Bone marrow invasion by aspergillus specie in a sickle cell trait patient with invasive aspergillosis: a fatal case in association with disseminated intravascular coagulation

Background: Invasive aspergillosis has been predominantly associated with pulmonary infection, particularly amongst immunocompromised individuals. Extrapulmonary infections with Aspergillus specie have been reported rarely irrespective of immune status. Risk factors for invasive aspergillosis include prolonged and severe neutropenia, haematopoietic stem cell and solid organ transplantation, advanced AIDS, and chronic granulomatous disease. The most frequently involved specie is Aspergillus fumigatus that constitutes over 90% of cases, followed by Aspergillus flavus, usually associated with a primary skin infection. Haematogenous spread to the bone causing osteomyelitis is the commonest form of disseminated aspergillosis and a surprisingly high proportion of these patients have no immunosuppression. We present a rare case of bone marrow invasion by Aspergillusspp. in a 3-year-old patient with sickle cell trait and chronic Aspergillosis. Case report: A 3-year-old patient with sickle cell trait was brought to the paediatric unit with recurrent diarrhoea, abdominal distention, weight loss and persistent cough. The child was severely wasted with generalised peripheral lymphadenopathy. She had marked respiratory distress and hepatosplenomegaly but no demonstrable ascites. Haematologic examination revealed leukaemoid reaction (leukocyte count of 44.0 x 109/L) with monocytosis (10%) and thrombocytopenia (platelet count of 97,000/mm3); no blast cells were seen on blood film. The bone marrow was hypercellular with a myeloid/erythroid ratio of 20:1, consistent with infection. Bone Marrow culture yielded Aspergillus spp. and other results of sepsis work up were negative. Conclusion: Cases of extrapulmonary invasive aspergillosis have been reported rarely in both immunocompetent and immunocompromised patients. Haematogenous spread to the bone is the commonest form of disseminated disease

Outcome of pregnancy in Saudi women with sickle cell disease attending the tertiary care university hospital in eastern province of Saudi Arabia

Sickle cell disease (SCD) is a chronic genetic hematological disorder with multiorgan involvement and is associated with complications during the pregnancy. This is a well-known disorder in Saudi Arabia, but no study has reported its outcomes in pregnant Saudi females of the Eastern region. This study was carried out to compare the fetomaternal outcome in patients with SCD with those without SCD. This was a retrospective cohort study done in the Eastern Province of Saudi Arabia in a tertiary care, teaching hospital, by retrieving the data through the code ICD-9 for SCD, the control group was also selected with comparable characteristics. A total of 302 SCD pregnant patients were included for comparison with 600 pregnant women without SCD as control, during the period of Jan 1, 2008 to December 31, 2018. After the data retrieval, percentages of complications were calculated between the study and control groups. Fischer's exact test and t-test were used for statistical analysis by using SPSS version 22. The results showed higher complication rates in pregnancies of patients with SCD. Hypertensive disorders (13.3%), abruptio placenta (1.6%), intrauterine growth restriction (19.2%), thromboembolism (6.6%) and stroke (2.6%) were all higher in SCD as compared to the control group .The complications of SCD itself including anemia (89.4%), acute chest syndrome (13.2%) and sickle cell crisis (39.2%) were also increased during the pregnancy. Both still birth (3.3%) and neonatal intensive care unit admission (1.6%) were also higher in SCD. SCD during the pregnancy is a high-risk situation and can lead to many fetomaternal complications; however, preconceptional counselling, early booking, a careful monitoring during pregnancy and multidisciplinary management approach can prevent potential adverse outcome in this regard

Effects of the Sikle Cell (S) Gene on Serum Protein Profile

Total serum protein, albumin, total globulin levels, albumin/globulin ratio as well as the various globulin fractions were determined in 96 subjects, 39 are sickle cell (SS) disease subjects (steady state), and 30 are heterozygous sickle cell (AS) trait and 27 normal control subjects. The mean standard deviation of total protein was significantly higher (P< 0.05) in sickle cell disease when compared with heterozygous AS and normal controls. There was also significant difference (P<0.05) between AS and AA. The albumin level in sickle cell disease was significantly higher than in the other two groups. There was hyperglobuneamia observed in SS individuals with a mean ±SD of 32.6±10.0g/L when compared with AS and AA subjects with a mean ±SD of 30.8± 5.9g/L and 26.7±6.2g/L respectively. There also exist a significant difference between AS and AA (P <0.005). The Albumin/Globulin ratio is significantly lower (P <0.05) in SS than the other two groups but there was no significant difference (P>0.05) observed between AS and AA controls. The globulin fractions were observed to be higher in AS individuals except the gamma globulin which is higher in SS subjects. The hyperproteineamia as a result of the hyperglobulineamia is due to the globulin fraction present in the serum of SS individuals. The AS individuals are protected from various infectious disease conditions because of the high acute phase reactants and ß- globulin present in their serum as these has been observed to exert some immunioregulatory role

When the Inheritance of two Heterozygote States become a Diagnostic Problem : Misdiagnosis of the Sickle Cell Trait

BACKGROUND : The sickle cell trait is a benign asymptomatic condition that should not ordinarily be associated with clinical manifestations of a haemoglobinopathy. METHOD : This is a case control study of sickle cell trait patients who presented with symptomatology of a haemoglobinopathy. HbA2; HbF and HbS levels as well as the haematocrit and the peripheral film pictures of 10 symptomatic individuals (patients) with the sickle cell trait were compared with those of 20 asymptomatic individuals (controls) with the sickle cell trait. RESULTS : the mean HbA2 of the cases was 4.9compared to the mean of 2.2 for the controls (p0.0001). Nine of the patients and none of the controls had a raised HbA2 ( 3.5). The mean HbF of the patients was 5.6 with a range of 1.2-14.0 while the mean of the control was 2.0 and a range of 0.7-8.4 (p=0.006). Six (30) of the controls had normal HbF level (1) while none of the patients had a normal HbF level. The mean haematocrit of the patients and controls were 0.33 and 0.37 respectively (p

La retinopathie chez le porteur du trait drepanocytaire AS : aspects cliniques et angiographiques

Notre etude; portant sur 75 cas de trait drepanocytaire AS; s'etait fixee pour but de contribuer a une meilleure connaissance de la retinopathie drepanocytaire. Dans cette forme Hb AS; les objectifs specifiques pour conduire cette etude se proposaient; apres l'identification et la classification des lesions retiniennes; d'evaluer les risques de COMPLICATIONS et de proposer un protocole therapeutique. A travers un examen complet clinique et ophtalmologique; nous avons confirme l'existence de retinopathie drepanocytaire chez les heterozygotes AS. Les caracteristiques epidemiologiques et cliniques suivantes ont ete retrouvees : l'age de nos patients variait de 6 a 59 ans avec une moyenne de 32;59 ans; 72 pour cent de notre effectif avait des lesions retiniennes; la frequence des porteurs de retinopathie etait plus importante apres 20 ans; les lesions non proliferatives etaient predominantes avec 49;33 pour cent contre 22;67 pour cent des lesions proliferatives; nous avons observe un cas d'hemorragie pre-maculaire; mais pas d'hemorragie intra-vitreenne et de decollement de retine; la retinopathie de l'heterozygote AS est moins importante et moins grave que celle observee dans les formes drepanocytaires majeures. Nous suggerons : la multiplication et l'equipement des services d'ophtalmologie notamment en angiographie et en laser; un renforcement de la collaboration interdisciplinaire entre ophtalmoloogistes et hematologues