MyMedR

Selective IgA deficiency presenting with hypersplenism

Teh A, Bosco JJ

Br J Clin Pract, 1994 Sep-Oct;48(5):276-7.

A young patient presenting with splenomegaly and hypersplenism was inadvertently found to have selective IgA deficiency. There were no symptoms of immunodeficiency and the patient responded well to splenectomy, with return of blood counts to normal without adverse effects. No other cause for the hypersplenism was found. We postulate selective IgA deficiency as a cause of splenomegaly and hypersplenism.

Matched MeSH terms: Splenomegaly/etiology; Splenomegaly/surgery

Virulence of Malaysian isolates of Orientia tsutsugamushi in mice

Tay ST, Rohani MY, Ho TM, Devi S

Southeast Asian J Trop Med Public Health, 2002 Sep;33(3):565-9.

PMID: 12693592

The pathogenicity of Malaysian isolates of Orientia tsutsugamushi was investigated by a mouse virulence assay. The isolates could be differentiated as low (4 isolates), moderately (3 isolates) and highly virulent (2 isolates) based on the different responses in infected mice. No direct correlation between severity of human scrub typhus infections and virulence of the O. tsutsugamushi in mice was observed. Mice infected with virulent strains of O. tsutsugamushi showed splenomegaly, ascitis accumulation and enlargement of kidneys and livers whereas avirulent O. tsutsugamushi strains were asymptomatic and exhibited ruffled fur for a short period after infection. There was low antibody response in mice infected with isolates of low pathogenicity as compared with those of highly virulent isolates. Upon dissection of the infected mice, enlargement of mouse organs such as spleen, kidney and liver was noted. Presence of rickettsemia in mice was confirmed by the growth of O. tsutsugamushi in the L929 cells when inoculated with blood from infected mice. O. tsutsugamushi was also cultured from the peritoneal exudates of the infected mice. However, DNA of O. tsutsugamushi was only detected in the peritoneal exudates (by PCR) and blood (by cell culture) and not from other tissue samples.

Matched MeSH terms: Splenomegaly/microbiology; Splenomegaly/pathology

Hypoechoic liver in fetuses with trisomy 21

Omar PM, Lim WT, Ting YH, Lao TT, Law KM, Cheung AHK, et al.

J Matern Fetal Neonatal Med, 2019 Oct;32(19):3315-3317.

PMID: 29631451 DOI: 10.1080/14767058.2018.1459556

The association between hypoechoic hepatomegaly in the third trimester and transient abnormal myelopoiesis (TAM) was reported previously in six fetuses with trisomy 21 (T21). We report a series of three cases of T21 in which hypoechoic liver (HL) was found in the second trimester but without evidence of TAM on both hematological and histological examination. We postulate that the hypo-echogenicity may be due to liver congestion secondary to hemodynamic disturbances seen in T21 fetuses. All three cases had negative first trimester Down syndrome screening and one case was detected solely because of the isolated finding of HL. HL per se may be associated with T21 and more positive cases are required to support this association.

Matched MeSH terms: Splenomegaly/complications; Splenomegaly/diagnosis; Splenomegaly/pathology

IDIOPATHIC HYPERSPLENISM IN A CHINESE BOY; BENEFICIAL EFFECT OF SPLENECTOMY

LIE-INJOLUAN EN, PILLAY RP

Med J Malaysia, 1963 Dec;18:95-8.

PMID: 14117288

Matched MeSH terms: Splenomegaly*

Fulltext Splenic lymphoma with hypersplenism--a case report

Ng SC, Kuperan P, Jayalakshmi P, Chua CT

Singapore Med J, 1990 Feb;31(1):80-2.

PMID: 2185555

A 47-year old man had hypersplenism from massive splenomegaly, the cause of which was undetermined for 2 years. He was initially asymptomatic though there was mild pancytopenia. However, 18 months after presentation he manifested both clinical and haematological deterioration, almost succumbing to sepsis. Splenectomy finally provided a definite diagnosis of follicular lymphoma and also restored his blood counts to within normal range.

Matched MeSH terms: Splenomegaly/complications*

Splenomegaly and blood pressure in an Orang Asli community in West Malaysia

Burns-Cox CJ

Am Heart J, 1970 Nov;80(5):718-9.

PMID: 5474109

Matched MeSH terms: Splenomegaly/epidemiology*

Fulltext Combined needlescopic cholecystectomy and laparoscopic splenectomy for the treatment of thalassaemic splenomegaly and cholelithiasis

Jasmi AY, Thambidorai CR, Khairussalleh J

Med J Malaysia, 2003 Aug;58(3):443-5.

