosis. Since ocular lesions were detected in many asymptomatic leukemia patients, eye examination should be included as a part of routine evaluation at initial diagnosis in these patients.
Tetraploid/near-tetraploid acute promyelocytic leukaemia with double (15;17) translocation

Tay Za K, Jackson N, Chin EFM

Malays J Pathol, 2020 Apr;42(1):127-130.
PMID: 32342942

A 57-year-old man presented with intermittent fever and bleeding following dental surgery. Peripheral smear and bone marrow aspirate exhibited unusually large and bizarre-looking abnormal cells which were found to be myeloblasts with aberrant CD56 and CD2 expression on immunophenotyping. Fluorescence in situ hybridization analysis revealed an extra RARA gene rearrangement. This finding correlated well with a near-tetraploid karyotype with double t(15;17)(q22;q21). Bcr-3 type PML/ RARA copies were identified in reverse transcriptase-polymerase chain reaction. The diagnosis of near-tetraploid acute promyelocytic leukaemia (APML) was established. The patient was treated with all-trans retinoic acid and idarubicin and six weeks later achieved complete remission. Tetraploid/ near-tetraploid APML is exceedingly rare. It is a distinct cytogenetic subgroup with unique clinical and biological features as highlighted by atypical morphology, frequent CD2 expression and association with the bcr-3 type PML/RARA fusion transcripts. Early recognition of this rare entity is essential for timely and appropriate treatment.
Fulltext Puerperal acquired factor VIII inhibitor causing a von Willebrand-like syndrome in a patient with anti-DNA antibodies

Jackson N, Hashim ZA, Zainal NA, Jamaluddin N

Singapore Med J, 1995 Apr;36(2):230-1.
PMID: 7676276

A 30-year-old Malay lady, with no previous or family history of bleeding, presented with severe gum bleeding 25 days post-partum. The factor VIII:c was 0.03 iu/ml with evidence of a slow-acting factor VIII inhibitor. Von Willebrand factor antigen (VWF:age) varied from less that 0.05 to 0.17 iu/ml, and there was absent ristocetin-induced platelet aggregation. Anti-nuclear and anti-DNA antibodies were present, but there were no other features of systemic lupus erythematosus. There was some clinical response to cryoprecipitate and tranexamic acid, and slight improvement with corticosteroid. Fifteen months later, the patient has no active bleeding problem, and her VWF-ag is increasing spontaneously. However, factor VIII:c is less than 0.01 iu/ml and her factor VIII inhibitor titre is still > 20 Bethesda units/ml.
Fulltext Acute myocardial infarction survival rate and complications after streptokinase therapy in Hospital Universiti Sains Malaysia, Kelantan--a comparative study

Hishamuddin HM, Azmi NN, Jackson N

Singapore Med J, 1993 Aug;34(4):316-8.
PMID: 8266202

Thrombolytic therapy is a well-established therapy in acute myocardial infarction (AMI), reducing mortality and infarct size. This study is a retrospective analysis of survival and complications after the use of streptokinase at Hospital Universiti Sains Malaysia. Streptokinase was first used here in March 1990. Between then and February 1992, 126 patients were admitted to the Coronary Care Unit. Thirty-two patients who fulfilled our criteria for thrombolytic treatment were given an hour intravenous infusion of 1.5 MU streptokinase, and started on aspirin. A control group of 64 patients selected from before March 1990, and matched for age, sex and site of infarct, was given standard therapy. The survival at 4 weeks post-AMI was 91% in the streptokinase therapy group and 91% in both groups (p > 0.05). The complications encountered were reperfusion arrhythmias (2 patients), hypotension(1), maculopapular rash(1) and gum bleeding(1). None of these complications were statistically increased when compared to the control group and none resulted in the death of a patient. We conclude that streptokinase therapy can be given safely in a rural Malaysian setting. Our survival and complication rates are comparable with other published series.
Why is acute leukemia more common in males? A possible sex-determined risk linked to the ABO blood group genes

Jackson N, Menon BS, Zarina W, Zawawi N, Naing NN

Ann Hematol, 1999 May;78(5):233-6.
PMID: 10391104

Acute leukemia is more common in males at almost every age, and this fact remains unexplained. A study was carried out in northeast peninsular Malaysia, where the population is predominantly Malay, to examine whether there was a difference in ABO blood group distribution between males and females with acute leukemia (AL). The ABO blood groups of 109 male and 79 female patients with AL (98 ALL, 90 AML) were compared with those of 1019 controls. In the control population, 39.7% were group O. Among males with AL, 39.4% were group O, whereas among females with AL, the proportion was 24.1% (p=0.03). The same trend to a lower proportion of group O among females was seen if the group was divided into adult/pediatric or lymphoblastic/myeloblastic groups, though these differences were not statistically significant. If these findings can be confirmed, they suggest the presence of a "sex-responsive" gene near to the ABO gene locus on chromosome 9, which relatively protects group O women against AL, at least in our population. The existence of such a gene might also partly explain why acute leukemia, and possibly other childhood cancers, are more common in males.
Prognostic significance of retinopathy at presentation in adult acute leukemia

Reddy SC, Quah SH, Low HC, Jackson N

Ann Hematol, 1998 Jan;76(1):15-8.
PMID: 9486919

Retinal changes are common in adult acute leukemia patients at presentation, but their prognostic significance is controversial. A 5-year study has been carried out with newly diagnosed acute leukemia patients aged 12-77 years. Seventy-seven cases (49 AML, 28 ALL) were studied prospectively for the presence of intraretinal hemorrhages (IRH), white-centered hemorrhages, cotton-wool spots, and macular hemorrhages. They were treated according to standard chemotherapy protocols, and then achievement of complete remission (CR) and the duration of overall survival (OS) were compared between the groups with and without these different retinal features. No association was found between the presence of any retinal abnormality and CR induction rate, although there was a trend to a lower CR rate among patients with IRH. The median OS of those with IRH was 72 days, compared with 345 days among those without IRH (p=0.002). A WBC at presentation greater than 50x10(9)/l and age greater than 40 years were also associated with shorter OS (p<0.0001 and p=0.0045, respectively). However, after regression analysis, IRH remained statistically significant as a poor prognostic indicator (p=0.01). We conclude that the presence of IRH is an indicator of poor prognosis in acute leukemia.
Macular haemorrhage in adult acute leukaemia patients at presentation and the risk of subsequent intracranial haemorrhage

Jackson N, Reddy SC, Harun MH, Quah SH, Low HC

Br J Haematol, 1997 Jul;98(1):204-9.
PMID: 9233585

Retinal changes are common in adult acute leukaemia patients at presentation, but whether they correlate with the risk of subsequent intracranial haemorrhage is unknown. A 4-year study has been carried out in 82 newly-diagnosed acute leukaemia patients, aged 12-77 years, who were studied prospectively for the presence of intra-retinal haemorrhages (IRH), white-centred haemorrhages (WCH), cotton-wool spots (CWS) and macular haemorrhages (MH). Groups with and without these features were compared for their risk of intra-cranial haemorrhage (ICH) within the first 30 d following diagnosis. There was no association between the incidence of ICH and the presence of IRH, WCH or CWS. However, 6/13 of those with MH developed ICH, compared to 6/69 of those without MH (relative risk 5.0, CI 95% [2.03-12.33], P=0.003). The only other identifiable risk factor for ICH was the M3 subtype of AML, but if the four cases of M3-AML were discounted from analysis, MH remained a highly significant risk factor for ICH. Patients with MH should be monitored intensively for the development of ICH, and receive priority in the allocation of platelets where these are in short supply.

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