Dear Editor, Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis, usually affecting middle-aged to older adults. It is a multisystemic disease with protean clinical manifestations. It can involve single or multiple organs, and presentations range from asymptomatic lesions detected incidentally on imaging to severe organ dysfunction. Hence, accurate and timely...

The management of severe aortic stenosis (AS) in patients with small aortic annulus (SAA) represents a therapeutic challenge due to the increased mortality and major adverse cardiac events (MACE) seen in this group of patients even after surgical aortic valve replacement (SAVR).1,2 In recent times, the role of transcatheter aortic...

Dear Editor, Thrombosis is an unexpected complication of COVID-19 initially reported in 3 patients from China.1 These patients tested positive for immunoglobulin (Ig) A anticardiolipin (ACA), IgG anti-β2-glycoprotein I antibodies (aβ2GPI) and IgA aβ2GPI, though not for the lupus anticoagulant (LAC). In a Singapore study comprising 47,527 patients, 19 (0.04%) developed...

Dear Editor, Peripheral artery disease (PAD) is characterised by the debilitating atherosclerotic occlusion of arteries in the lower extremities, with chronic limb threatening ischaemia (CLTI) representing the most advanced stage of this disease process. Left untreated, these sequelae will invariably progress to major lower extremity amputation (LEA) and premature death....

Dear Editor, Haemoglobin H (HbH) disease is a haemoglobinopathy affecting 3 of 4 α-globin genes on chromosome 16. It results from the deletion of 2 linked α-globin genes (α0-thalassaemia) on one chromosome and either a single α-globin gene deletion (--/-α) or non-deletional gene mutation (--/αTα) on the other chromosome.  The...

Neutrophilic eccrine hidradenitis (NEH) is a neutrophilic dermatosis primarily affecting the eccrine glands and occurs most commonly in patients undergoing chemotherapy for a malignancy. This article is available only as a PDF. Please click on “Download PDF” on top to view the full article.

Idiopathic thrombocytopenic purpura (ITP) is an immunoregulatory disorder in which antibodies damage platelets leading to their removal by cells of the reticuloendothelial system (RES). This occurs mainly in the spleen which is also the primary site of synthesis of these antiplatelet antibodies. This article is available only as a PDF....

In the last two decades there has been a surge of interest concerning the psychosocial correlates of life-threatening illnesses. Research in this area has focused especially on cancer and the possible link between immunology and psychological factors. This article is available only as a PDF. Please click on “Download PDF”...

Injuries form the fifth commonest cause of death locally. They accounted for 27 out of 100 000 deaths in 1993. This article is available only as a PDF. Please click on “Download PDF” on top to view the full article.

Our patient was a 71-year-old Chinese man who presented with a 6-year history of recurrent papular erythematous eruptions to the dermatologist. This article is available only as a PDF. Please click on “Download PDF” on top to view the full article.

First-generation fibrinolytic agents (streptokinase and urokinase) are effective thrombolytic drugs, but are not fibrin specific as they also have the property to convert circulating plasminogen to plasmin when given at therapeutic doses. Because the plasminogen in the thrombus is in equilibrium with the plasminogen pool in plasma, the plasminogen...

The tremendous interest in finding new anti-aggregating agents stems from the preeminent role that platelets play, not only in haemostasis and thrombosis, but also in atherogenesis and, be it less definitive, in arterial spasm. Compelling data from large-scale trials have established the role of platelet inhibitors in reducing coronary...

In 1993, Dahlback and colleagues described a new hereditary thrombophilia state-activated protein C resistance (AK-R). Further studies showed this to be the commonest genetic cause of thrombophilia. This article is available only as a PDF. Please click on “Download PDF” on top to view the full article.

Pseudomembranous tracheobronchitis caused by Aspergillus is a rare and lethal variant of invasive aspergillosis. It has been reported in a few series of immunocompromised patients. This article is available only as a PDF. Please click on “Download PDF” on top to view the full article.

A number of specific chromosome abnormalities are associated with acute lymphoblastic leukaemia (ALL). The commonest in adults with B lineage ALL is the Philadelphia (Ph) chromosome t(9;22)(q34;q11). This article is available only as a PDF. Please click on “Download PDF” on top to view the full article.

Patients with mechanical heart-valve prosthesis receive lifelong anticoagulation. The management of these patients taking oral anticoagulants is aimed at achieving and maintaining adequate levels of anticoagulation to prevent thrombotic manifestations and at the same time to keep the risk of severe haemorrhagic complications to the minimum. This article is available...

