Extramedullary hematopoiesis of the liver in a child with sickle cell disease: A rare complication

2015 ◽  
Vol 57 (4) ◽  
pp. 770-772 ◽  
Author(s):  
Angela Barrier ◽  
Simo Willy ◽  
Jeremy S. Slone
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Rare Complication ◽  
Extramedullary Hematopoiesis ◽  
Cell Disease

scholarly journals Protection of Microvascular Liver Perfusion with 5-Hydroxymethylfurfural in Sickle Cell Disease Mice Following Hypoxia-Induced Vasoocclusion

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 3382-3382 ◽  
Author(s):  
Michael Wright ◽  
Derek Sim ◽  
Magdalena Alonso-Galicia ◽  
Katalin Kauser ◽  
Keith Abe
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Genetic Disorder ◽  
Liver Perfusion ◽  
Organ Damage ◽  
Extramedullary Hematopoiesis ◽  
T Test ◽  
Vehicle Group ◽  
Cell Disease ◽  
And Control

Abstract Introduction: Sickle cell disease (SCD) is a debilitating genetic disorder, and the resultant "sickling" deformity of red blood cells leads to acute vasoocclusive (VOC) events and chronic disease in multiple organs. Sickle cell hepatopathy, arising from VOC events in the hepatic sinusoids, which can lead to fatal sickle cell intrahepatic cholestasis, is estimated to occur in approximately 10% of the SCD population. Using contrast-enhanced ultrasound (CEUS), we measured microvascular liver perfusion (MVLP) in SCD and control mice at basal levels to determine if noninvasive CEUS can be utilized to assess the underlying extent of disease and also investigated the effect of hypoxia-induced VOC with and without treatment with an antisickling agent, 5-hyroxymethylfurfural (5-HMF). Methods: Townes sickle cell mice (SCD), homozygous for hα:βs -globulin, approximately 7−9 weeks of age (n=28), and control mice, homozygous for hα:β-globulin (n=11), were used in these studies. CEUS perfusion imaging (Vevo® 2100) was performed on a central cross-section of the liver at the renal artery. Contrast agent was administered as an intravenous bolus via tail vein to anesthetized mice (isoflurane with ~21% O2); peak enhancement (PE) was analyzed with VisualSonics software. CEUS measurements were obtained at baseline and following either (1) hypoxia, 60 minutes with 5.5.% O2 followed by ~60 minutes of reoxygenation at room oxygen (~21% O2) or (2) normoxia, ~120 minutes at room oxygen. 5-HMF at 20 and 200 mg/kg PO or vehicle was administered following baseline PE measurement and approximately 30 minutes before start of hypoxia. Results: MVLP in SCD (n=28) was significantly reduced by approximately 40% compared with controls (n=11) at baseline (PE of 14.0±0.7 linear arbitrary units [l.a.u.] vs. 23.6±2.1 l.a.u.), respectively, P<0.001 [Student t test]). Normoxic SCD maintained similar PE to baseline levels (Table 1); however, hypoxia significantly reduced MVLP by 49% in SCD mice. In contrast, hypoxia had no significant effect in control mice. 5-HMF at 20 and 200 mg/kg resulted in a dose-dependent increase in posthypoxia MVLP. 5-HMF at 200 mg/kg was not significantly different from baseline PE, and 5-HMF at 20 mg/kg increased MVLP by approximately 50% compared with the vehicle group posthypoxia (26% vs 49% reduction in MVLP, respectively). Pathologic evaluation of naive SCD formalin-fixed liver tissues (n=10) showed congestion, necrosis, hepatocellular hypertrophy, and extramedullary hematopoiesis. Table 1. CEUS-Acquired Microvascular Liver Perfusion in SCD and Control Mice (mean ± SEM) Strain Dose Oxygen Status Mice, n Baseline PE, l.a.u. Posthypoxia/Normoxia PE, l.a.u. Change From Baseline, % Control Vehicle Hypoxic 11 23.6±2.1 22.1±2.1 -7 SCD Vehicle Normoxic 4 14.0±2.1 13.3±1.6 -3 SCD Vehicle Hypoxic 8 15.4 ±1.6 7.0±0.9* -49 SCD HMF, 20 mg/kg Hypoxic 8 12.8±0.9 9.2±0.7* -26 SCD HMF, 200 mg/kg Hypoxic 8 13.8±1.5 11.9±1.2 -12 *Statistically significant reduction compared with baseline PE (P <0.01, Student t test). Summary: CEUS measured lower basal levels of MVLP in SCD compared with control mice, which correlated with pathologic findings of congestion and necrosis in the livers of SCD mice. The hypoxia-induced VOC decrease in MVLP was present only in the SCD mice; no effect was observed in control mice. Treatment with the antisickling agent, 5-HMF, dose-dependently ameliorated the hypoxia-induced VOC decrease in MVLP in SCD mice. Based on these results, CEUS may be considered as a noninvasive method to measure acute and chronic organ perfusion changes for evaluating new therapeutics for sickle cell-mediated VOC events and end-organ damage. Disclosures Wright: Bayer HealthCare LLC: Employment. Sim:Bayer HealthCare LLC: Employment. Alonso-Galicia:Bayer HealthCare LLC: Employment. Kauser:Bayer HealthCare LLC: Employment. Abe:Bayer HealthCare LLC: Employment.

