Bleomycin-induced pneumonitis in a young Ghanaian male with Hodgkin’s Lymphoma

We report a case of a young Ghanaian male who developed Bleomycin Induced Pneumonitis (BIP) after being treated for Hodgkin’s Lymphoma. Pulmonary toxicity is the most feared complication of bleomycin therapy despite its effectiveness in achieving cure in patients with Hodgkin’s lymphoma and germ cell tumors. BIP has a significant mortality rate if detected late and a high index of suspicion is required in all patients on bleomycin-based therapies with sudden onset of respiratory symptoms

Adherence to hydroxyurea therapy and health-related quality of life in children with sickle cell anaemia at Korle Bu Teaching Hospital in Ghana

Background: Sickle cell anaemia (SCA) causes numerous acute and chronic complications, which can result in significant morbidity and mortality. Hydroxyurea (HU) therapy in individuals with SCA is associated with a reduction in disease severity.Objective: The study aimed to determine the relationship between adherence to HU therapy and health-related quality of life (HRQOL) scores in children with SCA (HbSS) at Korle Bu Teaching Hospital (KBTH) and identify any barriers to HU adherence.Methods: A cross-sectional descriptive study was conducted at the paediatric sickle cell clinic of the KBTH. One hundred and fifteen children aged 2 – 12 years with HbSS receiving HU therapy and their primary caregivers were enrolled on this study. Demographic and HU-related data were obtained from medical records and interviews with caregivers. The 8-item Morisky Medication Adherence Scale (MMAS-8©) and haematologic response based on mean corpuscular volume (MCV) were used to assess HU adherence. Caregivers and children aged 8 - 12 years also completed the Paediatric Quality of Life Inventory (PedsQLTM) SCD-module version 3.0. For children aged 5 – 7 years, the PedsQLTM SCD-module version 3.0 was administered by interview.Results: Seventy-nine (68.7%) of children had high adherence to HU therapy using the MMAS-8©. The mean PedsQLTM scores were 96.9 ± 6.0 and 96.3 ± 7.2 for the child (n = 91) and caregiver (n = 114), respectively. Children with high HU adherence had significantly higher PedsQLTM scores than those with low or moderate adherence. Neither child's current MCV nor mean change in MCV correlated with child or caregiver PedsQLTM scores. The main barrier to HU adherence identified by children aged 8 years was forgetfulness, while the cost of HU was the main barrier to adherence reported by caregivers.Conclusion: Children with SCA with high adherence to HU had the highest HRQOL scores using the PedsQLTM SCD-module version 3.0. Routine assessment of barriers to HU adherence can provide important information to help guide relevant interventions

Sjögren's and plasma cell variant Castleman disease: a case report

Castleman disease is a rare cause of lymphoid hyperplasia and may result in localized symptoms or an aggressive, multisystem disorder. It can mimic other diseases like lymphoma or tuberculosis. It classically presents as a mediastinal mass that involves the lymphatic tissue primarily but can also affect extra lymphatic sites including the lungs, larynx, parotid glands, pancreas, meninges, and muscles. In HIV and HHV8-negative patients with idiopathic multicentric Castleman disease, pathogenesis may involve autoimmune mechanisms. We highlight and report a case of a 34-year-old Ghanaian female who was successfully diagnosed and managed for Sjögren's as well as plasma cell variant Castleman disease with combination chemotherapy and rituximab followed by eighteen months maintenance therapy with pulse chlorambucil and prednisolone and three monthly rituximab.Keywords: lymphadenopathy, chemotherapy, Rituximab, Plasma Cell Variant Castleman Disease, Sjögren's syndrom

Steroid-induced dysglycaemia in patients with haematological disorders a ten-year review in a tertiary hospital in Ghana

