Complement haemolytic activity, circulating immune complexes and the morbidity of sickle cell anaemia.

The aim of this study was to find out if the number of crises and complications of sickle cell anaemia (SCA) relate to complement function, or the levels of circulating immune complexes (CIC), complement factor B (Bf), C3 and C4. In 73 steady-state HbSS patients and 50 HbAA control subjects, we determined the haemolytic activity of the alternative pathway of complement (AP50), of the classical pathway (CH50); and the serum concentrations of Bf, C3, C4 and CIC. By clinical examination of each patient and review of the medical records, we determined the number of complications of SCA which had occurred and the mean number of crises per year over a minimum period of 3 years. The mean+/-SD AP50 for the patients (14+/-2 U/ml) was significantly lower than the control value of 16+/-3 U/ml (p<0.001). AP50 had a significant inverse correlation with the number of crises (r=-0.30, p<0.02). Mean+/-SD CIC in patients (0.45+/-0.38 g/l) was significantly higher than in controls: 0.24+/-0.15 g/l (p<0.002). CIC showed a significant direct correlation with the number of complications of SCA (r=+/-0.28, p<0.02). Mean+/-SD Bf in SCA patients (0.19+/-0.09) was higher than in controls (0.17+/-0.05). The difference reached marginal statistical significance (p=0.049). SCA patients and controls had no significant differences in CH50, C3 and C4. These parameters and Bf did not correlate with either the number of crises or complications. The mechanisms underlying the correlations observed in this study are yet to be fully elucidated.

A review of cephalhaematoma in adult sickle cell disease patients.

Twelve patients with sickle cell disease (SCD), eleven males within the age range 21 to 24 years and one female, fourteen years old presenting with cephalhaematoma at the Haematology Day Care Unit (H.D.C.U.) of the University College Hospital, Ibadan during a three year period were reviewed. Only one patient was sickle cell haemoglobin SC (HbSC) while others were homozygous (HbSS). They all presented with a triad of symptoms consisting of fever, headaches and scalp swelling. Significant findings include the exclusive male preponderance and the almost exclusive presentation in HbSS patients. Treatment include analgesic and intravenous fluids.

Immunoglobulin and immune complex levels in Nigerians with acute lymphoblastic leukaemia.

Twenty-five patients with acute lymphoblastic leukaemia (ALL) aged 10 months to 43 years and twenty-five age and sex matched healthy control subjects were investigated in this study. Serum immunoglobulins A, G and M levels were measured by single radial immunodiffusion method and immune complex levels estimated by polyethylene glycol precipitation technique. Significant increase in immune complexes and decrease in immunoglobulin G were observed in the patients. Although not statistically significant, the patients had a lower mean level of immunoglobulin A, and a higher mean immunoglobulin M concentration than the controls. Hypoimmunoglubulinaemia observed in this study may contribute to the aetiology of ALL or be an effect of the disease. Raised immune complexes could result from specific antibodies combining with tumour associated or microbial antigens. Immunoglobulin G levels showed a significant positive correlation with survival in the patients. The adverse effect of reduced immunoglobulin G on the prognosis of ALL is probably due to compromised immunity in the patients.

HIV seropositivity in Nigerians with lymphoproliferative malignancies.

One hundred and four Nigerians with lymphoproliferative malignancies were tested for antibodies to human immunodeficiency virus types 1 and 2 (HIV-1 and 2). All the patients with high grade lymphomas in this series were seronegative for HIV-1 and 2. Only 1 of the 104 patients had antibody to HIV-1 and none had antibody to HIV-2. Although the single positive result would suggest a higher HIV-1 seropositivity rate among the patients compared with the general Nigerian population, it should be interpreted with caution. This preliminary data emphasizes the need for an urgent countrywide study of the problem among this category of patients in Nigeria in view of the low prevalence rate of HIV-1 and 2 in the country.

