Introduction: Sickle cell disease and thalassemia are the most frequent hemoglobinopathies. During their evolution, they present certain complications, among which are two neurosurgical emergencies, namely spontaneous...Introduction: Sickle cell disease and thalassemia are the most frequent hemoglobinopathies. During their evolution, they present certain complications, among which are two neurosurgical emergencies, namely spontaneous cranial epidural hematoma and non traumatic radiculo-medullary compression, with some particularities for each. Method: In order to highlight these particularities, we compared the characteristics of these two complications, from a number of publications reported between 2000 and 2021. Results: Sickle cell disease was complicated by spontaneous cranial epidural hematoma. Forty-two cases were reported, the mean age was 14.7 years (2 - 21 years) and the sex ratio was 6.4. The clinical presentation combined, in a non-traumatic context, signs of intracranial hypertension with those of neurological focalization. Neuroimaging showed epidural-type collection, often frontal and parietal in location. The incriminating mechanisms were ischemia, hemorrhage and extra medullary hematopoiesis. The treatment was surgical. Non traumatic radiculo-medullary was the complication of thalassemia. Of the 77 cases reported, the mean age was 27.5 years (9 - 66 years) and the sex ratio was 4.1. The lesions were epidural with a clear thoracic predominance and resulted from extra marrow hematopoiesis. Treatment included: hypertransfusion, radiotherapy, hydroxyurea and surgery. Vital and functional prognosis were globally satisfactory when the management was rapid. Conclusion: Cranial and spinal epidural lesions, respective complications of sickle cell disease and thalassemia, result from similar mechanisms. Their prognosis depends on the rapidity of management. .展开更多
Hemoglobinosis C occurs mainly in Africa and America with a high frequency in West Africa. In Senegal, homozygous hemoglobinopathy CC constitutes a very rare profile of which only 3 cases are followed in the clinical ...Hemoglobinosis C occurs mainly in Africa and America with a high frequency in West Africa. In Senegal, homozygous hemoglobinopathy CC constitutes a very rare profile of which only 3 cases are followed in the clinical hematology department of Dakar. The 1<sup>st</sup> case is a 49-year-old female patient, with notion of 1<sup>st</sup> degree consanguinity, and a long history of abdominal pain who presented a poorly tolerated anemic syndrome and splenomegaly. The biological assessment showed moderate anemia (7.6 g/dL) with microcytic hypochromia and a CC profile (HbC = 99.2%;HbA2 = 0.8%) on hemoglobin electrophoresis. The second case was a 22-year-old female patient with a notion of 2<sup>nd</sup> degree consanguinity who presented a Chauffard triad. The haemogram showed mild anaemia (11 g/dL), microcytic and hypochromic. Hemoglobin electrophoresis confirmed a CC profile (HbC = 95.3%;HbA2 = 4.7%). The third patient was 27 years old, with a history of diffuse abdominal pain and 2<sup>nd</sup> degree consanguinity. The haemogram and haemoglobin electrophoresis confirmed the CC profile (HbC = 94.6%;HbA2 = 5.4%). The negativity of the Emmel test in front of this presentation suggestive of sickle cell disease means that this type of hemoglobinopathy is diagnosed late in our regions. We therefore recommend the systematic performance of hemoglobin electrophoresis in the presence of any chronic hemolytic anemia.展开更多
Aims: To study the frequency of sickle cell retinopathy and its severity. Material and Methods: Retrospective study over a period of 3 years (July 2013 thru June 2016) of cases of adults over 18 years old sent by the ...Aims: To study the frequency of sickle cell retinopathy and its severity. Material and Methods: Retrospective study over a period of 3 years (July 2013 thru June 2016) of cases of adults over 18 years old sent by the Hematology Department of the Campus-Teaching Hospital of Lomé in the context of a sickle-cell disease check-up. The parameters studied were age, sex, genotype, examination of the fundus by the Goldmann lens. The classi-fication after the fundus was in non-proliferative retinopathy and in 5 proliferative stages according to Goldberg’s classification. Results: Hundred and eighty-one patients were included with an average age of 29.39 ± 9.84. The sex ratio was 0.72 in favor of female. The SC genotype was 50.3%;SS 37.6%;CC 11% and SF 1.1%. Sickle cell retinopathy was found in 22.7% of patients, among which 14.4% were proliferative and 8.3% non-proliferative. Seventy-three percent of patients with proliferative retinopathy were SC genotype, 23% SS and 3% CC genotype. Among SC patients, 20.9% had proliferative retinopathy;8.8% SS;and 5% CC. Fifty percent of patients with proliferative retinopathy had Goldberg’s stage ≥ 3. Conclusion: We found 22.7% of sickle cell retinopathy which proliferative form was strongly represented by the SC genotype.展开更多
The authors report a case of systemic lupus associated with a composite heterozygosis SC with thalassemic component in a 19-year-old patient hospitalized for anemia and polyarthralgia who has a staturoponderal delay, ...The authors report a case of systemic lupus associated with a composite heterozygosis SC with thalassemic component in a 19-year-old patient hospitalized for anemia and polyarthralgia who has a staturoponderal delay, macular erythematous lesions in butterfly wings on the face and ears, photosensitivity, puffy face, alopecia, pubic and axillary hair loss, scalp dermatophytosis, painful swelling of the interphalangeal joints, wrists and knees. The hemoglobin electrophoresis showed a compound heterozygote SC associated with β thalassemia. Antinuclear antibodies were positive with an anti-Sm positive antibody. Conclusion: The diagnosis of both diseases can be difficult when symptoms are concomitant and look alike.展开更多