PMID: 14750388

Gallstone disease is a common association in patients with haematological splenomegaly. When indicated, simultaneous splenectomy and cholecystectomy should be performed and traditionally this is accomplished by open surgery. We report a 17 year old thalassaemic girl with splenomegaly complicated by gallstone pancreatitis. We treated her with a combination of needlescopic cholecystectomy and laparoscopic splenectomy as well as delivering the huge spleen via a pfannenstiel incision to hide the scar. We believe this technique is an acceptable alternative mainly for rapid delivery of the spleen and to minimize visible scars hence improving cosmesis.

Matched MeSH terms: Splenomegaly/etiology; Splenomegaly/surgery*

Fulltext Favourable response to splenectomy in familial myelodysplastic syndrome

Abdul-Wahab J, Naznin M, Suhaimi A, Amir-Hamzah AR

Singapore Med J, 2007 Jul;48(7):e206-8.

PMID: 17609817

Familial myelodysplastic syndrome occurring at a young age is a very rare childhood haematological malignancy. Two siblings, aged three and 18 years, from a consanguineous marriage, presented with pancytopenia and was subsequently diagnosed to have myelodysplastic syndrome. Both remained clinically stable throughout the illness. Splenectomy appeared to have fully corrected the cytopenia in one of them.

Matched MeSH terms: Splenomegaly/etiology; Splenomegaly/surgery*

Fulltext Malaria endemicity in an Orang Asli community in Pahang, Malaysia

Gurpreet K

Trop Biomed, 2009 Apr;26(1):57-66.

PMID: 19696728 MyJurnal

An epidemiological cross-sectional study was undertaken to determine the endemicity of malaria among the Orang Asli population of Raub, Pahang. Malaria endemicity was measured in terms of the prevalence of parasitaemia and splenomegaly. A total of 520 Orang Asli were examined. The point prevalence of malaria was 24.2% (95% CI 20.7-25.1), with Plasmodium falciparum (67.5%) being the predominant species. Children < 12 years were at least 3.7 times more likely to be parasitaemic compared to those older. The prevalence of malaria among children 2-<10 years was 38.1% (95% CI 31.6-50.0). Spleen rate among children 2-<10 years old was 22.3% (95% CI 17.1-28.3). The average enlarged spleen size was 1.2. These findings classify the study area as being mesoendemic. Malaria control activities among the Orang Asli should focus on protecting vulnerable subgroups like young children. Measuring the level of malaria endemicity at regular intervals is fundamental in evaluating the effectiveness of malaria control programs.

Matched MeSH terms: Splenomegaly/diagnosis; Splenomegaly/ethnology; Splenomegaly/epidemiology*

Essential thrombocytopenia

Haridas G

Journal of the Malaya Branch of the British Medical Association, 1940;4:198-204.

Matched MeSH terms: Splenomegaly

Fulltext Transient abnormal myelopoeisis in newborns with Down syndrome

Zarina AL, Hamidah A, Yong SC, Rohana J, Hamidah NH, Azma RZ, et al.

Malays J Pathol, 2007 Dec;29(2):107-11.

PMID: 19108403 MyJurnal

Transient abnormal myelopoeisis (TAM) is a haematological phenomenon commonly seen in newborns with Down syndrome. Although the majority show spontaneous resolution, this condition should not be dismissed too readily as there have been associated fatalities. Furthermore, even for those who do show spontaneous resolution, a significant percentage will develop acute megakaryoblastic leukaemia within the next few years of life. We report a series of four patients with TAM who presented with hepatosplenomegaly and leucocytosis detected on preliminary investigations.

Matched MeSH terms: Splenomegaly/etiology

Seroepidemiology of malaria: age-specific pattern of Plasmodium falciparum antibody, parasite and spleen rates among children in an endemic area in peninsular Malaysia

Thomas V, Hock SK, Leng YP

Trop Doct, 1981 Oct;11(4):149-54.

PMID: 7027557

A seroepidemiological study was carried out on Orang Asli (Aborigines) children who lead a semi-nomadic life in the deep jungles of Ulu Kelantan, Malaysia. Out of a total of about 190 children below 14 years, 143 were studied. Blood was collected from finger pricks on standard "strip type" filter papers for indirect fluorescent antibody (IFA) tests with Plasmodium falciparum antigen. A positive reaction at 1:10 dilution in infants and young children was considered positive and the reasons are given. The P. falciparum antibody prevalence rate was 84.6% compared to 81.8% spleen and 43.4% parasite rates. Both P. Falciparum and P. vivax were present in children. The age-specific patterns of antibody, spleen and parasite rates were those of a hyperendemic community. There was a positive correlation between antibody and spleen rates up to the age of 9 years. In older children, the antibody rates increased while the spleen and the parasite rates dropped.

