Haematological Values in Sickle Cell Anaemia in Steady State and During Vaso-occlusive Crisis in Benin City, Nigeria

Background:Sickle cell anaemia (SCA) is a major cause of morbidity and mortality in Africa where there is no readily available effective treatment. This study was designed to determine the haematological values that can be used in monitoring the status and management of SCA patients. Method: A prospective study of 200 patients (81.3%) in steady state, 46 patients (18.7%) during vaso-occlusive crisis (VOC) and 84 control subjects seen between August 2001 and July 2002 in 3 centers in Benin City, Nigeria had their blood samples analyzed within two hours of collection. Automated Coulter Counter was used to determine the complete blood counts while the foetal haemoglobin (HbF) was estimated by the modified Betke method and haemoglobin A2 by HbS-free microcolumn chromatography. Results: The mean cell volume (MCV), mean cell haemoglobin (MCH) and mean cell haemoglobin concentration (MCHC) in steady state were 79.38fl \ub1 22.41, 28.31pg \ub1 3.58 and 32.56g/dl \ub1 2.27 while in VOC they were 85.50fl \ub1 8.14, 28.79pg \ub1 2.78 and 33.76g/dl\ub13.44 respectively. The red cell distribution width (RDW), haemoglobin A2 and F in steady state were 23.76% \ub1 6.49, 4.52% \ub11.16 and 2.17% \ub11.81 while during VOC they were 21.62% \ub15.11, 3.82%\ub11.27 and 2.05%\ub11.19 respectively. The neutrophil count (P<0.01), MCV (P<0.01) and MCHC (P<0.05) were significantly higher during VOC than steady state while the RDW (P<0.05) and haemoglobin A2 were significantly higher in steady state than during VOC. Conclusion:Parameters, which are not usually reported in previous studies, have been shown in steady state and VOC.Introduction : La dr\ue9panocytose est la cause principale de la morbidit\ue9 et mortalit\ue9 en Afrique o\uf9 il n' y a aucun traitement efficace facilement dispobible. L'objet de cette \ue9tude est de d\ue9terminer des valuers h\ue9matologiques qu'on pourrait utiliser dans la surveillance du statut et la prise en charge des patients atteints de la dr\ue9panocytose. M\ue9thode : Une \ue9tude potentielle compos\ue9e de 200 patients soit 81,3% dans un \ue9tat normal, 46 patients soit 18,7% pendant la crise vasco-occlusive (VOC) et 84 sujets cont\uf4les vus entre ao\ufbt 2001 et juillet 2002 dans 3 centres \ue0 la ville de Benin, Nig\ue9ria avaient eu leur prise du sang analys\ue9 en moins de deux heures de la collection D\ue9compte Coulter automatis\ue9 \ue9tait utili\ue9 pour d\ue9terminer le d\ue9compte du sang total tandis que l'h\ue9moglobine foetale (hbF) \ue9tait \ue9valu\ue9e \ue0 travers la chromatographie microcolumn libre \u2013 HbS. R\ue9sultats : Le moyen du volume de la cellule (MVC), d'h\ue9moglobine cellulaire moyenne (HCM) et la concentration h\ue8moglobine cellulaire moyenne (CHCM) dans un \ue9tat stable \ue9taient 79,38fl+- 22,41, 28,31pg +- 3,58 et 32,56/dl+- 2,27 tandis que dans le VOC ils \ue9taient 85,50fl+- 8;14,28,79pg +- 33,76g/dl+- 3,44 respectivment. La largeur de la distribution du globule rouge (LDGR), h\ue9moglobine A2 et F dans un \ue9tat stable \ue9taient 23,76 %+-6.49,4,52%+-1,16 et 2,17%+-1,81 tandis que pendant le VOC ils \ue9taient 21,62%%+- 5,11, 3,82%+- 1,27 et 2,05%+-1,19 respectivement, le d\ue9compte neutrophil, (P<0,01), MVC (P<0,01) et CHCM (P<0,05) \ue9taient sensiblement tr\ue8s \ue9lev\ue9 pendant VOC que \ue9tait stable tandis que la LDGR (P<0,05) et h\ue9moglobine A2 \ue9taient sensiblement plus \ue9lev\ue9e dans l'\ue9tat stable plus que pendant VOC. Conclusion :Des param\ue8tres, qu'on n'avait pas rapport\ue9 normalement dans des \ue9tudes pr\ue9c\ue9dentes, ont \ue9t\ue9 montr\ue9s dans l'\ue9tat stable et VOC

