HYPERTENSION AND DIABETES MELLITUS ARE ASSOCIATED WITH DEEP VENOUS THROMBOEMBOLISM: A CASE CONTROL STUDY.

Introduction: Identifying risk factors for venous thromboembolism (VTE) is useful in deciding thromboprophylaxis for VTE. A retrospective study had shown an association between hypertension and diabetes mellitus with VTE in our population. The objective of this study was to confirm these findings and to determine if the complete blood count and coagulation tests can also be useful parameters in stratifying VTE patients for prophylaxis. Methods: This is a gender and age matched prospective case-control study of 45 Doppler's confirmed DVT and 43 apparently healthy controls. Results: Identified risk factors included history of hypertension, diabetes mellitus, previous DVT, recent surgery, recent trauma, malignancy, sepsis, and immobility. The cases had a significantly lower mean haematocrit (33±7.4% vs 38±4.6%, p<0.001). Though no differences were observed in leucocyte and platelet counts between cases and controls but stratification as leucocytosis vs leucopaenia (P=0.003) and thrombocytosis vs thrombocytopaenia (P=0.045) differed between both groups. Also, the International normalized ratio (INR) was higher in cases (1.1±0.2 vs 1.0±0.1;P=0.001), hypercoagulable state (INR<0.9) and hypocoagulable state (INR>1.2) were observed in 4.4% and 28.9% of cases respectively but not in controls (P <0.001). Also, aPTT>40 seconds was seen in 4.4% vs 4.7% of cases and controls respectively and aPTT< 30 seconds in 22% of cases but not in controls (P =0.004). Conclusion: Hypertension and diabetes mellitus are identified risk factors not traditionally associated with DVT. These in addition to a complete blood count and coagulation tests can be useful in stratifying patients for prophylaxis in our population and other similar communities.

The Pattern of Haemoglobin Phenotypes and the Clinical Identity of Clients Tested at a Private Diagnostic Center in South-West Nigeria.

BACKGROUND: Haemoglobinopathies are diseases of global importance and the countries with high disease burdens have inaccurate national data on the incidence of the various haemoglobin (Hb) phenotypes. OBJECTIVE: To review the Hb phenotype distribution, the referral identity of patients, and the clinical reasons for phenotyping. METHODS: A retrospective descriptive study conducted at a private diagnostic laboratory in Lagos, South-West, Nigeria. The anonymous results of Hb phenotypes of the patients determined using gel electrophoresis were retrieved from the laboratory information system (LIS). RESULTS: There were 942 patients: 519(55.1%) males and 423(44.9%) females with median ages 30yrs (11months-89yrs) vs 27yrs (9months-89yrs), p=0.0018. The phenotypes were HbAA, 592(63.2%); HbAS(26.4%); HbSS(7.1%); HbAC(2.1%), HbSC(0.7%) and HbCC(0.4%). Of the total, 757(80.4%) were referred by health care providers and 185(19.6%) were selfreferred, and there was no association between gender and referral identity of patients. However, there were more males [353(46.6%)] with clinical reasons for referrals than females [94(12.4%)], p<0.0001, and annual check was the commonest reason for Hb phenotyping. CONCLUSION: Most patients were referred for Hb phenotyping and an annual health check is the commonest reason for phenotype requests. This emphasizes the need for policies to support national Hb phenotype/genotype screening programmes to aid the early detection of sickle cell disease.

KNOWLEDGE, ATTITUDE AND USE OF HYDROXUYREA AMONG ADULT SICKLE CELL DISEASE PATIENTS.

