We reported a young patient with myelodysplastic syndrome (MDS) with eosinophilia, in which her chromosomal analysis revealed the presence of trisomy X and a marker chromosome at chromosome 11. The technique used to detect the chromosomal abnormalities is a multicoloured –fluorescent in situ hybridization technique (M-FISH). Our observation suggested that these underlying chromosomal abnormalities were probably responsible for her development of MDS with eosinophilia. Myelodysplastic syndrome (MDS) is a condition whereby there is ineffective production of haematopoietic stem cells and poor quality of cells produced. The cause can either be a primary bone marrow problem, de novo or therapy related. Most MDS cases are secondary rather than primary. Many chromosomal abnormalities have been found in cases of myelodysplastic syndrome. We described a case of MDS with eosinophilia in association with presence of trisomy X and a marker chromosome in chromosome 11.

The goal of treatment in distal radius fracture is to restore the anatomy of the distal radius, however the criteria currently used to evaluate the quality of reduction are based on Western based published figures. This goal of this study was to investigate whether there are variations in the morphology of the distal radius among the multiracial population of Malaysia. Consecutive normal wrist radiographs of patients who presented to the accident and emergency unit in three major hospitals in Malaysia were measured. . The palmar tilt of the distal radius averaged 12.6o ± 3.55o, and the radial inclination averaged 25.1o ± 3.42o. The ulnar variance averaged – 0.1 ± 1.31mm, 38.4% of the patients had neutral ulnar variance, 28.8% have negative ulnar variance and 32.9% have positive ulnar variance. Our results indicate that distal radius morphometric parameters in the Malaysian population are comparable to Western figures.

Cavernous lymphangioma is a congenital malformation of lymphatic system causing dilated lymphatic sinuses that involve the skin and subcutaneous tissues. This was an interesting case of dystrophic macrodactyly of the left ring and little finger in a 18-month-old girl who presented with swollen and sausage like fingers deformity which turned out to be an isolated cavernous lymphangioma. This tumor, although rare to occur in the extremeties, must be differentiated from other congenital vascular lesions of the hand that include arteriovenous malformations and hemangiomas. Diagnosis should be solely based on histopathological analysis of the excised tissue mass. Surgical excision is usually necessary for satisfactory functional and cosmetic outcome.

Clonal disorders of LGL may either be CD3+ CD56- or CD3- CD56+ phenotype and these have been designated as T-cell leukaemia (T-LGL) or natural killer cell (NK)-LGL leukaemia respectively. Clonality is usually demonstrated by clonal rearrangement of T-cell receptor gene rearrangement or identified by flowcytometry analysis. Most patients with T-LGL will have an indolent course. In this report we described an aggressiveness of disease in a patient with clonal CD3+ LGL leukaemia whose cells also co-expressed CD56 diagnosed by flowcytometry. The patient responded well to interrupt ALL standard risk protocol however succumbed to her disease while waiting for upfront stem cell transplant. This case highlights on both the classical laboratory findings of rare entity of disease as well as a review of the literature pertaining particularly on its management.

Allogeneic bone marrow or peripheral blood stem cell transplantation traditionally uses myeloablative regimen for conditioning to enable grafting of donor's stem cells. Animal experiments have shown that a milder non-myeloablative conditioning regimen does allow engraftment to occur. Nonmyeloablative conditioning regimens are low-intensity immunosuppressive treatment given to the recipient before infusion of donor's stem cells. It was reported to have decreased immediate procedural mortality, in particular those secondary to acute graft versus host reaction. However, it did give rise to higher risks of graft rejection, tumour tolerance and disease progression. Fortunately, appropriately administered donor lymphocyte infusion has been shown to establish full donor chimerism (complete donor stem cell grafting in the recipient's bone marrow) and potentiate antitumour effect (graft versus tumour reaction). The reduction of immediate transplant mortality allows the procedure to be carried out in older age groups, patients with concomitant diseases that otherwise would have made the patients unfit for the procedure, patients with non-malignant disorders such as congenital immune deficiencies, autoimmune disorders or thalassaemia majors. The regimen also allows transplantation of genetically manipulated haemopoietic stem cells (gene thrapy) to be carried out more readily in the immediate future. Lastly, the regimen may serve as a platform for immunotherapy using specific T cell clones for anti-tumour therapy with or without the knowledge of known tumour antigen.
seconds ; regimen ; Conditioning (Psychology) ; Stem Cells ; Exertional dyspnea