Two Rare Presentations of Plasma Cell Neoplasm

A case of extramedullary plasmacytoma (EMP) in a 50year old woman and another case of solitary plasmacytoma of bone (SPB) with myeloma in a 34year old young man is reported. Atypical features of the SPB with myeloma patient were: a) age of the patient; b) lack of typical features of multiple myeloma (renal failure, hypercalcaemia and bone pain). The maxillary sinus was the site of the EMP who was referred for surgical excision. Biochemistry and haematological parameters were within normal range for both cases. Monoclonal paraprotein on serum electrophoresis was absent in EMP while it was present in SPB. Radiotherapy alone was given to the EMP patient while four courses of VAD (vincristine, adriamycine and dexamethasone) chemotherapy in addition to radiotherapy was given to the SPB with myeloma patient. Although EMP and SPB are both localized forms of plasma cell dyscrasias, SPB seems to have a greater tendency to multiple myeloma.Il s'agit d'un cas du plasmacytome extram\ue9dullaire (PEM) chez une femme \ue2g\ue9e du 50 ans et un autre cas du plasmacytome solitaire d'os (PSO) avec my\ue9lome chez un jeune homme \ue2g\ue9 de 34 ans, Un trait typique du PSO chez un patient atteint du my\ue9lome \ue9taient (a) \ue2ge du patient, (b) p\ue9nurie des traits typiques de my\ue9lome multiple (insuffisance r\ue9nale, hypercalc\ue9mie et douleur d'os). Le sinus maxillaire \ue9tait le si\ue8ge de PEM qui \ue9tait envoy\ue9e pour une excision chirurgicale. Biochimie et param\ue8tre h\ue9matologiques \ue9taient dans des limites de la tranche normale des deux cas, paraprot\ue9ine sur le s\ue9rum \ue9lectrophor\ue8se \ue9tait absent en PEM tandis qu'il est pr\ue9sent chez PSO. Le patient atteint du PEM avait eu la radioth\ue9rapie seulement tandis que quatre cours de VAD (vincristine adriamycine et dexamethasone) chimioth\ue9rapie en plus de la radioth\ue9rapie \ue9tait donn\ue9 au patient my\ue9lome avec PSO. Quoique PEM et PSO les deux sont des formes localis\ue9es de plasma cellule dyscasie. Il parait que le PSO a une tendance plus \ue9lev\ue9e pour my\ue9lome multiple

Stroke prevalence amongst sickle cell disease patients in Nigeria: a multi-centre study

Background: Stroke is a life-changing, debilitating complication of sickle cell disease (SCD). Previous studies had recorded high stroke prevalence amongst this group of patients. Nigeria has a large population of people affected by this condition and this study aims to assess the stroke prevalence in this large population.Methodology: Stroke prevalence data from 14 physicians working in 11 tertiary health centres across the country was collated by doctors using the sickle cell registers and patient case notes. This data was then collated and used to obtain the overall stroke prevalence in adult and children.Results: The stroke prevalence in sickle cell disease patients in Nigeria was observed to be 12.4 per 1000 patients. Prevalence in the adult patients was 17.7 per 1000 patients and 7.4 per 1000 patients in children. Twenty three percent of the affected patients had more than stroke episode.Conclusion: The stroke prevalence in Nigeria is lower than previously recorded rates and further studies will be required to investigate other factors which may play a role.Keywords: sickle cell, stroke, Nigeria, prevalenc

CASE REPORT - Maxillary Herpes Zoster with Corneal Involvement in a HIV Positive Pregnant Woman

Corneal involvement in maxillary herpes zoster is very rare. This report presents the case of a 32 years old 7 months pregnant para2+1 female, who presented with vesiculopapular rashes with hyperpigmented crusts over the maxillary area of the face on the left side with periocular oedema, conjunctivitis and mild punctate keratitis in the left eye. She was HIV positive and was on treatment with the highly active antiretroviral therapy. She was treated with topical and systemic acyclovir with rapid resolution of the ocular features. Les herpès maxillaries de zona avec les atteintes cornéennes chez la femme enceinte séropositive L\'atteinte cornéenne dans les herpès maxillaries de zona est très rare. Le cas d\'une femme pare2+1 âgée de 32 ans et qui est enceinte de 7 mois, qui a présenté des éruptions papulovésiculeuses avec des croûtes hyperpigmentés à travers la région maxillaire à côté gauche du visage avec l\'oedème périoculaire, la conjuctivite, une kératite ponctuée faible dans l\'oeil gauche. Elle était séropositive et suivait un traitement à l\'aide de la thérapie antirétrovirale hautement active. Elle a été traitée à l\'aide de l\'acyclovir systémique et topique avec une résolution rapide des manifestations oculaires Keywords: maxillary, herpes zoster, HIV positive, cornea African Journal of Reproductive Health Vol. 11 (1) 2007: pp. 133-13

