Anaemias of oral significance

Anaemia is not a disease in itself. It is a sign of a single or multiple diseases. Anaemia is said to exist when the haemoglobin concentration is below normal for the age and sex of an individual. The synthesis and normal circulatory level of haemoglobin in any given individual depend on factors such as an adequate supply of haemopoietic nutrients, normal functioning of bone marrow, and proper utilization of haemoglobin. Based on these factors anemia can be broadly grouped into three categories: 1. Anaemia due to lack of haemopoietic nutrients (nutritional anemia) 2. Anaemia due to bone marrow dysfunction (aplastic anaemia) 3. Anaemia due to excessive breakdown of red blood cells (haemolytic anaemia) (AU)

Severe aplastic anaemia - complete response to antilymphocyte globulin and cyclosporin

Severe aplastic anaemia is uniformly fatal unless treated with immunosuppressive therapy or bone marrow transplantation. The latter is curative in 65 percent of patients and is the treatment of choice in children and young adults. Antilymphocyte globulin (ALG) and cyclosporin may be used successfully in the absence of an HLA matched sibling donor. We report the case of a twelve year old boy with severe aplastic anaemia who received immunosuppressive with ALG and cyclosporin and is alive and well three years an six months post treatment.(AU)

Management of sickle cell disease; lessons from the Jamaican Cohort Study

Sickle cell disease is enormously variable in its expression and outcome. In addition to this intrinsic variablity are the problems of symptomatic selection biasing observations towards the sever end of a wide clinical spectrum and a truly changing natural history as a result of better management. Against this background, there was a need for a description of the disease in a truly representative sample of patients and this objective has been approached in the Jamaican Cohort Study of Sickle Cell Disease. Initiated in 1973, this study is based on all cases of sickle cell disease detected among 100,000 consecutive normal deliveries in Kingston, Jamaica. All affected children as well as age matched normal controls have been followed prospectively and are currently ages 11 to 19 years. The following review is based on lessons learnt from this cohort study. It is not intended to be a comprehensive survey of knowledge of sickle cell disease and does not address major contributions from studies elsewhere. In some ways, therefore, the review may appear unbalanced because of this specific objective. However, a great deal has been learnt about the evolution of the abnormal haematology of sickle cell disease and its relationship to clinical features. The causes of early mortality in sickle cell disease in Jamaica are described and the major complications such as acute splenic sequestration, pneumococcal septicaemia, aplastic crisis, hypersplenism, and acute chest syndrome have been addressed with varying success. Overall survival to the age of 19 years has been 75 percent and it is planned that the study should continue to define the problems of late adolescence and early adult life (AU)

Human parvovirus infection in homozygous sickle cell disease

We studied the epidemiology of human parvovirus B19 infection in 308 children with homozygous sickle cell (SS) disease and 239 controls with a normal haemoglobin (AA) genotype followed from birth in a cohort study. Annual serum samples identified the time and frequency of B19 infection, which did not differ between SS and AA children, about 40 percent of each age group developing specific IgG by age 15. B19 infection followed an epidemic pattern similar to that observed for aplastic crises; accounted for all 91 aplastic crises that occurred; and was found in an additional 23 SS patients, of whom 10 showed mild haematological changes and 13 no changes. The magnitude or duration of IgG response did not differ between these groups. No patient had 2 attacks of aplasia and no patient nor control had 2 attacks of B19 infection. Following B19 infection, serial specific IgG concentrations remained high after 5 years in only 45 percent of SS patients, although the rarity of recurrent aplasia suggests lifelong immunity. B19 infection accounts for most if not all aplastic crises in SS disease, but at least 20 percent of infections do not result in aplasia. An effective vaccine against B19 might make an important contribution to the management of sickle cell disease (AU)

Fanconi's aplastic anaemia in two sisters in Trinidad and Tobago

This report describes two cases of Fanconi's anaemia in Trinidadian sisters of East Indian origin. The cases have been confirmed by chromosomal analysis which showed increased breaks and ring forms. Anorexia was a persistent finding in both cases. One of the sisters also had a poor growth hormone response. (AU)

Parvovirus-associated aplastic crisis in homozygous sickle cell disease - abstract

