Myelodysplastic syndrome in pregnancy--a rare cause of severe anaemia in pregnancy

A 28-year-old woman presented with severe anaemia in pregnancy at a period of gestation of 20 weeks. She was immediately admitted and after proper investigation it was seen that her Hb was 3.5 g/dl and platelet count was 62,000/cmm, RBC showed normocytic, normochromic morphology. There was neither hepatosplenomegaly nor any purpuric spot over the body. Bone marrow showed dyserythropoiesis and its chromosomal study revealed monosomy-7. Her pregnancy was continued till term with repeated packed cell and platelet concentrate transfusions. Normal healthy baby was delivered by caesarean section and she was discharged after 6 days.     

Prevalence of HIV-related autoimmune haemolytic anaemia in Lagos,Nigeria

Background:

Despite a high frequency of anaemia, a positive direct antiglobulin test (DAT) and bone marrow hyperplasia HIV-infected patients, lack of reticulocytosis may cause underdiagnosis autoimmune haemolytic anaemia (AIHA) in them. This study was carried out to determine the prevalence of autoimmune haemolytic anaemia in HIV-infected patients and to compare the haematological/immunological characteristics of subjects with anaemia and those without.

Materials and Methods:

A total of 350 HIV-infected subjects attending the Lagos University Teaching Hospital who consented were recruited for the study. This included 250 subjects with anaemia (haemoglobin concentration <10 g/dl) as cases and 100 subjects without anaemia as controls. Five milliliters of venous blood drawn from each subject was used for the full blood count, reticulocyte count and DAT.

Results:

Subjects with anaemia had lower mean CD4 cell count (284.3 cells/μl) and higher mean reticulocyte per cent (1.5%) than the non-anaemic subjects. The frequency of reticulocytosis was higher in female subjects than in males. Only 0.8% (2 of 250) of the study group screened positive to DAT, p = 0.0339. None of the subjects in control group screened positive to DAT.

Conclusion:

Autoimmune haemolytic anaemia is a rare complication of HIV infection in our geographical location.     

Occlusal features of sickle cell anaemia patients in Lagos, Nigeria

One hundred and four sickle cell anaemia patients aged 10-45 years and attending the out patients' clinic of the Lagos University Teaching Hospital as part of a routine check-up were seen. They were in a steady state of health. By means of a structured questionnaire the Biodata of each consecutive eligible patient was obtained and physical examination was carried out. Oral examination was done with emphasis on occlusal variables including Angle's Classification of occlusion, overjet, overbite, tooth: bone ratio, lip competence and skeletal pattern. Results showed that the majority of the patients 92 (88.5%) seen presented with Angle's Class 1 malocclusion. Increased overjet was observed in 50 (48.2%) of the sample population while an overbite depth of 2 was most frequently seen in these patients. Spacing in the anterior segment of the upper and lower arches was a fairly common occurrence, occurring in 51 (49.0%) and 39 (30.8%) of the patients seen respectively. Normal dental base relationship (Skeletal 1) was observed in 72 (69.2%) while lip incompetence was seen in 41 (39.4%) of the patients examined. Occlusal anomalies vary from mild to severe and may be attributable to hyperplastic maxillary bone and the resultant skeletal discrepancy.     

Electrocardiographic changes in chronic severe anaemia

Electrocardiogram of total 35 patients of chronic severe anaemia with haemoglobin value < or = 5 g/dl was analysed and compared with same number of age and sex matched healthy subjects. Diminished QRS voltages in all limbs and precordial leads were the dominant finding, found in 100% patients. Compensatory hyperdynamic circulatory state with myocardial scarring and chronic fibrotic changes probably explains these ECG findings in this series of patients of chromic severe anaemia.     

Hepatitis C virus infection in patients with sickle cell anaemia at the Lagos University Hospital

The objective of this studies is to determine the frequency of antibodies to Hepatitis C virus (anti-HCV), (2) assess the role of blood transfusion in transmission of infection, and (3) evaluate the clinical implication of anti-HCV sero-positivity in patients with Sickle cell anaemia (SCA). Two hundred and seventy-eight (278) patients with SCA were evaluated by questionnaire interviews for risk factors, clinical examination and serum testing for anti-HCV using a third generation ELISA kit. The overall anti-HCV prevalence was 5.0% (14/278). Anti-HCV was positive in 7% (5/76) of never transfused compared with 5% (9/202) of previously transfused sicklers. (p = 0.5). Clinically, splenomegaly alone or in combination with hepatomegaly was associated with a positive anti-HCV (p = 0.04 and 0.01 respectively). Anti-HCV was detected in 5% of adult patients with Sickle cell anaemia, especially in patients with persistent splenomegaly alone or in combination with hepatomegaly. Blood transfusion did not appear to be the major route of HCV transmission.     

Successful pregnancy following aplastic anaemia.