Background: Glucocorticoids (steroids) play a key role in the management of multiple medical conditions including haematological disorders. This study looked at the prevalence of steroid induced dysglycaemia in patients with haematological disorders receiving steroids as part of their treatment with the view of modifying its use and selection of patients where necessary.Methods: A retrospective review of haematology patients on treatment regimens including steroids. Information extracted included, demographic characteristics, clinical information such as age, gender, haematological disorder, type of steroid, daily and cumulative dose of steroid, duration of therapy, family history of diabetes and alcohol use.Results: The case records of 351 haematology patients were reviewed. However, eight patients with dysglycaemia before therapy were excluded. The median age of patients was 51.0 ± 26.0(IQR: Interquartile Range) years, with an age range of 13 to 87 years, and a female: male ratio of 1.2: 1 (p= 0.778). The prevalence of Steroid-Induced Dysglycaemia(SID) was 3.79% with a mean diagnosis interval of 8.8 + 2.1 months. Overall, 245 (71.4%) patients were on continuous steroids. Among the 13 patients who developed SID, 11 (84.6%) were on continuous steroids. In the majority of the patients (97.1%) there was no family history of diabetes in a first degree relative. Significant differences were found between patients with normoglycaemia and those with dysglycaemia with respect to age (p=0.049) and duration of steroid therapy (p=0.024).Conclusion: The prevalence of steroid-induced dysglycaemia is relatively low among Ghanaian patients with haematological disorders on steroid based chemotherapy.Keywords: steroids, haematological disorders, dysglycaemia, Ghana, risk factors.Funding: None declare

Establishing Ghanaian adult reference intervals for hematological parameters controlling for latent anemia and inflammation

CITATION: Bawua, A. et al. 2020. Establishing Ghanaian adult reference intervals for hematological parameters controlling for latent anemia and inflammation. International journal of laboratory hematology, 42(6):705–717. doi:10.1111/ijlh.13296The original publication is available at https://onlinelibrary.wiley.com/journal/1751553xBackground: In Ghana, diagnostic laboratories rely on reference intervals (RIs) provided by manufacturers of laboratory analyzers which may not be appropriate. This study aimed to establish RIs for hematological parameters in adult Ghanaian population. Methods: This cross-sectional study recruited 501 apparently healthy adults from two major urban areas in Ghana based on the protocol by IFCC Committee for Reference Intervals and Decision Limits. Whole blood was tested for complete blood count (CBC) by Sysmex XN-1000 analyzer, sera were tested for iron and ferritin by Beckman-Coulter/AU480, for transferrin, vitamin-B12, and folate was measured by Centaur-XP/Siemen. Partitioning of reference values by sex and age was guided by “effect size” of between-subgroup differences defined as standard deviation ratio (SDR) based on ANOVA. RIs were derived using parametric method with application of latent abnormal values exclusion method (LAVE), a multifaceted method of detecting subjects with abnormal results in related parameters. Results: Using SDR ≥ 0.4 as a threshold, RIs were partitioned by sex for platelet, erythrocyte parameters except mean corpuscular constants, and iron markers. Application of LAVE had prominent effect on RIs for majority of erythrocyte and iron parameters. Global comparison of Ghanaian RIs revealed lower-side shift of RIs for leukocyte and neutrophil counts, female hemoglobin and male platelet count, especially compared to non-African countries. Conclusion: The LAVE effect on many hematological RIs indicates the need for de-liberate secondary exclusion for proper derivation of RIs. Obvious differences in Ghanaian RIs compared to other countries underscore the importance of country-specific RIs for improved clinical decision-making.https://onlinelibrary.wiley.com/doi/10.1111/ijlh.13296Publishers versio

One third of alloantibodies in patients with sickle cell disease transfused with African blood are missed by the standard red blood cell test panel

Studies on red blood cell (RBC) antibodies in Africa routinely use standard test cells from donors of Caucasian descent. There are no systematic data on alloimmunization against antigens that are almost exclusively present in Africans. We studied the prevalence of antibodies in transfused Ghanaian patients with sickle cell disease (SCD) using standard test cells (representing predominantly antigens more common in Caucasians (Caucasian antigens) and cells expressing antigens more common among Africans (African antigens). Antibodies were present in 16% of 221 patients; 31% of these were directed against African antigens that were not detected with standard test cells. Our findings are not only relevant for an African setting, but also for Western blood banks that are developing strategies to recruit more African donors

