Leptospirosis is an important zoonotic disease with worldwide distribution and nonspecific clinical manifestation. We report a case of fatal leptospirosis in a previously healthy woman with a causative agent. A young adult Indian woman was brought in dead to the forensic department. Ten days before, she developed fever, dizziness with headache, myalgia, diarrhea, and vomiting. Routine inquest and autopsy were performed on the deceased, revealing hemorrhagic lungs with extensive intra-alveolar hemorrhages, pale liver with dissociation and separation of hepatocyte plates, and edematous brain with histiocyte and lymphocyte infiltration in the parenchyma and meninges. Heart tissue depicts myocarditis and pericarditis inflammatory changes. Cerebrospinal fluid (CSF) was turbid in appearance with mildly elevated leukocytes, predominantly lymphocytes. Real-time PCR targeting lipL32 gene of pathogenic Leptospira was detected in the blood, CSF, brain, kidney, heart, and liver. The genetic profile of the causative agent was ST149 (multi-locus sequence typing Scheme 3). This study illustrates the usefulness of Leptospira PCR assay in postmortem diagnosis and addresses the need for further surveillance to identify the epidemiological link of the disease.
True vehicular homicides are defined as those occurrences in which a motor vehicle is intentionally used as a weapon in taking of a life. A case is presented in which the deceased was traveling in the front passenger seat of a motor car that was deliberately rammed by a heavy jeep that came in the opposite direction, resulting in a serious frontal collision. Immediately after the impact, while the occupants of the car were lying in a dazed condition, the two persons riding in the jeep escaped with a bag containing money that was in the car, leaving the jeep behind. The impact mainly involved the driver's sides of both vehicles. The driver of the car sustained serious injuries but was found to be alive, whereas the front-seat passenger, who did not show any serious external injuries, was found to be in a collapsed state and was pronounced dead on admission to the hospital within 30 minutes of the accident. The autopsy revealed that death was caused by closed hemopericardium from a ruptured right atrium. The evaluation of the external and internal injuries confirmed that the fatal injury and a few serious internal injuries were caused by the seat belt (tertiary-impact injuries). The ruptured right atrium was attributed to blunt abdominal trauma by impacting against the lap belt. The case was a true vehicular homicide in which a motor vehicle had been used as a weapon to kill a person. Various aspects pertaining to road accidents, the safety of the occupants, and the advantage and disadvantage of the safety devices are discussed.
Hemophagocytic lymphohistiocytosis (HLH) is a disorder characterized by uncontrolled mature histiocyte proliferation, hemophagocytosis, and hypercytokinemia. We describe a previously healthy pregnant patient who presented in the third trimester of pregnancy with HLH.
INTRODUCTION: HPS is a potentially life-threatening histiocytic disorder that has been described in various viral infections including dengue. Its involvement in severe and fatal dengue is probably more common but is presently under recognized.
CASE DESCRIPTION: A 38-year-old female was admitted after 5 days of fever. She was deeply jaundiced, leukopenic and thrombocytopenic. Marked elevation of transaminases, hyperbilirubinemia and hypoalbuminemia were observed. She had deranged INR values and prolonged aPTT accompanied with hypofibrinogenemia. She also had splenomegaly. She was positive for dengue IgM. Five days later she became polyuric and CT brain image showed gross generalized cerebral edema. Her conditions deteriorated by day 9, became confused with GCS of 9/15. Her BMAT showed minimal histiocytes. Her serum ferritin level peaked at 13,670.00 µg/mL and her sCD163 and sCD25 values were markedly elevated at 4750.00 ng/mL and 4191.00 pg/mL, respectively. She succumbed to the disease on day 10 and examination of her tissues showed the presence of dengue virus genome in the bone marrow.
DISCUSSION AND EVALUATION: It is described here, a case of fatal dengue with clinical features of HPS. Though BMAT results did not show the presence of macrophage hemophagocytosis, other laboratory features were consistent with HPS especially marked elevation of ferritin, sCD163 and sCD25. Detection of dengue virus in the patient's bone marrow, fifteen days after the onset of fever was also consistent with the suggestion that the HPS is associated with dengue virus infection.
CONCLUSIONS: The findings highlight HPS as a possible complication leading to severe dengue and revealed persistent dengue virus infection of the bone marrow. Detection of HPS markers; ferritin, sCD163 and sCD25, therefore, should be considered for early recognition of HPS-associated dengue.
KEYWORDS: Bone marrow; Dengue; Ferritin; Hemophagocytic syndrome; MAS; Macrophage
Study site: University Malaya Medical Centre (UMMC), Kuala Lumpur, Malaysia
The rapid diagnosis and subtyping of influenza is particularly important in areas where avian influenza (H5N1) is present. The ability to recognise both typical and atypical presentations of influenza is also critical in such settings. A six-month-old male child who visited a H5N1-affected area subsequently died from a severe febrile diarrhoeal illness with minimal respiratory symptoms, and was initially diagnosed with influenza A of an unknown subtype. The final microbiological results showed a highly unusual combination of influenza A (H3N2) and Campylobacter jejuni infection.
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.
Rhabdoid tumour is a rare childhood tumour with poor prognosis. We report a 13-month-old Malay girl suffering from this tumour that was located at the left fronto-temporo-parietal region of the brain. Computed tomography showed a large irregular enhancing mass that caused obstructive hydrocephalus. The tumour did not reduce in size after three operations and finally the patient succumbed to the disease four months after diagnosis.