PIP: Several workers have reported an association between lymphoproliferative malignancies and HIV infection. The revised diagnostic criteria for AIDS which include HIV-seropositivity in patients with Non-Hodgkin's lymphoma of the small, noncleaved, immunoblastic and large cell types are consistent with the observed relationship between HIV infection and lymphoproliferative malignancies. Following this revised definition, it was deemed necessary to determine the prevalence of HIV infection among the patients with lymphoproliferative malignancies seen in the University College Hospital, Ibadan, Nigeria. 104 newly-diagnosed Nigerian patients with lymphoproliferative malignancies seen at the hospital between December 1986 to May 1990 were tested for antibodies to HIV-1 and HIV-2. One patient was HIV-1-seropositive and none were HIV-2-seropositive. All patients with high-grade lymphomas were seronegative for both viruses. These preliminary data indicate the urgent need for a countrywide study designed to learn more about the relationship between HIV infection and lymphoid malignancies in Nigeria. The authors stress that while Burkitt's lymphoma coexisting with HIV-seropositivity is currently taken as diagnostic of AIDS, it is important to establish that the viral infection was acquired before the tumor developed in each patient. This observation is important due to the commonality of Burkitt's lymphoma in Nigeria where some hospitals are still unable to screen blood for HIV before transfusion.

Acute phase reactants and severity of homozygous sickle cell disease.

Serum concentrations of seven acute-phase reactants: albumin, transferrin (Tf), alpha-1-antitrypsin (AIAT), caeruloplasmin (Cp), alpha 2-macroglobulin (alpha 2-MG), haptoglobin (hp) and C-reactive protein (CRP) were determined in 73 subjects with varying severities of homozygous sickle cell (HbSS) disease. Fifty healthy subjects of comparable sex, age and socio-economic class distributions as the HbSS subjects served as controls. Albumin and alpha 2-MG were comparable in all the subject groups. Tf and hp levels were significantly reduced in the HbSS groups relative to the control group. Conversely, AIAT, CRP and CP were significantly elevated. However only Tf and CRP manifested significant correlations with any of the indices of disease severity employed. Transferrin and CRP are suggested as plasma proteins worthy of further evaluation as indicators of severity in homozygous sickle cell disease.

Foetal haemoglobin levels in Nigerians with sickle cell anaemia. A revisitation.

Foetal haemoglobin (HbF) levels were estimated by the alkali denaturation method in 73 Nigerians with sickle cell anaemia (SCA). Subjects were studied during their asymptomatic periods and were divided into three groups based on HbF levels. Group I: HbF levels < 10%; group II: HbF levels > or = 10% but < 15%; group III: HbF levels > or = 15%. Mean crises per year, number of organ (system) complications, degree of anaemia, as well as total severity scores derived from these three parameters did not vary significantly in the three groups. Similarly, HbF levels failed to manifest significant correlation between either frequency of crises, occurrence of complications, degree of anaemia or the derived total severity scores. It is probable that in Nigerian SCA subjects whose HbF concentrations are mostly < 20%, other variables apart from HbF may influence the severity of their disease.

Socio-economic class distribution of the prognostic variants of lymphoproliferative cancers in Nigerians.

One hundred and twenty Nigerians with various lymphoproliferative cancers were grouped according to socio-economic class and the prognosis of their disease. The best prognostic variant of acute lymphoblastic leukaemia occurred mainly in the highest socio-economic class and the worst variant mostly in the low socio-economic class. Chronic lymphocytic leukaemia, a low grade malignancy, and the high grade Burkitt's type of Non-Hodgkin's lymphoma occurred predominantly in the low class. The relationship between prognosis and socio-economic class was not so remarkable in patients with Hodgkin's disease and multiple myeloma. The precise mechanisms by which socio-economic strata influence development of the different prognostic variants of individual lymphoproliferative cancers are not certain.

Lecithin cholesterol acyltransferase activity in acute lymphoblastic leukemia.

Lecithin cholesterol acyltransferase (LCAT) activity and free cholesterol and cholesterol ester concentrations were determined in serum samples from 28 patients with acute lymphoblastic leukemia (ALL) and in an equal number of healthy controls. No significant alterations in LCAT activity, free cholesterol, or esterified cholesterol levels were observed in the ALL patients compared with the controls.

Hepatitis B surface antigenaemia in patients with malignant lymphoproliferative disorders.

