Intracranial Hemorrhage Associated with Splenic Sequestration in a Saudi Child with Sickle Cell Anemia: Case Report and Review of the Literature

Hawsawi, Zakaria M. Al

doi:10.5144/0256-4947.2001.210

Cited by 3 publications

(3 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…42 In a French cohort, 2.8% of children with SCD (7 of 251) had either an intracranial hemorrhage or an unruptured aneurysm. 43,44 Risk factors for cerebral hemorrhage are poorly defined, but include hypertension associated with the use of corticosteroids 40 or phenylephrine, 45 recent transfusion, 40 splenic sequestration, 46 and hyperviscosity associated with higher hemoglobin levels. 16 Cerebral hemorrhage in adults is commonly related to aneurysm formation (see supplemental Figure 1C-D).…”

Section: Blood 18 February 2016 X Volume 127 Number 7 Cns Complicatmentioning

confidence: 99%

Central nervous system complications and management in sickle cell disease

DeBaun

Kirkham²

2016

Blood

216

234

View full text Add to dashboard Cite

With advances in brain imaging and completion of randomized clinical trials (RCTs) for primary and secondary stroke prevention, the natural history of central nervous system (CNS) complications in sickle cell disease (SCD) is evolving. In order of current prevalence, the primary CNS complications include silent cerebral infarcts (39% by 18 years), headache (both acute and chronic: 36% in children with sickle cell anemia [SCA]), ischemic stroke (as low as 1% in children with SCA with effective screening and prophylaxis, but ∼11% in children with SCA without screening), and hemorrhagic stroke in children and adults with SCA (3% and 10%, respectively). In high-income countries, RCTs (Stroke Prevention in Sickle Cell Anemia [STOP], STOP II) have demonstrated that regular blood transfusion therapy (typically monthly) achieves primary stroke prevention in children with SCA and high transcranial Doppler (TCD) velocities; after at least a year, hydroxycarbamide may be substituted (TCD With Transfusions Changing to Hydroxyurea [TWiTCH]). Also in high-income countries, RCTs have demonstrated that regular blood transfusion is the optimal current therapy for secondary prevention of infarcts for children with SCA and strokes (Stroke With Transfusions Changing to Hydroxyurea [SWiTCH]) or silent cerebral infarcts (Silent Infarct Transfusion [SIT] Trial). For adults with SCD, CNS complications continue to be a major cause of morbidity and mortality, with no evidence-based strategy for prevention.

show abstract

Section: Blood 18 February 2016 X Volume 127 Number 7 Cns Complicatmentioning

confidence: 99%

Central nervous system complications and management in sickle cell disease

DeBaun

Kirkham²

2016

Blood

216

234

View full text Add to dashboard Cite

show abstract

“…Potential weaknesses include the diligence of different doctors recording splenomegaly, difficulties of assessment in sometimes sick children and that clinical splenic enlargement imperfectly reflects spleen size on ultrasonography 25. The prevalence of ASS in the Jamaican cohort at 35% exceeded the 10% reported from the cooperative study in the USA,26 7% in western Saudi Arabia27 and 16% in a French study 15. All studies used similar definitions for ASS, and the greater frequency in the cohort may reflect clinically mild cases from auditing serial haematology, a hypothesis consistent with the relatively benign clinical course, 90 out of 183 (49%) events resolving without admission and 90 out of 183 (49%) without transfusion.…”

Section: Discussionmentioning

confidence: 90%

“…Recurrence in the cohort was 45%, and 14%–67% elsewhere 15 26 27. Splenectomy was deemed necessary in 20 patients, mostly for recurrence at shorter intervals or the emergence of hypersplenism.…”

Section: Discussionmentioning

confidence: 99%

Acute splenic sequestration in HbSS: observations from the Jamaican birth cohort

Serjeant,

Mason,

Hambleton

et al. 2023

Arch Dis Child

View full text Add to dashboard Cite

ObjectiveTo document the prevalence, clinical features, haematology and outcome of acute splenic sequestration (ASS) in homozygous sickle cell disease (HbSS).Study designA cohort study from birth.SettingThe Medical Research Council Laboratories at the University of the West Indies, Kingston, Jamaica.Patients311 cases of HbSS detected during the screening of 100 000 deliveries at the main government maternity hospital between 1973 and 1981.InterventionsLong-term follow-up and free patient care focusing on ASS.Main outcome measureAcute splenic sequestration.ResultsThere were 183 episodes of ASS in 105 patients representing 35% of the cohort. The median age for first event was 1.07 years. During ASS, median values for haemoglobin fell by 32 g/dL, reticulocytes increased by 8% and total nucleated cells increased by 10.5%. ASS recurred in 47 (45%) patients. Conservative therapy in 133 episodes of 85 patients was associated with five deaths and splenectomy in 20 patients with 50 episodes had no deaths. Symptoms were generally non-specific but acute chest syndrome occurred in 17, and blood cultures revealed coagulase negative staphylococci in 5. The ASS case fatality rate was 3.6% and may be higher if autopsy evidence of ASS is included. There was no seasonal pattern but higher levels of fetal haemoglobin predicted patients less prone to ASS and its later occurrence.ConclusionsASS remains an important cause of morbidity and mortality in HbSS in developing societies. ASS appears to be a non-specific response to many possible risk factors including coagulase negative staphylococci.

show abstract