Venous sinus thrombosis leading to stroke in a patient with sickle cell disease on hydroxyurea and high hemoglobin levels: Treatment with thrombolysis

Abstract

A 21-year-old man with homozygous sickle cell disease maintained on hydroxyurea for 1 year developed thrombosis of the superior sagittal, right transverse, and right sigmoid dural sinuses with a large venous infarct. He was successfully treated with endovascular thrombolysis. Investigation did not reveal any inherited or acquired hypercoagulable state. This patient however had consistently elevated hemoglobin levels both at the time of the initial event and on follow up. One year later he developed symptomatic avascular necrosis of the right hip. High hemoglobin levels resulting from hydroxyurea therapy may have contributed to development of complications in this patient. Am. J. Hematol. 83:818-820, 2008. V

Unusual Distribution of Cerebral Venous Thrombosis in a Patient With Sickle Cell Disease: A Case Study

“…Follow-up details were scant in the literature. However, available data indicated a generally positive prognosis [7][8]. In line with this, our patient's follow-up CT scan revealed no acute cerebrovascular complications.…”

“…The majority had frequent emergency visits due to painful vaso-occlusive crises. Only two reported a history of acute chest syndrome [7][8][9][10][11][12][13][14]. Our patient's presentation, characterized by generalized body pain, aphasia, ataxia, and confusion, aligns with a diagnosis of CVT and highlights cerebral involvement.…”

“…A literature review uncovered 10 instances of CVT in patients diagnosed with sickle cell disease. Our patient, at 29 years of age, falls within the age range (18 to 37 years) identified in these cases; the average age reported was 26 years [7][8][9][10][11][12][13][14][15]. While the man-to-woman ratio was 7:3, the available data isn't sufficient to form definitive conclusions.…”

Unusual Distribution of Cerebral Venous Thrombosis in a Patient With Sickle Cell Disease: A Case Study

“…Follow-up details were scant in the literature. However, available data indicated a generally positive prognosis [7][8]. In line with this, our patient's follow-up CT scan revealed no acute cerebrovascular complications.…”

“…The majority had frequent emergency visits due to painful vaso-occlusive crises. Only two reported a history of acute chest syndrome [7][8][9][10][11][12][13][14]. Our patient's presentation, characterized by generalized body pain, aphasia, ataxia, and confusion, aligns with a diagnosis of CVT and highlights cerebral involvement.…”

“…A literature review uncovered 10 instances of CVT in patients diagnosed with sickle cell disease. Our patient, at 29 years of age, falls within the age range (18 to 37 years) identified in these cases; the average age reported was 26 years [7][8][9][10][11][12][13][14][15]. While the man-to-woman ratio was 7:3, the available data isn't sufficient to form definitive conclusions.…”

Risk of avascular necrosis of the femoral head in children with sickle cell disease on hydroxyurea: MRI evaluation

“…16 Sidani et al reported a patient with SCD on HU with increased Hb and hematocrit, who developed venous sinus thrombosis and AVNFH. 17 However, there have been several other reports that did not find this association. A study from Guadeloupe and France compared a group of adult patients with SCD with or without AVN and found that while older age, more frequent pain episodes, and co-existent -thalassemia trait were predisposing factors, neither hyperviscosity nor HU therapy were significant factors.…”

“…Mahadeo et al confirmed these associations, but also found that HU independently increased the risk of AVNFH by a factor of 3 . Sidani et al reported a patient with SCD on HU with increased Hb and hematocrit, who developed venous sinus thrombosis and AVNFH . However, there have been several other reports that did not find this association.…”

Unusual Distribution of Cerebral Venous Thrombosis in a Patient With Sickle Cell Disease: A Case Study

“…Follow-up details were scant in the literature. However, available data indicated a generally positive prognosis [7][8]. In line with this, our patient's follow-up CT scan revealed no acute cerebrovascular complications.…”

“…The majority had frequent emergency visits due to painful vaso-occlusive crises. Only two reported a history of acute chest syndrome [7][8][9][10][11][12][13][14]. Our patient's presentation, characterized by generalized body pain, aphasia, ataxia, and confusion, aligns with a diagnosis of CVT and highlights cerebral involvement.…”

“…A literature review uncovered 10 instances of CVT in patients diagnosed with sickle cell disease. Our patient, at 29 years of age, falls within the age range (18 to 37 years) identified in these cases; the average age reported was 26 years [7][8][9][10][11][12][13][14][15]. While the man-to-woman ratio was 7:3, the available data isn't sufficient to form definitive conclusions.…”

Risk of avascular necrosis of the femoral head in children with sickle cell disease on hydroxyurea: MRI evaluation

“…16 Sidani et al reported a patient with SCD on HU with increased Hb and hematocrit, who developed venous sinus thrombosis and AVNFH. 17 However, there have been several other reports that did not find this association. A study from Guadeloupe and France compared a group of adult patients with SCD with or without AVN and found that while older age, more frequent pain episodes, and co-existent -thalassemia trait were predisposing factors, neither hyperviscosity nor HU therapy were significant factors.…”

“…Mahadeo et al confirmed these associations, but also found that HU independently increased the risk of AVNFH by a factor of 3 . Sidani et al reported a patient with SCD on HU with increased Hb and hematocrit, who developed venous sinus thrombosis and AVNFH . However, there have been several other reports that did not find this association.…”

Unusual Distribution of Cerebral Venous Thrombosis in a Patient With Sickle Cell Disease: A Case Study

“…Follow-up details were scant in the literature. However, available data indicated a generally positive prognosis [7][8]. In line with this, our patient's follow-up CT scan revealed no acute cerebrovascular complications.…”

“…The majority had frequent emergency visits due to painful vaso-occlusive crises. Only two reported a history of acute chest syndrome [7][8][9][10][11][12][13][14]. Our patient's presentation, characterized by generalized body pain, aphasia, ataxia, and confusion, aligns with a diagnosis of CVT and highlights cerebral involvement.…”

“…A literature review uncovered 10 instances of CVT in patients diagnosed with sickle cell disease. Our patient, at 29 years of age, falls within the age range (18 to 37 years) identified in these cases; the average age reported was 26 years [7][8][9][10][11][12][13][14][15]. While the man-to-woman ratio was 7:3, the available data isn't sufficient to form definitive conclusions.…”

Risk of avascular necrosis of the femoral head in children with sickle cell disease on hydroxyurea: MRI evaluation

“…16 Sidani et al reported a patient with SCD on HU with increased Hb and hematocrit, who developed venous sinus thrombosis and AVNFH. 17 However, there have been several other reports that did not find this association. A study from Guadeloupe and France compared a group of adult patients with SCD with or without AVN and found that while older age, more frequent pain episodes, and co-existent -thalassemia trait were predisposing factors, neither hyperviscosity nor HU therapy were significant factors.…”

“…Mahadeo et al confirmed these associations, but also found that HU independently increased the risk of AVNFH by a factor of 3 . Sidani et al reported a patient with SCD on HU with increased Hb and hematocrit, who developed venous sinus thrombosis and AVNFH . However, there have been several other reports that did not find this association.…”