INTRODUCTION: Hydroxyurea is the only cytotoxic drug approved for use in the care of sickle cell disease patients. It has been found to be effective in fixed low dose in low resource economy where the cost of the drug and frequent monitoring may be a barrier. This study looked at the knowledge, attitude and use of hydroxyurea in a low resource country with a high burden of the disease. METHODS: Data was obtained using a self-administered questionnaire in 101 consecutive adult sickle cell disease patients in a tertiary health care facility. RESULTS: All the patients responded to the questionnaire, majority (67%) of the respondents were below 40years with equal numbers of male and female patients. Of the respondents, 15.5% have ≥ 3 painful crises in a year. Half of the respondents were not admitted in the preceding year with 6 (5.9%) of them having had ≥3 admissions. Less than a quarter (21.2%) had knowledge of hydroxyurea, but 52.5% were willing to use the drug while 4.3% had previously or are currently using it. Only 11 respondents were aware of the side effects and the information was mostly through their Physicians. Those willing to use the drug were also willing to attend follow-up clinic monthly. CONCLUSION: Though the awareness about hydroxyurea is low in our group of patients, many are willing to use the medication and attend follow up clinics. There is a need to create more awareness about the drug especially by Physicians who care for the patients.

Hypereosinophilic syndrome with severe hypokalaemia in a Nigerian woman: A case report.

Introduction: Hypereosinophilic syndrome (HES) is a rare disorder. It is defined as eosinophilia of greater than1.5x109 /L persisting for at least 6 months or death before 6 months without an identifiable cause and with eosinophil-mediated organ dysfunction. We present a rare case of hypereosinophilic syndrome with severe hypokalaemia in a Nigerian female patient. Case Presentation: A 43year old food vendor referred to the Haematology Department, University College Hospital, Ibadan on account of a 6-week history of cough productive of mucoid, brownish, foul smelling sputum with associated breathlessness, high grade intermittent fever, and intense pruritus. She had accompanying non-projectile,non-bloody vomiting of recently ingested meals. There was absolute eosinophilia of 83x109/L and bone marrow cytology revealed marked eosinophilia with blasts of less than 5%. She also had asymptomatic severe hypokalaemia (1.9mmol/l) likely due to vomiting and reduced dietary intake. The aetiology of the hypereosinophilia could not be ascertained.She was admitted and commenced on intranasal oxygen, Tabs Loratidine, intravenous hydration.The severe hypokalaemia was corrected with IV KCL over 48hours followed with the administration of slow K tablets 600mg tds. She also had tabs Hydroxyurea for cytoreduction and Allopurinol to prevent hyperuricaemia. She improved with the above line of management. Conclusion: This appears to be the first reported case of HES with asymptomatic severe hypokalaemia in the literature. Being a rare disorder it could easily have been missed without a review of the peripheral blood film and marrow aspirate. This finding suggests a possible relationship between hypereosinophilia and hypokalemia which needs to be explored.

ADDICTION OR PSEUDOADDICTION IN SICKLE CELL DISEASE PATIENTS: TIME TO DECIDE - A CASE SERIES.

OBJECTIVE: The objective of this report is to highlight the background factors associated with opioid abuse among Sickle Cell Disease (SCD) patients. PATIENTS: Eleven patients aged 13-53 years (mean, 26.1yrs) which included six female and five male were seen in the last six year at a tertiary health facility. The modes of abuse ranged from obtaining analgesic prescription from multiple sources, injecting analgesics and sharing analgesics between patients in the hospital. RESULTS: Five female had either avascular necrosis of the femoral head or pathological fracture secondary to chronic osteomyelitis, so were classified as pseudoaddiction while five male and one female without any identifiable cause of chronic pain were adjudged to be addicted to opioids. The role of a dysfunctional family background in the response of SCD patients to pain treatment and substance abuse is highlighted. CONCLUSION: These cases reveal drug abuse as an emerging or understudied problem among SCD patients and its association with chronic pain in some patients. It is therefore necessary for pain physicians and SCD experts to address the use of opioid in chronic sickle cell pain and provide alternatives and a suitable guideline for their use.

Steroid induced diabetes mellitus in patients receiving prednisolone for haematological disorders.

