A fulminant clinical presentation with high fever and hepatosplenomegaly, together with a course of worsening pancytopenia, coagulopathy and liver failure, is suggestive of the haem syndrome (HPS). Bone marrow examination is diagnostic. We present 3 cases of HPS associated with different aetiologies including acute Ebstein Barr virus infection, T cell lymphoma, and malignant histiocytosis. In all the cases, the diagnosis was made late and the patients succumbed before definitive therapy could be administered.
The clinical presentation and behaviour of giant cell tumours of bone vary. The progression of the disease and metastases are unpredictable, but the overall prognosis is good. We describe the natural history and different clinical presentations of two cases of giant cell tumour of bone where the patients had refused the initial treatment and presented several years later with the disease.
Chronic neutrophilic leukaemia is a rare myeloproliferative disease characterised by splenomegaly, sustained neutrophilia, raised vitamin B12 level and absence of the Philadelphia chromosome. We report a 74-year-old man who presented first with Sweet's syndrome and subsequently leukocytosis. He had splenomegaly, a raised vitamin B12 level, serum uric acid and neutrophil alkaline phosphatase score. Cytogenetic study of the marrow was normal and peripheral blood for BCR-ABL gene transcript was not detectable. He subsequently passed away with bronchopneumonia.
We report a 14 year old Indian-Muslim girl who developed a fulminant, disseminated and fatal varicella infection while receiving steroids for nephrotic syndrome. The terminal phase of her illness was complicated by a bleeding dyscrasia and circulatory collapse. Varicella infection in healthy children is a benign disease. However in neonates and immunosuppressed patients it may be severe and often fatal. There are many reports of fatalities occurring in cancer patients receiving chemotherapy, patients on immunosuppressives for asthma, haemolytic anaemia, rheumatic fever, and renal and bone marrow transplantation. Patients with nephrotic syndrome receiving cyclophosphamide treatment are at particular risk of developing severe chickenpox infection. To our knowledge, there has been only one report of fatal chickenpox infection in a child who received steroids for nephrotic syndrome. We report here a case of fatal haemorrhagic chickenpox complicating nephrotic syndrome.
Acute respiratory distress syndrome (ARDS) associated with severe respiratory syncytial virus infection is rare. We report a 5-month-old Indian girl who was admitted to our intensive care ward with severe respiratory failure who fulfilled the criteria for ARDS using both Murray's Lung Injury Score of > 2.5 and the American-European Consensus Conference definition for ARDS. She developed diffuse bilateral alveolar infiltrates, severe hypoxaemia (PaO2/FiO2 < 100) and required high PEEP (> 15 cm H2O) 24 hours after admission. RSV was isolated from her nasopharyngeal secretion. She also had clinical features suggestive of a primary immunodeficiency and had laboratory evidence of combined T and B cell defect. There was unsustained clinical improvement with a dose of surfactant administered at 36 hours of PICU stay, and she continued to deteriorate and succumbed after 19 days in the PICU.
There have been very few reports of swordfish attacks on humans and none have resulted in death. Although there are no reports of unprovoked attacks on humans, swordfish can be very dangerous when provoked and they can jump and use their swords to pierce their target. We describe here an unusual case of death that resulted from intracranial penetrating injury caused by a swordfish.
Primary malignant melanoma arising in the oesophagus is a rare condition with a dismal prognosis. The diagnosis is often made following surgical resection even though the endoscopic features may be pathognomonic. The classical treatment is oesophagectomy even though the advanced disease stage at the time of presentation and aggressive biological behaviour of the tumour usually results in a fatal outcome. We report the case of a male patient initially diagnosed with squamous oesophageal carcinoma and treated with conventional neo-adjuvant chemo-radiotherapy. Poor clinical and radiological response resulted in a review of the original histology confirming a diagnosis of primary malignant melanoma of the oesophagus. The subsequent alteration in management conferred the patient an improved quality of life. A short review of the literature on primary malignant melanoma of the oesophagus supplements this case report.
A young East Malaysian lady presented with haemoptysis in 1989. Since then she had recurrent episodes of dyspnoea and two occasions of respiratory failure requiring assisted ventilation. An open lung biopsy showed intra-alveolar haemorrhage with diffuse interstitial fibrosis consistent with idiopathic pulmonary haemosiderosis after excluding secondary causes of pulmonary haemorrhage. She failed to respond to corticosteroid and continued to depend on oxygen until she succumbed to the illness 2 years after the presentation.