A patient was admitted for breathlessness associated with post-splenectomy multiple pseudocysts and succumbed after internal drainage of the pseudocyst. Although the occurrence of pseudocyst following splenectomy is uncommon, failure to identify and treat this condition at an early stage could result in fatal consequences. Imaging plays an important role in the diagnosis and management of pseudocyst occurring after splenectomy. The advent of interventional radiology has provided better treatment option for patients with solitary pancreatic pseudocysts with success rates similar to those with open surgery but with lower morbidity and mortality rates. However, its role in the management of multiple pseudocysts remains to be defined.
In melioidosis caused by Burkholderia pseudomallei, although every organ in the body may be involved, the highest mortality of 73% occurs when the respiratory system is affected. These patients invariably die of acute respiratory failure. Most of them also have underlying predisposing factors like diabetes mellitus.
Two children with non-Hodgkin's lymphoma (NHL) as the presenting illness of acquired immunodeficiency syndrome (AIDS) are described. There was a delay in diagnosing the underlying AIDS in both cases. In the first case, an 18-month-old boy with stage IV, high-grade,T-cell NHL, the diagnosis of underlying AIDS was suspected only when he developed recurrent and profound opportunistic infection during chemotherapy. The second case, an eight-month-old female infant presented initially with hepatosplenomegaly and thrombocytopenia of undetermined cause. She had progressive abdominal distension and swelling of her right eye one year later due to high grade B-cell NHL. She was later found to be sero-positive for HIV during pre-chemotherapy screening. As the prevalence of HIV infection continues to increase, HIV infection should be considered in the differential diagnoses of childhood hepatosplenomegaly and thrombocytopenia, and as a possible underlying cause of childhood cancer, especially NHL.
The diagnosis of disseminated tuberculosis should be entertained in all patients with unexplained fever associated with hepatomegaly and/or splenomegaly with or without anomalies in liver function tests and haemogram. It should be considered as a possible cause of septic shock especially in patients with typical risk factors such as advanced age, diabetes, alcoholism or immunosuppression. Prompt therapy could be life saving in an otherwise potentially fatal condition. It is therefore appropriate to initiate anti-tuberculosis treatment as soon as such a diagnosis is suspected and not await final confirmation.
Hyperimmunoglobulin E syndrome (HIE) is a rare condition characterised by marked elevation of serum IgE level, chronic dermatitis, intense pruritus, and recurrent serious infection. The major organism is usually S aureus. We report a case of an infant with HIE, who had pulmonary nocardiosis. The clinical features, immunological abnormalities, and radiological features of the condition are described. The child finally succumbed to the complications of pulmonary nocardiosis.
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.
A case of desmoplastic small round cell tumour (DSRCT) is presented. This aggressive and rare neoplasm predominantly affects males and is almost exclusively intraabdominal in location. It is unique in that neural, mesenchymal and epithelial markers are co-expressed. Despite multi-modal therapy, the prognosis is extremely poor. The present report details the clinical features and typical pathological findings of DSRCT in an 11-year-old boy, who succumbed to the disease 16 months after diagnosis despite multiple chemotherapeutic regimes.
Primary germinomas of the central nervous system carry a good prognosis because of their radiosensitivity. Recurrences are rare and extraneural metastases are even more unusual. One of the possible routes of extraneural spread is via ventriculo-peritoneal shunts which may be required to reduce intracranial pressure. One such case of germinoma metastasizing via a ventriculo-peritoneal shunt is reported. Patients with intracranial germinomas and ventriculo-peritoneal shunts should have close surveillance of their abdomens and may require systemic chemotherapy.
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.
Melioidosis is known to cause abscesses in various organs, including the cranium, though less commonly. We present a patient with scalp abscess and subdural empyema that was visible on computed tomography of the brain. The neurosurgical drainage grew Burkholderia pseudomallei. Despite our best effort to treat the patient using parenteral antibiotics and surgical drainage, the patient did not survive.
Extramedullary plasmacytoma is a rare malignant plasma cell tumour. We report an extremely aggressive case of extramedullary plasmacytoma of the right maxillary sinus, which had metastasised to the brain and rib. A 56-year-old man presented with recurrent epistaxis and acute anaemia. Nasendoscopy revealed a medialised medial wall of the right maxilla and a mass occupying the whole nasopharynx. Magnetic resonance imaging revealed a right maxillary tumour with extension to the ipsilateral nasal cavity, nasopharynx, right sphenoid and ethmoidal sinuses. There was an extra-axial brain metastasis. There were metastases to the right parietal region and left eighth rib. Histopathology examination of the maxillary mass revealed abundant plasma cells with kappa-chain restriction. He was planned for four cycles of chemotherapy. Unfortunately, in view of the advanced stage of disease, he succumbed to his disease during the first cycle of chemotherapy.
We present a previously-healthy 12-year old girl from a rural community and who was admitted to a district general hospital in Malaysia with coagulopathy and septic shock. Despite receiving intensive care, she succumbed to her illness. Blood cultures grew Burkholderia pseudomallei. Melioidosis is an unusual cause of paediatric Gram-negative sepsis among children in Malaysia.
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.