INTRODUCTION: Steroids are a useful component of combination chemotherapy or as a single agent in the treatment of haematological disorders even though there are adverse effects associated with its use. METHODS: We report four patients who developed diabetes mellitus (DM) during treatment with steroids for haematological disorders despite a negative history of DM. RESULTS: The mean age of the patients was 55 yrs and DM was diagnosed by fasting plasma glucose (FPG) after a cumulative steroid dose of 500-1800 mg. CONCLUSION: It is necessary to have a baseline and frequent FPG in patients who are considered for combination chemotherapy which include steroid since the development of DM does not appear to be dose dependent or related to history of DM in the patient or family.

A revisit of venous thromboembolism.

OBJECTIVE: To determine the effect of the introduction of Doppler ultrasonography to the management of venous thromboembolism in a health care facility in Nigeria. METHOD: A descriptive study of cases of venous thromboembolism (VTE) over a five year period (March 2007-February 2012) was compared with a five year review done two decades ago before the introduction of Doppler ultrasonography. RESULTS: This review showed an increase of VTE cases from 60 to 178 with a median age of 53 yrs, median age of female patients was 5 yrs younger than male patients with Male/female ratio of 1:1.2. There was an even distribution of patients seen over the five year period with a mean prevalence of 35.6 patients per year. Patients with distal deep vein thrombosis (DVT) were in the majority (76%) compared with proximal DVT and PE which were 21% Vs 3%. About 4% of the patients had a recurrence of the VTE with male patients having twice the likelihood of a re-thrombosis. Common comorbid states observed are cerebrovascular disease (14.5%), cancers (12.2%), retroviral disease (6.7%) and diabetes (6.2%). Prostatic cancer was more common than other cancers while patients with retroviral disease are more likely to have an extensive DVT. CONCLUSION: The introduction of Doppler ultrasonography increased the awareness and diagnosis of VTE, we suggest that patients with associated disease conditions are placed on prophylactic anticoagulation, it is also necessary to determine the prevalence of inherited causes of VTE in the population.

Beta thalassaemia in Nigeria: myth or fact?

BACKGROUND: The high prevalence of sickle cell disorders and the mild deletional á-thalassaemia among Nigerians is well known, but â-thalassaemia is believed to be almost nonexistent. Beta thalassaemia trait (BTT) was screened for in patients with unexplained recurrent haemolytic anaemia and healthy individuals. METHODS: â-thalassaemia trait (BTT) was screened for using MCH and HbA2 of 27 pg and 3.5% respectively as cut off in 151 Nigerians which included 29 patients and 122 apparently healthy individuals. The subjects were categorized into four, Group I (high HbA2, low MCH), Group II (low HbA2 and MCH), Group III (high HbA2 and high/normal MCH) and Group IV (normal HbA2 and MCH). RESULTS: Group I are possible carriers of BTT while group IV are least likely to carry either alpha or beta thalassaemia genes. There were 36 (26%), 39 (28%), 27 (19%) and 38 (27%) in groups I-IV respectively. The mean MCH, MCV, haematocrit and HbA, for the study population were 26 +/- 2.8, 81 +/- 7.9, 37.5 +/- 6.8 and 3.4 +/- 1.7 respectively. The mean MCV and haematocrit were significantly lower for group I compared to group IV (76.9 Vs. 86.6, p=0.00) and (36.5Vs39.7, p=0.03) respectively. Group II had significantly lower MCV and haematocrit than group IV (75.4 (p=0.00) and 36.4 (p=0.01) respectively. There was a positive correlation between the MCH and MCV with the haematocrit (p=0.004, p=0.001 respectively) but HbA2 showed a stronger negative correlation with the haematocrit (p<0.0001). CONCLUSION: This does not only show the presence of BTT, but a higher prevalence than previously thought, mutations responsible for it should therefore be characterized.

Beta thalassaemia in Nigeria: myth or fact?