Acute promyelocytic leukaemia (APL), or acute myeloid leukaemia (AML)-M3 under the French-American-British (FAB) classification, is characterised by the following: a distinct expansion of promyelocytes and immature myeloblasts in the bone marrow; balanced reciprocal translocation between the long arms of chromosome 15 and 17, t(15;17) which results in apposition of...

Deep vein thrombosis (DVT), pulmonary embolism (PE), the postphlebitic syndrome and recurrent venous thromboembolism (VTE) are important and often preventable causes of early morbidity or death and of long-term disability in western populations. The reported annual incidence of VTE is about 2 per 10,000 persons in Scotland. This article is...

Multiple myeloma (MM) is a disorder in which malignant plasma cells accumulate in the bone marrow and produce an immunoglobulin, usually monoclonal IgG or IgA. The incidence of MM in Singapore is 1 to 2 per 100,000 per year with a median age of 65 to 70 years at...

Injury to blood vessels triggers haemostasis, a process that has evolved to achieve two superficially incompatible outcomes: arresting blood loss while protecting vessel patency and blood flow to distal organs. Contributors to haemostasis include subendothelial tissues and endothelial cells, blood platelets, tissue factor, plasma clotting factors and their physiological...

Essential thrombocythaemia (ET) is a rare myeloproliferative disease which manifests with an elevated platelet count. It presents predominantly in the middle-aged population, occurring slightly more frequently in women than men. This article is available only as a PDF. Please click on “Download PDF” on top to view the full article.

Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder in which platelets are sensitised with an antiplatelet autoantibody and then destroyed by the reticuloendothelial system.1 Hence, it is also known as primary autoimmune thrombocytopenic purpura. There are two forms of ITP—acute and chronic. This article is available only as a PDF....

A clear understanding of thrombophilia is becoming increasingly important in the practice of high-risk obstetrics. In addition to their role in thromboembolic disease, there is increasing evidence linking thrombophilia and adverse pregnancy outcomes such as pre-eclampsia, intrauterine growth restriction, placental abruption and recurrent pregnancy loss. This article is available only...

Malignancy complicates the course of about 1 in 1000 pregnancies and is the second leading cause of death in women of reproductive age. The most commonly diagnosed malignancies during pregnancy are breast cancer, cervical carcinoma, Hodgkin’s lymphoma and melanoma. This article is available only as a PDF. Please click on...

Idiopathic thrombocytopaenic purpura (ITP) is the most common autoimmune haematological disorder in pregnancy. The pathophysiology of the disease is peripheral destruction of platelets mediated by antiplatelet antibodies. This article is available only as a PDF. Please click on “Download PDF” on top to view the full article.

Deep vein thrombosis (DVT), and its acute complication–pulmonary embolism (PE), is one of the most important preventable causes of death in hospital patients. It also contributes to significant morbidity in terms of post-phleblitic syndrome and chronic venous ulcers. This article is available only as a PDF. Please click on “Download...

Development of inhibitors against factor VIII is one of the main complications in the treatment of haemophiliacs. Inhibitors block the procoagulant effects of factor VIII and prevent the successful arrest of bleeding episodes when administered to these haemophiliacs. This article is available only as a PDF. Please click on “Download...

Oral anticoagulation therapy is used for a number of conditions, namely deep venous thrombosis, pulmonary embolism, mechanical heart valves, embolic stroke as well as atrial fibrillation. Patients receiving this therapy are carefully monitored in order to maintain the intensity of anticoagulation in the appropriate therapeutic range. This article is available...

Trauma represents a leading cause of death globally, and post-traumatic haemorrhage accounts for 40% of early mortality in spite of recent improvements in modern trauma care.1 Post-traumatic haemorrhage occurs primarily from direct injury to blood vessels, leading to exsanguination and hypovolaemic shock depending on the type and calibre of...

Myelodysplastic syndromes (MDS) are a heterogeneous group of acquired clonal haemopoietic stem cell disorders characterised by ineffective haematopoiesis and peripheral cytopenia. The natural history ranges from the indolent forms spanning years to those with rapid evolution to blast transformation. This article is available only as a PDF. Please click on...

Haematopoietic stem cell transplantation (HSCT) is now a well-established therapeutic modality for a broadening spectrum of medical problems. Umbilical cord blood (CB) has emerged as an alternative source of HSCT in recent years due to the lack of a human leukocyte antigen (HLA)-identical sibling donor in the majority of...

Allogeneic haematopoietic stem cell transplantation (HSCT) is an established form of treatment for many high-risk haematological malignancies and marrow failure syndromes. The use of HSCT is still limited by the availability of human leukocyte antigen (HLA) matched donors, even though unrelated donor registries have markedly improved the chances of...