Lung Function Abnormalities in Sickle Cell Anaemia

Background. Abnormalities in lung function tests have been shown to commonly occur in a majority of patients with sickle cell disease (SCD) even at steady state. The prevalence and pattern of these lung function abnormalities have been described in other populations but this is unknown among our sickle cell cohort. There is generally little information available on risk factors associated with the lung function abnormalities and its relevance in patient care. Method. This was an analytical cross-sectional study involving 76 clinically stable, hydroxyurea-naive adult Hb-SS participants and 76 nonsickle cell disease (non-SCD) controls. A structured questionnaire was used to obtain sociodemographic data and clinical history of the participants. Investigations performed included spirometry, pulse oximetry, tricuspid regurgitant jet velocity (TRV) measurements via echocardiogram, complete blood counts, free plasma haemoglobin, serum urea, and creatinine. Results. Weight, BMI, mean FVC, and FEV1% predicted values were comparatively lower among the Hb-SS patients (p 0.05). The mean oxygen saturation was comparatively lower among Hb-SS patients (p < 0.001). Conclusion. Measured lung volumes were significantly lower in Hb-SS patients when compared to non-SCD controls and this difference was not influenced by anthropometric variance. Lung function abnormalities, particularly restrictive defects, are prevalent in Hb-SS patients but showed no significant association with recognized markers of disease severity

Burden of Sickle Cell Disease in Ghana: The Korle-Bu Experience

In Africa, sickle cell disease (SCD) is a major public health problem with over 200,000 babies born per year. In Ghana, approximately 15,000 (2%) of Ghanaian newborns are diagnosed with SCD annually. A retrospective review of medical records of all SCD patients aged 13 years and above, who presented to the sickle cell clinic at Ghana Institute of Clinical Genetics (GICG), Korle-Bu, from 1st January 2013 to 31st December 2014, was carried out, using a data abstraction instrument to document their phenotypes, demographics, attendance/clinic visits, pattern of attendance, and common complications seen. During the period under review 5,451 patients were seen at the GICG, with 20,788 clinic visits. The phenotypes were HbSS (55.7%) and HbSC (39.6%) with other sickle cell phenotypes (4.7%). Out of the 20,788 clinic visits, outpatient visits were 15,802 (76%), and urgent care visits were 4,986 (24%), out of which 128 (2.6%) patients were admitted to the Teaching Hospital for further management of their acute complications. There were 904 patient referrals (out of 5,451 patients) for specialist care; the 3 specialties that had the most referrals were Obstetrics and Gynaecology (168 patients), Orthopaedics (150 patients), and Ophthalmology (143 patients). In 2014, complications seen at KBTH included 53 patients with avascular necrosis (AVN) and 61 patients with chronic leg ulcers. Our centre has a large number of patients living with sickle cell disease. From our experience, early recognition and referral of sickle cell related complications can reduce morbidity and mortality associated with this disease. A multidisciplinary approach to care of SCD patients is therefore important

A case-control and seven-year longitudinal neurocognitive study of adults with sickle cell disease in Ghana

Ageing in sickle cell disease (SCD) is associated with a myriad of end-organ complications, including cerebrovascular damage and cognitive impairment (CI). Although CI is very common in SCD, little is known about cognitive functioning and how it changes with age. This study examines cognitive patterns of 63 adults with SCD and 60 non-SCD, age- and education-matched controls in Ghana. Of those adults with SCD, 34 completed the neuropsychological battery at baseline and again seven years later. In cross-sectional data, adults with SCD performed worse than controls in all cognitive test domains (p &lt; 0.01 for all). The seven-year follow-up data showed that the group exhibited a significant decline in visuospatial abilities (ranging from Cohen's d = 1.40 to 2.38), and to a lesser extent, in processing speed and executive functioning. Exploratory analyses showed a significant time-by-education interaction, indicating that education may be protective from decline in cognitive performance. These findings have implications for clinical practice. Early neuropsychological surveillance coupled with early assessment and remedial programmes will provide avenues for enhancing the quality of life of adults living with SCD in Ghana.</p