BACKGROUND: The high prevalence of sickle cell disorders and the mild deletional alpha-thalassaemia among Nigerians is well known, but beta-thalassaemia is believed to be almost nonexistent. Beta thalassaemia trait (BTT) was screened for in patients with unexplained recurrent haemolytic anaemia and healthy individuals. METHODS: beta-thalassaemia trait (BTT) was screened for using MCH and HbA2 of 27 pg and 3.5% respectively as cut off in 151 Nigerians which included 29 patients and 122 apparently healthy individuals. The subjects were categorized into four, Group I (high HbA2, low MCH), Group II (low HbA2 and MCH), Group III (high HbA2 and high/normal MCH) and Group IV (normal HbA2 and MCH). RESULTS: Group I are possible carriers of BTT while group IV are least likely to carry either alpha or beta thalassaemia genes. There were 36 (26%), 39 (28%), 27 (19%) and 38 (27%) in groups I-IV respectively. The mean MCH, MCV, haematocrit and HbA2 for the study population were 26 +/-2.8, 81 +/- 7.9, 37.5 +/- 6.8 and 3.4 +/- 1.7 respectively. The mean MCV and haematocrit were significantly lower for group I compared to group IV (76.9 Vs 86.6, p = 0.00) and (36.5Vs39.7, p = 0.03) respectively. Group II had significantly lower MCV and haematocrit than group IV (75.4 (p = 0.00) and 36.4 (p = 0.01) respectively. There was a positive correlation between the MCH and MCV with the haematocrit (p = 0.004, p = 0.001 respectively) but HbA2 showed a stronger negative correlation with the haematocrit (p < 0.0001). CONCLUSION: This does not only show the presence of BTT, but a higher prevalence than previously thought, mutations responsible for it should therefore be characterized.

Phenotypic and genotypic expression of alpha thalassaemia in Ibadan, Nigeria.

BACKGROUND: Alpha thalassaemia is a common monogenic disorder found in most parts of the world but the severe form is restricted in its distribution while the mild deletional form of alpha- thalassaemia has a wider geographical distribution. METHODS: One hundred and fifty one Nigerians which included 29 patients with unexplained recurrent haemolytic anaemia had their a-thalassaemia status determined by gap PCR after being screened using red cell indices. RESULTS: Only the -alpha(3.7) deletion occurs among Nigerians with a gene frequency of 0.21. Forty-two per cent of the study population are heterozygotes, 9% are homozygotes while 49% are normal for this deletion. The mean haematocrit for the study population is 37.5%: homozygote, heterozygotes and normal subjects had a mean haematocrit of 37.3%, 37.5% and 37.7% respectively. The mean mean corpuscular haemoglobin (MCH) and haematocrit are significantly lower for patients than controls (24 +/- 2.9 pg vs. 26.6 +/- 2.5 pg (p = .000), 29% vs. 40% (p = .000) respectively). Forty three percent of both patients and controls had MCH of less than 27 pg but only 10% of patients had MCH that is greater than 27pg. Homozygote patients had a higher mean haematocrit than patients who are normal for the deletion (34.7% vs. 25.4%, p = 0.06) but homozygote patients had a lower mean haematocrit than homozygote controls (34.7% vs. 38.5%, p = 0.4). CONCLUSION: This would suggest that alpha-thalassaemia is not responsible for the recurrent haemolytic anaemia observed in these patients and that the high prevalence of microcytosis among the normal populace needs further exploration.

Thalassaemia is a tropical disease.

Genes for thalassaemias, sickle cell disorders and Glucose-6- phosphate dehydrogenase (G6PD) deficiency are known to be associated with prevalent malaria infection. The prevalence in the heterozygote state for sickle cell anaemia (SCA), G6PD and alpha thalassaemia is between 25-30% in Nigerians but the prevalence for the beta thalassaemia trait (BTT) is low. Under-diagnosis of BTT may arise from the similarity in its clinical manifestation to that of SCA which is of high prevalence in Nigeria and secondly because the hypochromia and microcytosis associated with it may be misdiagnosed as iron deficiency anaemia. There is therefore the need to review this disorder in the light of the wide use of automation in processing a full blood count which will include red cell indices, a good screening method for the thalassaemias. This expectedly will aid easy and early diagnosis of the disorder.

Audit of the red cell units supply of a busy hospital blood bank in Nigeria.