Pregnancy following idiopathic aplastic anaemia is rare and is difficult to manage because of life-threatening episodes of bleeding and infections. Only a handful of cases has been reported in the literature. The pregnancies were unsuccessful in the majority. The present report describes a patient with moderately severe idiopathic aplastic anaemia who was managed with intensive haematological support leading to delivery of a healthy infant by caesarean section. Despite platelet transfusion refractoriness as a result of transfusions prior to pregnancy, adequate platelet transfusions prevented excessive bleeding. The literature is reviewed and management with platelet transfusions is discussed.     

Thymoma associated with severe diarrhoea and anaemia

About 40% of patients with thymoma have one or ,more paraneoplastic syndromes, including myasthenia gravis, pure red cell aplasia （PRCA） or hypogammaglobulinaemia, but Good syndrome occurs in only 5% of these conditions. To our knowledge, only 11 patients （including our cases） of thymoma accompanied with PRCA and Good syndrome have been reported, however, no such case was reported in China before 2000.     

异基因造血干细胞移植治疗重型再生障碍性贫血临床研究

目的 探讨异基因造血干细胞移植成功治疗重型再生障碍性贫血的疗效与治疗经验.方法 对15例重型再生障碍性贫血患者进行同胞间人组织相容性抗原(HLA)不全相合/HLA全相合造血干细胞移植,观察移植疗效及并发症,结合相关文献讨论分析.结果 移植后9～24 d(中位时间13.8 d)中性粒细胞大于0.5×109/L;移植后14～26 d(中位时间17.3 d)血小板大于20×109/L.2例于移植后26 d(HLA 3/6相合)和移植后48 d(HLA-A、DR各一个位点不合)发生Ⅱ度急性移植物抗宿主病(GVHD),予甲强龙治疗后控制;9例发生慢性GVHD,其中局限型6例(1例为HLA全相合,1例为HLA-B一个位点不合,4例HLA 3/6相合),广泛型3例[其中1例为HLA-DR一个位点不合,1例为4/6相合(HLA-A、B不合),1例HLA 3/6相合];4例发生肺部感染,3例发生肠道感染,1例巨细胞病毒感染;1例出现肝功能损害,无1例发生肝静脉闭塞病(VOD);随访3～37个月,14例患者无病生存,1例死于肺部侵袭性真菌感染.结论 异基因造血干细胞移植是治疗重型再生障碍性贫血的可靠方法,在充分考虑移植风险和积极准备的基础上可实施HLA不全相合造血干细胞移植.     

Determinants of anaemia in pregnancy in sekyere west district, ghana

SummaryAnaemia associated with pregnancy is a serious health problem and its control requires the initial identification of the major factors responsible. Haemoglobin (Hb) levels of 205 women, aged 15 to 49 years, in the last trimester of pregnancy and residing within 4 of 6 sub-districts in Sekyere West district of the Ashanti region of Ghana, were determined. Demographic characteristics, dietary habits and other information were obtained and analysed. Blood and stool samples were tested for the presence of malaria parasites and intestinal worms respectively. The data showed that, 57.1% of pregnant women had Hb<10g/dl, the Ministry of Health cut off point for anaemia. Anaemia was more prevalent in rural compared to urban parts of the district (p=0.01). Low parity and young age were significantly associated with low Hb and high prevalence of maternal anaemia. Presence of malaria parasites in peripheral blood constituted a significant risk for low Hb (Hb = 9.2g/dL vs 9.5g/dL p=0.03). Independent of this, hookworm was even more strongly associated with low Hb (Hb = 8.3g/dL vs Hb=9.5g/dL, p=0.01). Contribution of dietary intake to the prevalence of anaemia cannot be inferred from the data generated under the current study.     

异基因造血干细胞移植治疗重症再生障碍性贫血的评价

目的评价以环磷酰胺（CTX）为预处理方案行异基因造血干细胞移植（Allo—HSCT）治疗重症再生障碍性贫血（SAA）的疗效。方法对1例SAA患者行同胞供者Allo—HSCT治疗。预处理方案为CTX 50mg／kg^-1·d^-1×4d;干细胞来源采用外周血＋骨髓;输注单个核细胞数（MNC）为10．41×10^8／kg,CD34^＋细胞计数为6．86×10^6／kg。预防移植物抗宿主病（GVHD）采用环孢素A（CsA）加短程甲氨蝶呤（MTX）加霉酚酸酯（MMF）。结果患者获得造血重建,第14天中性粒细胞数（ANC）≥0．5×10^9／L、血小板计数（PLT）≥20×10^9／L,第96天血型转变为供者型（B→O）。患者出现Ⅳ度急性GVHD（aGVHD）,经积极治疗后控制。150d内患者出现急性化脓性扁桃体炎、口腔溃疡、急性支气管炎、带状疱疹病毒感染、巨细胞病毒血症、肺炎,经积极治疗后均好转。随访24个月,患者无病存活。结论以CTX为预处理方案allo-HSCT是治愈SAA的一种有效方法。