Homozygous sickle cell disease is one of the most frequent haemoglobinopathies in Morocco. Sickle cell disease is characterized by a large clinical and biological expression variability which depends on modulating genetic and environmental factors. Clinical manifestation includes regenerative anemia whose severity may vary among individuals. In the absence of treatment, it results in premature death. Sickle cell disease is characterized by a large clinical and biological expression variability which depends on genetic and environmental factors. A severe clinical picture marked by high early transfusion frequency, severe infectious complications and early mortality. A constant inflammatory condition characterized by elevated inflammatory proteins and compromised nutritional status. The objective of this study is to determine the hematological parameters profile in moroccan patients with homozygous sickle cell (SS) disease during stationary phases. We conducted a cross-sectional descriptive study of 87 patients with sickle cell (SS) disease. We performed a biological study based on: Hemogram with morphological assessment of red blood cells stained with MGG and automated reticulocyte counting; Hemoglobin electrophoresis test performed on alkaline agarose gel (pH 8.8) and densitometric integration. The average age is 13.22 years ± 16.36, ranging betrween 0.6 and 36 years, with a sex ratio (M/F) of 1.175. Biological effects of anemia were intense in 88.5% of patients; 67.8% of patients had normocytic anemia compared with 29.9% with microcytosis, and 2.3% with macrocytosis. The degree of anisocytosis was related to the degree of anemia, very evocative in patients with homozygous S/S (95.4%). Reticulocytosis was observed in 81.6% of patients; 52.9% of patients had thrombocytosis. Leukocytosis was observed in 64.4% of patients; 80.5% of patients had neutropenia. The parameters of the hemogram will serve as a basis for comparison during crises and will make it possible to evaluate the effectiveness of patient management. High white blood cell count, platelets and MCHC seem to be determinant of sickle cell anemia severity in Morocco. The haematological profile of moroccan patients with sickle cell disease exhibits data similar to those reported in literature relating to patients with leucocytosis from Central Africa. The results of our study suggest that sickle cell anemia is the most common health problem in Morocco and they are similar to those for major sickle cell syndrome.
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http://dx.doi.org/10.11604/pamj.2016.25.240.11118 | DOI Listing |
Sci Rep
December 2024
Molecular Biology and Genetics Laboratory (LGBM), UFMS - Federal University of Mato Grosso do Sul, Três Lagoas, Brazil.
Sickle cell anemia (SCA) is a monogenic blood disease with complex and multifactorial pathophysiology. The endocannabinoid system (ECS) could be a candidate for modulating SCA complications, such as priapism, as it has demonstrated an essential role in hematopoiesis, platelet aggregation, and immune responses. We evaluated the association of ECS-related single nucleotide polymorphisms (SNP) (FAAH rs324420, MAGL rs604300, CNR1 rs7766029, and CNR2 rs35761398) with priapism in a Brazilian SCA cohort.
View Article and Find Full Text PDFJ Pediatr Hematol Oncol
January 2025
Department of Ophthalmology, Hamilton Eye Institute, University of Tennessee Health Science Center.
This quality improvement initiative aimed to reduce the no-show rate at a hospital-based tertiary sickle cell ophthalmology clinic. Missed appointments place a significant burden on the healthcare system, resulting in prolonged waiting times and underutilized clinical resources that impact the quality of care provided. Individuals with sickle cell disease commonly require multiple appointments to address the myriads of comorbidities associated with their disease.
View Article and Find Full Text PDFJ Pediatr Hematol Oncol
January 2025
Departments of Neurosurgery, Montefiore Medical Center, Albert Einstein College of Medicine.
Spontaneous epidural hematoma (EDH) is a rare sickle cell disease (SCD) complication. We report 3 pediatric cases with SCD and spontaneous EDH and 1 with subgaleal hematomas in the setting of vaso-occlusive crises and elaborate on their presentation and management. Through a scoping review, we identified 71 additional cases reported from 1970 to 2024 and highlighted notable features.
View Article and Find Full Text PDFCureus
November 2024
Hematology and Medical Oncology, Al-Zahraa Medical College, Basrah, IRQ.
Autoimmune hemolytic anemia (AIHA) is a multifactorial disease that causes immune-mediated red blood cell destruction, resulting in anemia and hemolysis symptoms. Despite a significant understanding of its pathogenesis, the precise causes of AIHA remain largely unclear and are thought to be multifactorial. In this paper, we presented a case of sickle cell anemia who developed severe AIHA that failed to maintain response to multiple treatment lines, including steroids, intravenous immunoglobulin, rituximab, and immune suppressive medications.
View Article and Find Full Text PDFStem Cell Res Ther
December 2024
Centre for Stem Cell Research (CSCR), A Unit of InStem Bengaluru, Christian Medical College Campus, Vellore, Tamil Nadu, 632002, India.
Background: Sickle cell disease (SCD) and β-thalassemia patients with elevated gamma globin (HBG1/G2) levels exhibit mild or no symptoms. To recapitulate this natural phenomenon, the most coveted gene therapy approach is to edit the regulatory sequences of HBG1/G2 to reactivate them. By editing more than one regulatory sequence in the HBG promoter, the production of fetal hemoglobin (HbF) can be significantly increased.
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