Matched MeSH terms: Splenomegaly/etiology

Health status of Orang Asli (aborigine) community in Pos Piah, Sungai Siput, Perak, Malaysia

Norhayati binti Moktar M, Noor Hayati MI, Nor Fariza N, Rohani AK, Halimah AS, Sharom MY, et al.

Southeast Asian J Trop Med Public Health, 1998 Mar;29(1):58-61.

PMID: 9740269

A study of health status of Orang Asli population (based on physical examination findings) was conducted in 4 villages in Pos Piah, Sungai Siput Perak, Malaysia. In all 356 individuals between 4 months-72 years old (178 males and 178 females) participated in this study. Poor general health status, physical and mental handicaps were seen in 7.8%, 0.3% and 0.3% of the population, respectively. About one-fifth of the population had dental caries. Splenomegaly, hepatomegaly and hepatosplenomegaly were among the commonest abnormalities with the occurrence rates of 19.8%, 13.7% and 6.7%, respectively, being detected in the population. About one-fifth of the population showed signs suggestive of protein-energy deficiency; whilst less than 5% showed signs indicative of riboflavin, iodine and iron deficiencies. Vitamin A deficiency was the commonest nutritional deficiency identified in this community with almost 38.4% of them showing signs of the deficiency. The commonest skin infection was scabies.

Matched MeSH terms: Splenomegaly/epidemiology

Fulltext Combined analysis of ZAP-70 and CD38 expression in sudanese patients with B-cell chronic lymphocytic leukemia

Basabaeen AA, Abdelgader EA, BaHashwan OS, Babekir EA, Abdelateif NM, Bamusa SA, et al.

BMC Res Notes, 2019 May 23;12(1):282.

PMID: 31122288 DOI: 10.1186/s13104-019-4319-8

OBJECTIVE: To investigate the ZAP-70 and CD38 expressions and their combined expressions in Sudanese B-CLL patients and their relationships with clinical and hematological characteristics as well as the disease staging at presentation.

RESULTS: In the present cross-sectional descriptive study, analysis of ZAP-70 expression showed that 36/110 (32.7%) patients positively expressed ZAP-70 and insignificant higher presentation in intermediate and at advanced stages as well as no correlation was seen with hematological parameters and clinical features compared with negatively ZAP-70, on the other hand, 41/110 (37.3%) were CD38+ and no significant correlation was shown with the stage at presentation, clinical characteristics (except Splenomegaly, P = 0.02) and hematological parameters. However, in combined expressions of both ZAP-70 and CD38 together, 20/110 (18.2%) were concordantly ZAP-70+/CD38+, 53/110 (48.2%) concordantly ZAP-70-/CD38- and 37/110 (33.6%) either ZAP-70+ or CD38+, and these three groups showed insignificant correlation with clinical (except Splenomegaly, P = 0.03) and hematological parameters, and the stage at presentation. Our data showed the combined analysis of these two markers, lead to classify our patients into three subgroups (either concordant positive, negative or discordant expressions) with statistically insignificant correlation with clinical presentation (except Splenomegaly), hematological parameters and stage at presentation of B-CLL patients.

Matched MeSH terms: Splenomegaly/diagnosis*; Splenomegaly/genetics; Splenomegaly/immunology; Splenomegaly/pathology

Characterising granuloma regression and liver recovery in a murine model of schistosomiasis japonica

Chuah C, Jones MK, McManus DP, Nawaratna SK, Burke ML, Owen HC, et al.

Int J Parasitol, 2016 Apr;46(4):239-52.

PMID: 26812024 DOI: 10.1016/j.ijpara.2015.12.004

For hepatic schistosomiasis the egg-induced granulomatous response and the development of extensive fibrosis are the main pathologies. We used a Schistosoma japonicum-infected mouse model to characterise the multi-cellular pathways associated with the recovery from hepatic fibrosis following clearance of the infection with the anti-schistosomal drug, praziquantel. In the recovering liver splenomegaly, granuloma density and liver fibrosis were all reduced. Inflammatory cell infiltration into the liver was evident, and the numbers of neutrophils, eosinophils and macrophages were significantly decreased. Transcriptomic analysis revealed the up-regulation of fatty acid metabolism genes and the identification of Peroxisome proliferator activated receptor alpha as the upstream regulator of liver recovery. The aryl hydrocarbon receptor signalling pathway which regulates xenobiotic metabolism was also differentially up-regulated. These findings provide a better understanding of the mechanisms associated with the regression of hepatic schistosomiasis.

Matched MeSH terms: Splenomegaly

Fulltext Infantile Myofibromatosis: A Case Report

Hasniah, A.L., Rohana, J., Boo, N.Y., Thambidorai, C.R., Isa, M.R.