Ophthalmic Manifestations of Lymphoma

Background: Ophthalmic involvement in lymphoma is a relatively rare condition that can result from a primary intraocular lymphoma or an intraocular manifestation of systemic lymphoma. This report reviews the ophthalmic manifestations of lymphoma. Methods: Review of relevant information from journal articles and Internet search. Results: Almost all the structures in the orbit, adnexiae and eye can be involved in lymphoma. Lymphoma of the eye and adnexiae are most frequently of B lineage. Most of the ocular manifestations frequently masquerade as other more benign intraocular conditions including allergic or infectious conjunctivitis, uveitis, multiple evanescent white dot syndrome, acute retinal necrosis or herpetic retinitis. Correct diagnosis thus depends on a high index of suspicion and frequently requires radiologic imaging, histologic analysis, particularly vitreous biopsy or flow cytometry, subretinal aspiration and retinal biopsy. Diagnosis is often delayed and may lead to a fatal outcome. Recognition of its modes of presentation facilitates early diagnosis and treatment that may improve prognosis. Conclusions: It is important to review the ocular manifestations of lymphoma to assist the ophthalmologist to play a pivotal role in the prompt diagnosis and treatment of ocular lymphoma, and the haematologist/oncologist to recognize the need for a complete ophthalmic evaluation in the diagnosis, follow-up and management of lymphoma patients.Introduction: La participation ophtalmique dans lymphome est une condition rélativement rare qui peut être provoqué par lymphome intraoculaire primaire ou une manifestation intraoculaire du lymphome systématique. Ce rapport fait une rétrospective des manifestation ophtalmique du lymphome. Méthodes: Une rétrospective des informations pertinentes des articles dans les journaux et des recherches dans l'internet. Résultats: Prèsque toutes les structures dans l'orbite, adnexiae et l'oeil peuvent participer dans lymphome. Lymphome de l'oeil et d'adnexiae sont le plus souvent du lignage B. La plupart des manifestations oculaires les plus souvent mascarade comme d'autre conditions intraoculaire plus bénigne y compris des infections conjunctivite ou allergique, uveite, syndrome tacheté du blanc évanescent multiple, nécrose aigue rétine ou rétinite herpetique. Donc diagnostique correcte dépend d'index plus élevé du soupçon et l'imaging radiologique est très fréquemment éxigé, analyse histologique, la biopsie vitreouse en particulier ou bien écoulement cytométrie, aspiration sousrétine et la biopsie rétine. Le diagnostique est souvent rétardé et pourrait aboutir à un résultat sérieux. Réconnaissance de son mode de présentation facilitera un diagnostique précoce et un traitement qui pourrait améliorer la prognose. Conclusion: C'est très important de faire une rétrospective des manifestations oculaires du lymphome afin d'aider l'ophtamologiste de jouer un rôle clef dans un diagnostique précoce et traitement du lymphome oculaire, et pour aider l'hématologiste et oncologiste de réconnaitre le besoin pour une évaluation ophtalmique dans le diagnostique, des soins post-hospitaliers et la prise en charge des patients atteints du lymphome

Haematological values in sickle cell anaemia in steady state and during vaso-occulusive crisis in Benin City, Nigeria

Background:Sickle cell anaemia (SCA) is a major cause of morbidity and mortality in Africa where there is no readily available effective treatment. This study was designed to determine the haematological values that can be used in monitoring the status and management of SCA patients. Method: A prospective study of 200 patients (81.3%) in steady state, 46 patients (18.7%) during vaso-occlusive crisis (VOC) and 84 control subjects seen between August 2001 and July 2002 in 3 centers in Benin City, Nigeria had their blood samples analyzed within two hours of collection. Automated Coulter Counter was used to determine the complete blood counts while the foetal haemoglobin (HbF) was estimated by the modified Betke method and haemoglobin A2 by HbS-free microcolumn chromatography. Results: The mean cell volume (MCV), mean cell haemoglobin (MCH) and mean cell haemoglobin concentration (MCHC) in steady state were 79.38fl ± 22.41, 28.31pg ± 3.58 and 32.56g/dl ± 2.27 while in VOC they were 85.50fl ± 8.14, 28.79pg ± 2.78 and 33.76g/dl±3.44 respectively. The red cell distribution width (RDW), haemoglobin A2 and F in steady state were 23.76% ± 6.49, 4.52% ±1.16 and 2.17% ±1.81 while during VOC they were 21.62% ±5.11, 3.82%±1.27 and 2.05%±1.19 respectively. The neutrophil count (P<0.01), MCV (P<0.01) and MCHC (P<0.05) were significantly higher during VOC than steady state while the RDW (P<0.05) and haemoglobin A2 were significantly higher in steady state than during VOC. Conclusion:Parameters, which are not usually reported in previous studies, have been shown in steady state and VOC

Beta thalassaemia traits in Nigerian patients with sickle cell anaemia

Haematological values were determined in 246 sickle cell anaemia (SCA) patients in three centres in Benin City, Nigeria, as well as 84 control subjects with haemoglobin A (HbAA) confirmed by haemoglobin electrophoresis at pH 8.6. Automated Coulter counter was used to determine the complete blood counts while the foetal haemoglobin was estimated by the modified Betke method, and haemoglobin A2 by the HbS-free microcolumn chromatography. Six patients (2.4%), comprising of two males and four females, out of the 246 SCA patients were found to have elevated haemoglobin A2 (>3.5%). All the six patients also had elevated haemoglobin F (≥1.5%). The family members of three of these six patients were successfully screened. These three patients (1.2%) were found to have positive co-inheritance of thalassaemia trait and sickle cell anaemia. The erythrocyte indices were all reduced in these selected families except for one family whose mean cell haemoglobin concentration was within normal range. Peripheral blood film revealed the presence of target cells and occasional microcytes apart from the sickled cells. The possibility of co-inheritance of the beta thalassaemia gene with the sickle cell gene occurs in about 1.2% of Nigerians with sickle cell anaemia