Concurrent thoracic and abdominal aortic aneurysm is uncommon. It remains a formidable surgical challenge to vascular surgeons, as decision to treat in staged or simultaneous setting still debatable. We present, here, a case of a 62-year-old-man with asymptomatic concurrent thoracic and abdominal aortic aneurysms, which was successfully treated with two-stage hybrid endovascular repair. The aim of this case report is to discuss the treatment options available, possible associated complications and measures to prevent them.
Aortic Aneurysm, Abdominal ; Aortic Aneurysm, Thoracic

Aged ; Antibodies, Antineutrophil Cytoplasmic ; blood ; Comorbidity ; Glomerulonephritis ; etiology ; immunology ; physiopathology ; Humans ; Male ; Neutrophils ; pathology ; Thrombotic Microangiopathies ; diagnosis ; physiopathology

Anaplastic large cell lymphoma (ALCL) is a rare tumour, accounting for approximately 3% of adult non-Hodgkin lymphomas.1 Primary systemic ALCL frequently involves both lymph nodes and extranodal sites. A 44-year-old woman presented with a firm, mobile mass in the left iliac fossa region. Ultrasound findings showed a well defined inhomogenous soft tissue mass, measuring 4x4x2.6cm in the deep subcutaneous region. Histopathological examination revealed that the mass was infiltrated by large lymphoid cells with marked nuclear atypia including kidney-shaped nuclei. These neoplastic cells expressed anaplastic lymphoma kinase (ALK) (both nuclear & cytoplasmic staining), CD30 and EMA but not for T-cell (CD45RO and CD3), and B-cell (CD20 & CD79α) markers. Fluorescence in situ hybridization (FISH) analysis showed a t(2;5)(p23;q35) chromosomal translocation. Subsequently the patient developed shortness of the breath and a thoracic computed tomography (CT) scan showed a mass encasing the right upper lobe bronchus. She also had bilateral axillary lymph nodes, measuring 1 cm in diameter (biopsy was not done). The mediastinum and endobronchial region did not show any abnormalities. She received 6 cycles of CHOP chemotherapy and remained disease free 2 years after diagnosis. ALCL, rarely present as a soft tissue tumour and this disease should be included as a differential diagnosis of any soft tissue mass.

Two duplex PCR assays were established for the detection of C. trachomatis (Ct), N. gonorrhoeae (GC), M. hominis (Mh), and U. urealyticum (Uu). These assays were used on clinical specimens obtained from women with Premature Rupture of Membrane or Post Partum Fever, from preterm infants, as well as from women with uneventful pregnancies and their babies delivered vaginally at term. The analytical sensitivity of the duplex PCR assays with internal controls incorporated is 7.0, 19.0, 5x10(3) and 7x10(2) genome copies per reaction for Ct, GC, Mh and Uu respectively. Specificity was demonstrated by the amplification of only target DNA in the presence of other organisms. Among 40 women with normal, at term, deliveries, there were 6 positives for Ct, 2 for GC and 1 for Uu. None of these women had signs of genital tract infection. The Mh/Uu PCR was positive in 11 of 40 PROM cases, with 7 women positive for Uu, 2 for Mh and 2 others for both organisms. Of 40 blood cultures taken from post-partum maternal infections, 6 were positive for Ct and 1 for Mh. Respiratory secretions from 30 premature neonates yielded 5 positives for Uu and one each for Mh and Ct. In contrast, there was only 1 positive result (for Mh) in 30 mature neonates. With 1 exception, all mycoplasma and ureaplasma positives were confirmed by culture and the concordance between paired tracheal aspirates and nasopharyngeal swabs from neonates was 96.7%. These results show the potential use of the duplex PCR assays for the diagnosis of maternal and neonatal disease caused by the four urogenital pathogens.
Polymerase Chain Reaction ; Human Females ; seconds ; Neisseria gonorrhoeae ; Infant, Newborn

Kaposi’s sarcoma (KS) is strongly associated with Human Herpes Virus 8 (HHV8) and Human Immunodeficiency Virus infection (HIV). It was the first malignancy to be linked with Acquired Immunodeficiency Syndrome (AIDS) and it is still the most commonly encountered malignancy associated with HIV. We report a case of Kaposi’s sarcoma in a homosexual man.