International Medical Journal Malaysia, 2007;6(1):1-6.

MyJurnal

Case: A male infant was born at 36th week period of gestation with a birth weight of 1.99kg following an uneventful intrauterine period. At birth, he was noted to have multiple skin nodules. The largest nodule measuring 4.0 x 2.0 x 1.5 cm was at his pre-auricular area. It was initially fleshy and reddish in color. A few days later, it turned black and subsequently the black skin overlying the mass fell off, leaving a firm underlying mass with the appearance of a cauliflower. Other skin lesions, which were firm in consistency, were found on the medial aspect of his right supraorbital ridge, right mid-arm, right mid-thigh, plantar surface of his right big toe and his scalp. They were either skin-colored or reddish in colour, measuring between 1.0 and 2.0 cm in diameter. He also had hepatomegaly and splenomegaly palpable at 5 cm and 3 cm below the subcostal margins, respectively

Matched MeSH terms: Splenomegaly

Neonatal hepatitis among Malaysian infants

Lee, W.S., Boey, C.C.M., Koh, M.T.

Malaysian Journal of Child Health, 1998;10(1):40-45.

MyJurnal

From November 1996 to December 1997, 24 infants with neonatal cholestasis were referred to the Department of Paediatrics, University of Malaya Medical Center, Kuala Lumpur for further investigations. Nineteen had neonatal hepatitis. There was considerable delay in referral of infants with cholestasis; the mean age of referral was 63.7 days. None had a positive family history of neonatal hepatitis. All infant had hepatomegaly and ten had splenomegaly. The stools were slightly pale in thirteen, persistently acholic in three and normally pigmented in three infants. Liver synthetic functions were normal in most of the infants. Cytomegalovirus (CMV) IgM antibodies were positive in seven but none were positive for toxoplasma or rubella. al - antitrypsin deficiency, hypothyroidism, and galactosaemia were excluded in all infants. DISIDA scans were performed in seventeen infants, being non-excretory in eight. Liver biopsies were performed in fifteen infants, showing neonatal hepatitis in fourteen, while histological features of large duct obstruction was seen in one. In majority of infants (eight out of ten) the jaundice disappeared by six months. Two infants had progressive jaundice and liver function impairment.

Matched MeSH terms: Splenomegaly

Fulltext Polycythaemia Vera JAK 2 Mutation in a Patient with Underlying Chronic Obstructive Pulmonary Disease at a Primary Care Setting

Mahmud R, Ariffin F, Shanmuganathan P

Korean J Fam Med, 2020 Jul;41(4):263-266.

PMID: 32512984 DOI: 10.4082/kjfm.18.0161

The presence of erythrocytosis along with the diagnosis of chronic obstructive pulmonary disease (COPD) may veer a primary care clinician in a busy clinic towards attributing the erythrocytosis to hypoxia secondary to COPD; however, this is not always the case. This case highlights the importance of investigation and the significance not excluding a primary cause in COPD patients with erythrocytosis. A 57-year-old male, presenting with chronic cough, was subsequently diagnosed with COPD clinically and confirmed by spirometry. Erythrocytosis was also incidentally noted. The patient did not have any symptoms of polycythemia or hepatosplenomegaly. Therefore, the erythrocytosis was initially thought to be caused by hypoxia secondary to COPD. However, the JAK2 V617F gene mutation was detected and hence the diagnosis of polycythemia vera was made. Although the erythrocytosis was initially attributed secondary to the underlying pulmonary disease, investigations proved it to be primary in origin. This case report highlights the importance of investigating the underlying cause and to confirm the diagnosis of erythrocytosis as primary and secondary polycythemia differ in their management approach. This will avoid inappropriate diagnosis, treatment, and undesirable outcomes.

Matched MeSH terms: Splenomegaly

Fulltext Splenic amyloidosis: a rare cause of spontaneous splenic rupture

Chan RS, Abdul Aziz YF, Chandran P, Ng EK

Singapore Med J, 2011 Nov;52(11):e232-5.

PMID: 22173263

A 62 year-old woman who presented with an atraumatic acute abdomen was discovered to have haemoperitoneum with splenic rupture on urgent computed tomography and was immediately referred for life-saving emergency splenectomy. Histopathological examination revealed secondary splenic amyloidosis. The patient was later found to be suffering from infective endocarditis secondary to her permanent cardiac pacemaker. This report describes a patient who could have suffered from a long-standing infected vegetation on a permanent cardiac pacemaker, which led to splenic amyloidosis and spontaneous splenic rupture.

Matched MeSH terms: Splenomegaly/microbiology

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.

Matched MeSH terms: Splenomegaly/etiology

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