Plasma L-Arginine in Sickle Cell Anaemia Patients in Crises and its Correlation with Markers of Severity of Disease.

INTRODUCTION/OBJECTIVES: Sickle Cell Anaemia (SCA) is a chronic haemolytic and inflammatory disorder characterized by repeated vaso-occlusive (VOC) and hyperhaemolytic crises (HC). These crises determine the quality of life of SCA patients. This study estimated the plasma L-arginine levels in SCApatients during crises (HC and VOC), and correlated these levels with the markers of inflammation in the patients. METHODS: A cross-sectional study comprising of 120 consenting adults (60 SCA patients and 60 HbAA controls). The SCA were grouped as SCA patients in VOC or SCA patients in HC and HbAA controls. All SCA patients presented at the emergency room or the daycare unit of Haematology Department, University College Hospital, Ibadan, Nigeria. Blood samples were collected and haematological parameters and plasma L-arginine levels were determined. RESULTS: There were 30 SCA patients in VOC, 30 SCA patients in HC, and 60 HbAA controls with age ranging from 18 to 58years. The controls had a higher haematocrit than the SCA patients (HbAA>SCA VOC> SCA HC, p<0.001) in each pair wise analysis. The VOC and the HC groups had higher mean White Blood Cell (WBC), platelet count, Absolute Neutrophil Count (ANC), Absolute Lymphocyte Count (ALC), Absolute Monocyte Count (AMC), and reticulocytes count than the HbAA controls. Similarly, the HC group had higher mean WBC, platelet count,ANC,ALC,AMC, and reticulocytes count, and a lower mean percentage irreversible sickle cell than the VOC group. The mean plasma L-arginine was significantly higher in the HbAA control group than those of SCA patients in crises (HbAA controls>SCAVOC>SCAHC, p < 0.001), and higher in the SCAVOC group than in the HC group. There was no significant correlation between plasma L-arginine levels, reticulocyte count, markers of VOC severity and markers of inflammations. CONCLUSION: The plasma L-arginine levels of SCA patients in VOC and HC were lower than those of the HbAA controls. The degree of reduction was however marked in patients in hyperhaemolytic crisis. There was no significant association between the plasma L-arginine levels and the markers of inflammation in the studied patients. The findings in this study buttress the need for consideration of L-arginine supplementation in patients with SCA, especially during acute hemolytic crises.

INTRODUCTION / OBJECTIFS: La SCA est un mal hémolytique et inflammatoire chronique caractérisé par des crises vaso-occlusives (COV) et hyperhémolytiques (HC) répétées, qui déterminent la qualité de vie des patients atteints de SCA. L'étude a estimé les taux plasmatiques de L-arginine chez les patients atteints de SCA pendant les crises (HC et COV), et a mis en corrélation ces taux avec les marqueurs de l'inflammation chez les patients. MÉTHODES: Une étude transversale comprenant 120 adultes consentants (60 patients SCA et 60 contrôles HbAA) regroupés en patients SCA en COV; Patients SCA à HC (tous les patients SCA présentés à la salle d'urgence ou à l'unité de garde du département d'hématologie, University College Hospital, Ibadan, Nigéria); et contrôles HbAA. Des échantillons de sang ont été prélevés et les paramètres hématologiques et les taux de L-arginine ont été déterminés. RÉSULTATS: Il y avait 30 patients SCA en COV, 30 patients SCA en HC et 60 contrôles HbAA avec un âge allant de 18 à 58 ans. Les témoins avaient un hématocrite plus élevé que les patients SCA (HbAA> SCA VOC> SCAHC, p <0,001) dans chaque analyse par paire. Les groupes VOC et HC avaient des taux moyens de globules blancs, de plaquettes, d'ANC, d'ALC, d'AMC et de réticulocytes plus élevés que les témoins HbAA. De même, le groupe HC avait un nombre moyen de globules blancs, de plaquettes, d'ANC, d'ALC, d'AMC et de réticulocytes plus élevés, et un pourcentage moyen de de drépanocytose irréversible plus faible que le groupe COV. La L-arginine plasmatique moyenne était significativement plus élevée dans le groupe témoin HbAA que chez les patients SCA en crise (contrôles HbAA> SCA VOC> SCA HC, p <0,001), et plus élevée dans le groupe SCA VOC que dans le groupe HC, p <0,001. Il n'y avait pas de corrélation significative entre les taux de L-arginine, le nombre de réticulocytes, les marqueurs de gravité des COV et les marqueurs d'inflammations. CONCLUSION: Les taux plasmatiques de L-arginine chez les patients SCAen COV et HC étaient inférieurs à ceux des contrôles HbAA, et le degré de réduction est cependant marqué chez les patients en crise hyperhémolytique. Il n'y avait pas d'association significative entre les taux plasmatiques de L-arginine et les marqueurs de l'inflammation chez les patients étudiés. Les résultats de cette étude renforcent la nécessité d'envisager une supplémentation en L-arginine chez les patients atteints de SCA, en particulier pendant les crises hémolytiques aiguës. MOTS CLÉS: anémie falciforme, L-arginine, crise vaso-oclussive, crise hyperhémolytique.