Haematopoietic stem cell transplantation (HSCT) has become an established modality for the treatment of cancer and diseases of the bone marrow and immunological system. Such transplants effect their cures through 3 main avenues: namely, eradication of disease through intensive chemo/radiotherapy, replacement of defective blood-forming cells with normal haematopoietic progenitors...

In recent years, the term “haematopoeitic stem cell transplantation” has replaced “bone marrow transplantation”. It is more precise and emphasises the “haematopoeitic stem cell” as the key element. This article is available only as a PDF. Please click on “Download PDF” on top to view the full article.

Porphyrias are a rare and intriguing group of metabolic disorders. We report a case of congenital erythropoietic porphyria, one of the infrequent forms of porphyria. This article is available only as a PDF. Please click on “Download PDF” on top to view the full article.

Calcinosis cutis is characterised by the aberrant deposition of calcium salts in the skin. In metastatic calcinosis cutis, calcium salts are precipitated in normal tissue as a result of an underlying defect in calcium and/or phosphate metabolism. This article is available only as a PDF. Please click on “Download PDF”...

Cancer patients who become severely neutropenic as a result of intensive myelosuppressive chemotherapy are at high risk for developing life-threatening infections, and unless they are treated at the first sign of infection, the rate of mortality is high. Because of the defect in the inflammatory response, the classic signs...

Rising rates of antibiotic resistance prompted a review of antibiotic use policies hospitalwide. The Department of Haematology established a new set of consensus guidelines in 2002 for antibiotic use in febrile neutropenia. We were tasked by the hospital’s Pharmacy and Therapeutics Committee to audit adherence to the guidelines. This article...

A 38-year-old Chinese man who was previously well, presented with one month of fever and a non-pruritic rash involving the face, trunk and upper limbs. There was no oral, ocular or genital involvement. He denied weight loss, night sweats, fatigue or easy bruising and there was no history of...

Cytogenetic studies are important to the diagnosis and monitoring of various haematopoietic disorders such as myelodysplastic syndrome (MDS), acute myeloid leukemia (AML), acute lymphoblastic leukemia (ALL), chronic lymphocytic leukemia (CLL), chronic myeloid leukemia (CML), lymphoproliferative disease (LPD), and multiple myeloma (MM). It can also provide a prognosis to a...

A 20-month-old boy came from Bangladesh to Singapore for medical consultation. He presented with progressive pallor, easy bruising, intermittent dark-coloured urine, and failure to thrive since birth. This article is available only as a PDF. Please click on “Download PDF” on top to view the full article.

Febrile neutropenia (FN) is considered a medical emergency and remains a major cause of morbidity and mortality in Oncology and Haematology units worldwide. A study analysing pooled FN data from 115 US academic medical centres between 1995 and 2000 showed that the overall in-hospital mortality was 9.5%, whereas pooled...

Acute lymphoblastic leukaemia (ALL) is a heterogeneous disease with abnormal proliferation and accumulation of immature lymphoblasts within the bone marrow (BM), peripheral blood and lymphoid tissues, and is composed of different genetic, biological, and clinically relevant subtypes. Morphological and cytochemical methods were the main tools for diagnosis and classification...

Thalassaemia is a genetic haemoglobinopathy in which the production of normal haemoglobin is partly or completely suppressed because of a defective synthesis of 1 or more of its component globin chains. In haemoglobin (Hb) E beta-thalassaemia, an individual inherits 1 gene for Hb E from 1 parent and 1...

Thrombocytopenia is a common clinical problem found in laboratory results during health examinations. Blood platelets play an essential role in haemostasis, thrombosis and coagulation of blood. This article is available only as a PDF. Please click on “Download PDF” on top to view the full article.

In this issue of the Annals, a rapid review of adherence to evidence-based medical treatment highlights an important and underappreciated aspect of the management of patients with peripheral arterial disease (PAD). It is easy, upon meeting a patient, to prescribe appropriate medication in an outpatient setting and believe that...

Manifestations of the coronavirus disease 2019 (COVID-19) span a wide clinical spectrum, from asymptomatic carriers to critical illness with a wide range of complications. Our understanding of the pathophysiology of the disease process is still evolving. As part of the host response to viraemia, it has been postulated that...

The current coronavirus disease 2019 (COVID-19) pandemic has exerted significant strain on healthcare worldwide. Mostly asymptomatic or mildly symptomatic, COVID-19 caused by SARS-CoV-2 is described as a thrombo-inflammatory syndrome,1 with severe respiratory illness occurring in about 13% of affected patients. This can rapidly transform into a life-threatening condition in...