BACKGROUND/OBJECTIVE: Blood bank audit embraces all procedures from blood procurement to the long -term consequences of transfusion which helps to identify opportunities for improvement. We have therefore looked at red cell transfusion in our hospital to identify areas that will require improvement. METHOD: Data was collected retrospectively from the hard copy record of the blood bank for a period of one month. RESULTS: Request was made for 1210 red cell units; crossmatch was made for 64% of the request. All units crossmatched were issued, of these 4.15% were returned unutilized. 5.1% were issued as uncrossmatched. The Obstetrics/Gynaecology unit recorded the highest blood usage and the medical unit the least. The use of blood by the emergency and paediatric were the same. The crossmatch:transfusion ratio for the hospital was 0.9:1. This study has shown that the crossmatch and transfusion rates are almost equal and only a small percentage was returned unused. CONCLUSION: This showed that there is inadequate supply of blood. There is a need to assess the reason for transfusion and establishing a blood transfusion advisory committee by the hospital. This will ensure interaction and collaboration between blood suppliers and users to improve the quality of transfusion practice. The new policy establishing a national transfusion service will also improve the transfusion service but emphasis has to be put on donor recruitment and donor care.

Automated techniques in haematology.

BACKGROUND: Manual techniques have been mostly in use in developing countries until recently because of its low cost even though it is laborious. Many laboratories are however changing to automated techniques since many samples can be analysed within a short time. It is likely that more laboratories will use these instruments either as semi automated or fully automated multichannel instruments. There is therefore a need for laboratories to become more responsive to the needs of physicians making request by providing help in the presentation and interpretation of results. METHOD: Available literature on automation in Haematology was sourced for using both manual library search and Medline search. RESULTS: Quality control in automated blood cell counts and related topics are extensively researched but there is still dearth of knowledge on automation in other areas of Haematology and on the clinical implications of automation. CONCLUSION: It is therefore necessary to make provision for automated facilities in laboratories in view of the high precision and cost effectiveness of these machines. This review is geared towards a critical look at this technology and its application to disease states.

The pregnant Rhesus negative Nigerian woman.

OBJECTIVE: Alloimmunisation to Rhesus D (RhD) is a major factor in perinatal morbidity and may result in the compromise of the woman's obstetric career. In Nigeria accurate population based studies to determine the prevalence of Rhesus negative women and the incidence of alloimmunisation are lacking, hence we undertook to study pregnancy outcome in Rhesus negative women. METHOD: We studied retrospectively sixty-seven RhD negative women over a two year period; information was obtained from the case-file of all pregnant women who presented to the ante-natal clinic and were identified as Rhesus negative. This was corroborated with the blood bank record over the same period. RESULT: Forty per cent of these were nulliparae with an average of 0.5 abortions. Only 20% had the blood groups of their husbands documented, and only four babies born to these women had their Rhesus group recorded. Six of the babies appeared to have been severely affected by Rhesus isoimmunisation. Three of these had an exchange blood transfusion (EBT); all who had an EBT had a satisfactory outcome. Out of the other three, there were two neonatal deaths and one fresh stillbirth. Fourteen babies had neonatal jaundice with a mean bilirubin level of 6 mg/dl, all of which were mainly unconjugated. Evidence for the administration of anti D was obtained in only three patients; all had one ampoule (dose in i.u and/or _g not stated) administered within 72 hr. CONCLUSION: This preliminary study has shown that isoimmunisation due to Rhesus incompatibility is poorly studied among Nigerian women with many questions unanswered; therefore there is an urgent need for a management protocol for this condition, which will include both the clinicians and the laboratory physicians.

Management of acute painful crises in sickle cell disease.