One hundred and four patients with malignant lymphoproliferative disorders and 5,690 control subjects were screened for the presence of Hepatitis B surface antigen (HBsAg) in their sera. Lymphoproliferative disorders included in the study were acute lymphoblastic leukaemia (ALL), non Hodgkin's Lymphoma (NHL), chronic lymphocytic leukaemia (CLL), Hodgkin's disease (HD), Burkitt's lymphoma (BL) and multiple myeloma (MM). Screening was done by the Reverse Passive Haemagglutination method using the Welcome kit. The percentage antigenaemia in the patients and control subjects were 35.6 and 7.7% respectively (p less than 0.0001). Using the Odds ratio the relative risk was found to be 6.75. The Odds ratio for individual disorders ranged from 2.8 to 9.17. The results suggest an association between Hepatitis B surface antigenaemia and malignant lymphoproliferative disorders and highlights the risk involved in handling specimens from the patients.

Lymphomas in University College Hospital, Ibadan, Nigeria.

The authors reviewed 508 cases of lymphoma seen during a 16-year period in University College Hospital, Ibadan, Nigeria. Hodgkin's disease was diagnosed in 95 (18.6%) patients, of which 45% had mixed cellularity type; 19%, lymphocyte predominance; 18%, nodular sclerosis; and 18%, lymphocyte depletion. The age distribution showed a single mode in the 11 to 25-year-old group. Male preponderance was more marked in children who made up 32% of the Hodgkin's disease patients. Non-Hodgkin's lymphoma occurred in 413 cases, of which 296 (71.7%) were high-grade malignancies. Burkitt's tumor occurred in 211 (41.5%) patients, 95% of whom were children, and showed a male to female ratio of 1.7:1. Compared with previous findings in this hospital, Hodgkin's disease showed increased proportions of the lymphocyte predominant and nodular sclerosing types. However, the characteristics of Burkitt's lymphoma did not change appreciably in the last three decades.

The prognostic value of serum immunoglobulin G and immune complex levels in acute lymphoblastic leukaemia.

Twenty-five newly diagnosed patients with acute lymphablastic leukaemia and an equal number of controls matched for age and sex were admitted to this study. Serum immunoglobulin G concentration was determined by single radial immunodiffusion and immune complex level by polyethylene glycol precipitation. The patients had significantly higher immune complex and lower immunoglobulin G levels than the controls. No significant correlation was observed between length of survival and serum concentrations of immunoglobulin G or immune complexes in patients who died during the period of study. Decreased immunoglobulin G and raised immune complex levels reduce the ability to mount an immune response and imply bad prognosis. However, their practical value for estimating the length of survival in acute lymphoblastic leukaemia is limited.

Prognosis of acute lymphoblastic leukaemia in Ibadan, Nigeria.

Patients with acute lymphoblastic leukaemia (ALL) seen in University College Hospital, Ibadan, Nigeria, still have low rates of complete remission and relatively short survival. Yet the overall prognosis was expected to have improved because the proportions of adults, males and people of low socio-economic class among the patients have decreased steadily over the past three decades. Possible causes of the persistent poor performance were sought for in 30 new ALL patients seen in the hospital over a period of 2 years and 9 months. Unfavourable prognostic factors, lack of standard cytotoxic drugs, inadequate supportive care and absence of modern facilities for therapy combined to make their disease outcome worse than expected.

Changing patterns of acute lymphoblastic leukaemia in Nigeria.

An increase in the number of Nigerian patients with acute lymphoblastic leukaemia (ALL), relative to those having acute myelogenous leukaemia, was predicted some years ago. This was expected to result from an enhanced socio-economic status of most members of the general population and improved nutrition. A review of the features of ALL patients seen in University College Hospital, Ibadan, during different periods over the past three decades revealed not only the predicted changes but also an increasing proportion of pediatric and female patients. Except for an unusual number of patients with the rare L3 morphological variant seen within the last 2 years, little else changed in the clinical and laboratory features of the disease. Atrophy of the thymus resulting from malnutrition is thought to inhibit the occurrence of common-ALL in the first decade of life. This inhibition is removed by improved nutrition, thus allowing more cases of common-ALL to develop in children. The trend in Nigeria has been observed earlier among Arabs in the Gaza Strip in the Sinai Peninsula. If the hypothesis is correct, undernutrition occurring for a long enough time in a previously well-nourished population should lead to changes opposite to those observed. The increasing percentage of patients who are either young, female or of high socio-economic class imply better prognosis for more Nigerian ALL patients.