文摘Introduction: Sickle cell disease and thalassemia are the most frequent hemoglobinopathies. During their evolution, they present certain complications, among which are two neurosurgical emergencies, namely spontaneous cranial epidural hematoma and non traumatic radiculo-medullary compression, with some particularities for each. Method: In order to highlight these particularities, we compared the characteristics of these two complications, from a number of publications reported between 2000 and 2021. Results: Sickle cell disease was complicated by spontaneous cranial epidural hematoma. Forty-two cases were reported, the mean age was 14.7 years (2 - 21 years) and the sex ratio was 6.4. The clinical presentation combined, in a non-traumatic context, signs of intracranial hypertension with those of neurological focalization. Neuroimaging showed epidural-type collection, often frontal and parietal in location. The incriminating mechanisms were ischemia, hemorrhage and extra medullary hematopoiesis. The treatment was surgical. Non traumatic radiculo-medullary was the complication of thalassemia. Of the 77 cases reported, the mean age was 27.5 years (9 - 66 years) and the sex ratio was 4.1. The lesions were epidural with a clear thoracic predominance and resulted from extra marrow hematopoiesis. Treatment included: hypertransfusion, radiotherapy, hydroxyurea and surgery. Vital and functional prognosis were globally satisfactory when the management was rapid. Conclusion: Cranial and spinal epidural lesions, respective complications of sickle cell disease and thalassemia, result from similar mechanisms. Their prognosis depends on the rapidity of management. .
文摘Hemoglobinosis C occurs mainly in Africa and America with a high frequency in West Africa. In Senegal, homozygous hemoglobinopathy CC constitutes a very rare profile of which only 3 cases are followed in the clinical hematology department of Dakar. The 1<sup>st</sup> case is a 49-year-old female patient, with notion of 1<sup>st</sup> degree consanguinity, and a long history of abdominal pain who presented a poorly tolerated anemic syndrome and splenomegaly. The biological assessment showed moderate anemia (7.6 g/dL) with microcytic hypochromia and a CC profile (HbC = 99.2%;HbA2 = 0.8%) on hemoglobin electrophoresis. The second case was a 22-year-old female patient with a notion of 2<sup>nd</sup> degree consanguinity who presented a Chauffard triad. The haemogram showed mild anaemia (11 g/dL), microcytic and hypochromic. Hemoglobin electrophoresis confirmed a CC profile (HbC = 95.3%;HbA2 = 4.7%). The third patient was 27 years old, with a history of diffuse abdominal pain and 2<sup>nd</sup> degree consanguinity. The haemogram and haemoglobin electrophoresis confirmed the CC profile (HbC = 94.6%;HbA2 = 5.4%). The negativity of the Emmel test in front of this presentation suggestive of sickle cell disease means that this type of hemoglobinopathy is diagnosed late in our regions. We therefore recommend the systematic performance of hemoglobin electrophoresis in the presence of any chronic hemolytic anemia.
文摘Aims: To study the frequency of sickle cell retinopathy and its severity. Material and Methods: Retrospective study over a period of 3 years (July 2013 thru June 2016) of cases of adults over 18 years old sent by the Hematology Department of the Campus-Teaching Hospital of Lomé in the context of a sickle-cell disease check-up. The parameters studied were age, sex, genotype, examination of the fundus by the Goldmann lens. The classi-fication after the fundus was in non-proliferative retinopathy and in 5 proliferative stages according to Goldberg’s classification. Results: Hundred and eighty-one patients were included with an average age of 29.39 ± 9.84. The sex ratio was 0.72 in favor of female. The SC genotype was 50.3%;SS 37.6%;CC 11% and SF 1.1%. Sickle cell retinopathy was found in 22.7% of patients, among which 14.4% were proliferative and 8.3% non-proliferative. Seventy-three percent of patients with proliferative retinopathy were SC genotype, 23% SS and 3% CC genotype. Among SC patients, 20.9% had proliferative retinopathy;8.8% SS;and 5% CC. Fifty percent of patients with proliferative retinopathy had Goldberg’s stage ≥ 3. Conclusion: We found 22.7% of sickle cell retinopathy which proliferative form was strongly represented by the SC genotype.
文摘The authors report a case of systemic lupus associated with a composite heterozygosis SC with thalassemic component in a 19-year-old patient hospitalized for anemia and polyarthralgia who has a staturoponderal delay, macular erythematous lesions in butterfly wings on the face and ears, photosensitivity, puffy face, alopecia, pubic and axillary hair loss, scalp dermatophytosis, painful swelling of the interphalangeal joints, wrists and knees. The hemoglobin electrophoresis showed a compound heterozygote SC associated with β thalassemia. Antinuclear antibodies were positive with an anti-Sm positive antibody. Conclusion: The diagnosis of both diseases can be difficult when symptoms are concomitant and look alike.