Aplastic crises in patients with homzygous sickle-cell (SS) disease in Jamaica affect predominantly children and occur in epidemics. Of 67 cases in a cohort study of 314 children with SS disease, 62 were attributable to human parvovirus infection. Affected children were aged 0.5 - 12.5 years, and the incidence rose to 28 percent in 10 years. No recurrences were seen. Symptoms and signs on presentation were attributable to the viraemia, and acute anaemia. Asymptomatic thrombocytopaenia was common. Blood transfusion was given in 54 (87 percent) cases. Thirty-eight (61 percent) children were admitted to hospital, 16 of whom were extremely ill on presentation and one died soon after admission to hospital. Twenty-four (39 percent) were managed as out-patients, 16 of whom were transfused. Parvovirus-associated aplastic crisis is a self-limiting condition with excellent prognosis if diagnosed promptly and appropriately managed (AU)

Parvovirus infection and the aplastic crisis of sickle cell disease - abstract

The aplastic crisis is a temporary self-limited erythropietic maturation arrest which, because of the short red cell survival in homozygous sickle-cell (SS) disease, results in rapid fall in haemoglobin level. These aplastic crises occur in epidemics, predominantly affect children, frequently involve siblings simultaneously, and often follow an upper respiratory tract type of infection. Recent evidence suggests that these events may follow human parvovirus (HPV) infection and the role of HPV infection has been examined in the last three epidemics of aplastic crises observed among SS patients in Jamaica. Eighteen patients were affected in the 1973-1975 epidemics, 45 in the 1979-1981 epidemic, and 41 so far in an epidemic in 1984-1985. Serological studies have been possible in 73 patients and in 69 (95 percent) there was evidence consistent with recent HPV infections. This infection appears to confer long-lasting immunity, and recurrent attacks of aplastic crisis have never been described. These data suggest that a parvovirus vaccine may be beneficial in the prevention of aplastic crises (AU)

The development and use of an antibody capture radioimmunoassay for specific IgM to a human parvovirus-like agent

An IgM-antibody capture radioimmunoassay (MACRIA) was developed for detection of IgM antibody specific for the human parvovirus-like agent B19. Diagnosis of infection with this agent by either antigen detection or antibody seroconversion had been made by counter-current immunoelectrophoresis (CIE) in 18 cases of aplastic crisis occurring in children with homozygous sickle-cell desease. The MACRIA described here gave positive results in 17 of 18 cases; in the remaining case only an acute specimen taken from the patient during viraemia and late convalescent specimens taken 184 and 247 days after onset of illness were avaliable. The test was used to investigate 20 further cases of aplastic crisis in which neither viral antigen nor antibody seroconversion could be detected by CIE. Detection of virus-specific IgM permitted diagnosis of infection with this parvovirus-like agent in 17 of these cases. In the remaining three cases only single serum specimens taken late in convalescence, 82, days or more after the onset of symptoms, were available. In addition to these 34 cases of aplastic crisis in which primary infection with this agent was diagnosed by MACRIA, seven cases of apparent 'silent' infection detected by CIE were investigated. The test permitted the discrimination between primary infection and re-exposure to the virus in six of these patients. The use of this assay has added a considerable weight of evidence implicating primary infection with this parvovirus-like agent as an important cause of aplasic crisis in children with sickle-cell disease. Furthermore, MACRIA permits diagnosis of infection when only single serum specimens taken up to ten weeks after infection are available. Thus the use of this test will significantly facilitate the investigation of other clinical syndromes of presumptive infective infectious aetiology (AU)

The aplastic crisis in sickle-cell anaemia

Of 12 patients with aplastic crisis associated sickle-cell disease, eleven were children with sickle-cell anaemia, and one was an adult with sickle-cell haemoglobin-C disease. Ten of the cases were seen over a period of less than 7 months, and the syndrome may be commoner than has previously been supposed. Evidence is given to support the hypothesis that the distribution of sickle-cell and foetal haemoglobins in the erythrocytes in sickle-cell anaemia is not homogeneous. The fact that seven of the cases belonged to three families implies that infection may play an important aetiological role. This view is supported by the clinical presentation, and by the fact that one patient apparently had infectious mononucleosis. Bacterial and viral studies fail to demonstrate any aetiological agent in these cases (Summary)