Haematology Consultations in a Tertiary Health Care Centre in Nigeria.

BACKGROUND AND OBJECTIVES: The ease with which investigations are done in this technological age increases the possibility of finding abnormal haematological values, which inevitably leads to haematologic consultations. The Department of Haematology at the University College Hospital (UCH), Ibadan provides a busy consultative service for in-patients and out-patients suspected of having a haematological disorder. This study reviewed the consultations received from different clinical departments to Consultant Haematologists at our tertiary hospital. METHODS: Analysis of consultation requests on in-patients received in the Haematology department, UCH between June 2015 and January 2016 was done. During this period, the department had six Haematologists and nine resident doctors. SPSS version 22 was employed for the statistical analysis. RESULTS: A total of 285 consultation requests were received during the study period, with an average of 36 consults per month. The highest number was received in July 2015. The patients reviewed had a mean age of 36.9±19 years, and requests on female patients exceeded those on males, with a M:F ratio 1:1.5. Consultation for a presumptive diagnosis of sickle cell disease (SCD) were the commonest indication (17%). The highest consultation was from Obstetrics and Gynaecology (O&G) (25%) while the least was from Radiation Oncology (0.4%). CONCLUSION: There is an increasing role for the Haematologist in sub-Saharan Africa as evidenced by the high frequency of consultations observed in this review. The management team of each teaching hospital should bear in mind the need to have adequate number of Haematologists so as to ensure optimal haematological services.

PATTERN OF ANAEMIA AND ITS CORRELATES IN NIGERIANS WITH HEART FAILURE.

BACKGROUND: Heart failure often coexists with many comorbidities, including anaemia. However, the pattern of anaemia in heart failure and its clinical and echocardiographic associations have not been adequately studied among Nigerians. OBJECTIVE: To describe the pattern of anaemia, its clinical characteristics, and its echocardiographic associations among heart failure subjects in Nigeria. METHODOS: One hundred and forty subjects with heart failure were recruited from the cardiology clinics of two teaching hospitals in southwest Nigeria: Ladoke Akintola University of Technology and Bowen University Teaching Hospitals, Ogbomoso. Complete blood analyses, among other tests, were done. Statistical analysis was done with Statistical Package for the Social Sciences (SPSS) 20.0. P <0.05 was taken as statistically significant. RESULTS: Anaemia, as defined by the World Health Organisation, occurred in 106 (75.7%) of the heart failure patients. The patterns of anaemia among participants include combined anaemia of chronic diseases (ACD) with iron deficiency in 64 (45.7%) patients, and ACD alone in 40 (28.6%). Anaemia was more significantly associated with previous diagnosis of diabetes mellitus, presence of pulmonary hypertension, and heart failure with reduced ejection fraction. Mean systolic and diastolic blood pressures, ejection fraction, and fractional shortening were significantly lower among heart failure subjects with anaemia, while serum creatinine, left atrial dimension, left ventricular end diastolic dimension, and left ventricular mass index were significantly higher among heart failure subjects with anaemia compared to those without anaemia. CONCLUSION: Anaemia occurs very frequently among heart failure patients in southwest Nigeria. It is associated with many poor prognostic factors, including diabetes mellitus, pulmonary hypertension, and kidney failure.

Hypereosinophilic atopic transverse myelitis.

Atopic transverse myelitis is a rare disorder that is defined as a localized myelitis of an unknown cause in patients with either high immunoglobulin E (IgE) level or mite-specific IgE or coexistent atopic disease. It is a cause of intramedullary cord lesions, but its diagnosis does not need tissue confirmation. We report a case of a patient who presented with bladder and anal incontinence, paresthesia, and lower limb weakness. Neither IgE level nor mite-specific IgE level could be determined due to lack of fund; however, magnetic resonance imaging (MRI) of the thoracolumbar region showed hypotense-isotense lesion within the spinal cord at T4 vertebral level, suggestive of transverse myelitis. Blood cell count showed hypereosinophilia. Therefore, a diagnosis of atopic transverse myelitis based on high eosinophil count and MRI was made. Patient was commenced on prednisolone and had good response to treatment. Complete blood count is a cheap simple diagnostic tool in resource-poor country to distinguish atopic transverse myelitis from other causes of intramedullary cord lesions.

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.