Pain is a common mode of manifestation of sickle cell disease (SCD) but there is limited information on pain management in this disorder. This study examines the use of opioids and non-opioid analgesia in the management of painful crisis in adult SCD patients; the routine use of antimalarials and antibiotics as adjunct therapy was also examined. A total of 87% of the patients had had a form of analgesics before presentation, 20% of which had parenteral analgesia. Ten per cent had not used any form of medication while another 10% used non-steroidal anti-inflammatory drugs. When asked, 59% of the patients desired oral non-opioid analgesics while 31% were not concerned about the type of analgesic given. Only 8% requested opioids. Hospital admission was not necessary in 65% of the patients; they were observed in the day-care unit and allowed home within 24 h. Sixty per cent did not have a test for malaria; 66% of those who had the test performed were negative, 35% of those whose thick film for malaria was negative had antimalarials prescribed. Only five patients (7%) were febrile at presentation. Thirty-four per cent had antibiotics prescribed, a third of these parenterally. Thirty-nine per cent had no fever but received antibiotics.

The Lupus Anticoagulant in a Population of Healthy Nigerian Adults

Patients with the antiphospholipid syndrome are at a high risk of recurrent thrombosis and recurrent fetal loss. Infertility and a number of other clinical manifestations have also been attributed to this syndrome. There are many tests for detecting the presence of the lupus anticoagulant but the most sensitive remains controversial. In this study we have used a combination of activated partial thromboplastin time (aPTT) and Kaolin clotting time (KCT) to determine the presence of the antibody in 125 healthy individuals which included pregnant women. Six (4.8) and four (3.2) of the subjects had elevated aPTT and a KCT ratio 1.2 respectively. The tests showed a high prevalence of the lupus anticoagulant in the multiparous group than the other groups while there is paucity of the anticoagulant in the pregnant women who are not at risk. We suggest the use of both aPTT and KCT for the screening of patients in whom the antiphospholipid syndrome is suspected. (SLE) [2] is sought for when a clinician suspects the presence of the inhibitor due to its recognized clinical features or an unexplained prolonged Activated Partial Thromboplastin Time (aPPT). There are various tests available for detecting the presence of the lupus anticoagulant but the most sensitive remains a controversy. The tests used are the aPPT [3]; kaolin clotting time (KCT)[4]; dilute Russell's viper venom time (RVVT)[5]; and the platelet neutralization test (PNT)[6]. At least two of these tests must be performed when investigating a patient suspected of having the lupus anticoagulant [6]. Detection of anticardiolipin antibody using enzyme linked immunosorbent assay is another test which is sensitive to the presence of the anticoagulant but this may not be practicable in a developing country set up. In this study the presence of the lupus anticoagulant was sought for in healthy individuals using the aPTT and the KCT

Survival advantage in female patients with sickle cell anaemia.

OBJECTIVE: To evaluate the clinical and laboratory features of adult sickle cell anaemia patients in Nigeria. DESIGN: A cross-sectional study. SETTING: Haematology clinic of the University College Hospital, Ibadan, Nigeria. SUBJECTS: Sixty nine adult sickle cell anaemia patients randomly sampled. RESULTS: The mean steady state haematocrit, HbA2 and HbF were 22%, 4.6% and 7% respectively. Twenty five per cent of the patients had never received blood transfusion and a similar proportion recorded an infrequent occurrence of painful crises. CONCLUSION: An improved survival was observed with female patients showing an age related survival advantage over the males. Persistent splenomegaly was observed less frequently in the Nigerian sickle cell population despite the high frequency of alpha thalassaemia in the region. This was thought to be due to the lower levels of HbF in this population.

Hodgkin's disease after treatment for Burkitt's lymphoma: case report.

Hodgkin's disease and non-Hodgkin's lymphomas are interrelated disorders which have been reported to occur either simultaneously or sequentially in the same patient. We report here the development of nodular sclerosing type Hodgkin's disease in a patient two decades after successful treatment for Burkitt's lymphoma with cyclophosphomide and abdominal resection (AR). While the onset of symptoms after treatment for Burkitt's lymphoma was seven years definitive diagnosis of Hodgkin's disease was only made 22 years after the initial diagnosis of Burkitt's lymphoma. The recurrent and solitary nature ofthe lymphadenopathy and the fact that it was initially reported as reactive hyperplasia is typical of nodular lymphocyte predominant Hodgkin's disease. We believe that there was a transitory period of the malignancy as nodular lymphocyte predominant Hodgkin's disease.