We report the occurrence of X-linked lymphoproliferative disease (XLP) in two brothers in a Malaysian family. In this disorder, a primary Epstein-Barr virus (EBV) infection is followed by an abnormal proliferation of transformed B-cells that cannot be controlled by suppressor T-cells, leading to the development of deranged immune function. This results in fatal infectious mononucleosis, acquired hypogammaglobulinaemia, virus-infected haemophagocytic syndrome and non-Hodgkin's lymphoma. The diagnosis should be considered when there is a family history of any male having a fulminant course of infectious mononucleosis, an otherwise benign disease. Early diagnosis is important as bone marrow transplantation is the only curative option in this disorder.
We report the case of a 30-year-old woman who was referred to us for evaluation of a 2-week history of fever, headache, vomiting, bilateral ptosis, and blurred vision. Imaging obtained by the referring institution had identified a sphenoid sinus mass and diffuse meningeal infiltration, which was thought to represent an infective process. We subsequently identified the mass as a metastatic hepatocellular carcinoma (HCC). The patient was placed under palliative care, and she died 1 month later. Metastases to the sphenoid sinus from any primary source are very rare, and they are generally not considered in the radiologic differential diagnosis. HCC is known to metastasize to the lung, lymph nodes, and musculoskeletal system; again, reported cases of metastasis to the sphenoid sinus are rare. Indeed, our review of the English-language literature found only 6 previously reported cases of sinonasal metastasis of a primary HCC. A diagnosis of a sinonasal metastasis is more difficult in a patient who has no previous diagnosis of a primary malignancy. In presenting this case, our aim is to remind readers of this possibility.
A rare case of diffuse malignant peritoneal mesothelioma in a 71 year-old Malay man with no previous history of asbestos or radiation exposure is described. The clinical manifestation was a large abdominal mass. At laparotomy he was found to be in the advanced stage of the disease. The tumour was not resectable and patient was sent home. He gradually deteriorated and died three months after diagnosis was made. The magnetic resonance imaging (MRI) features of peritoneal mesothelioma which has not been previously reported are described.
A 19-year-old Malay male succumbed to a septicaemia caused by Chromobacterium violaceum 11 days after onset of illness. The organism is a common soil saprophyte and may be considered as contaminant on culture. It is essential to recognise its clinical significance in purulent processes so that appropriate therapy can be instituted. We report the first fatal case of Chromobacterium violaceum infection in the University Hospital, Kuala Lumpur.
Co-infection of melioidosis and leptospirosis is uncommon. We report here four such cases, confirmed by blood culture for melioidosis and blood polymerase-chain reaction for leptospirosis, which occurred among rescuers involved in a search and rescue operation for a young man who was suspected to have drowned in Lubuk Yu, a recreational forest in Pahang, Malaysia. Despite treatment, three of the patients died from the co-infection.
A case of large adrenocortical carcinoma extending into the inferior vena cava and right atrium is reported. Computed tomography showed a large mass displacing the left kidney inferiorly with an intravascular tumour thrombus extending into the inferior vena cava and right atrium. Radical surgery under hypothermia and cardiopulmonary bypass was performed and the tumour mass, together with the tumour thrombus, was successfully removed. The presence of intravascular tumour extension alone should not be a contraindication to radical surgical therapy, as it is the best hope for prolonged survival.
The latex of pokok ipoh (Antiaris toxocaria) and the root bark of akar ipoh (Strychnos species) have been the main sources of the poisonous principles in dart and arrow poisons prepared throughout south-east Asia. We report a fatal case of rhabdomyolysis and acute oliguric renal failure following oral ingestion of blowpipe dart poison. To our knowledge this is the first such report.
Most invasive fungal sinusitis occurs in immunocompromised adult patients. We present the case study of a 12-year-old boy diagnosed with acute myeloblastic leukemia undergoing chemotherapy. He developed a progressive darkening discoloration over the dorsum of the nose that turned into an eschar. Nasal endoscopy revealed extensive necrotic tissue in the nasal cavity mucosa, inferior and middle turbinates, and septal cartilage that extended to the eschar of the skin over the nasal dorsum. Histopathology showed aspergillus invasive fungal rhinosinusitis.
Primary primitive neuroectodermal tumour (PNET) and extraskeletal Ewing's sarcoma belongs to the Ewing's family of tumours. Primary tumours arising from breast are very rare. There are only a few case reports published on primary extraskeletal Ewing's sarcoma and PNET arising from breast. We present an extremely rare case of an inoperable primary Ewing's sarcoma arising from left breast with contralateral breast, lymphatic and lung metastasis.