Cranial epidural hematomas: A case series and literature review of this rare complication associated with sickle cell disease

2016 ◽  
Vol 64 (3) ◽  
pp. e26237 ◽  
Author(s):  
Jennifer Hamm ◽  
Nisha Rathore ◽  
Pearlene Lee ◽  
Zachary LeBlanc ◽  
Jeffrey Lebensburger ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Literature Review ◽  
Sickle Cell ◽  
Rare Complication ◽  
Case Series ◽  
Cell Disease

Acute Hepatic Sequestration Crisis: A Rare Complication of Sickle Cell Disease

2010 ◽  
Vol 105 ◽  
pp. S289-S290
Author(s):  
Bradford Whitmer ◽  
Fernando Gamarra ◽  
Laurence Stawick
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Rare Complication ◽  
Cell Disease

scholarly journals Clinical and laboratory parameters, risk factors predisposing to the development of priapism in sickle cell patients

2019 ◽  
Vol 245 (1) ◽  
pp. 79-83
Author(s):  
Salam Alkindi ◽  
Said S Almufargi ◽  
Anil Pathare
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cardiovascular Response ◽  
Rare Complication ◽  
Acute Chest Syndrome ◽  
Mean Corpuscular Hemoglobin ◽  
Cell Disease ◽  
Laboratory Parameters ◽  
Blood Exchange

Although sickle cell disease is very common in Oman, priapism is a relatively rare complication of this disease. This study was aimed to identify the clinical and laboratory risk factors that predispose sickle cell disease patients to priapism. In a retrospective, case-control study, data on 21 male sickle cell disease patients, with priapism, were compared to 20 age and sex-matched sickle cell disease patients without priapism from the hospital medical records. Specifically, the demographical, hematological, biochemical, and clinical parameters including complications attributable to priapism were studied. Means for continuous measures and independent t-test were used to evaluate the association between the parameter studied and the occurrence of priapism. Priapism occurrence was associated with low basal hemoglobin (Hb), along with an increase in other parameters such as white blood cell count (WBC) ( P = 0.010), platelet count ( P = 0.001), reticulocyte count ( P < 0.001), mean corpuscular volume (MCV) ( P < 0.001), and mean corpuscular hemoglobin (MCH) ( P < 0.001). Biochemical laboratory parameters revealed an association with an increased total bilirubin ( P < 0.001). Patients with priapism were more likely to have acute chest syndrome, stroke, pulmonary hypertension, asplenia ( P = 0.006), gallbladder stones, and consequently cholecystectomy. Blood exchange and Penile aspiration were the most frequent medical therapies ( P = 0.010 and P < 0.001 respectively). Patients with sickle cell disease who presented with priapism were young adults with low Hb, high WBC’s, platelets, reticulocytes, MCV, MCH, bilirubin, and LDH. These findings suggest an association of priapism with increased hemolysis. Impact statement Sickle cell disease is prevalent throughout the world with a 5.8% incidence of sickle cell trait among the Omani population, yet priapism was a relatively rare complication. Penile erection and detumescence are complex physiological processes, which require a delicate neurohormonal and cardiovascular response. In this cohort, young SCD patients developed priapism with a low incidence of complications. These patients had high WBCs, platelets, bilirubin, LDH, and reticulocytes with low hemoglobin as compared to controls, suggesting an increased hemolytic process. Favorable outcome was dependent on early intervention, with almost half of these patients needing surgical intervention.

scholarly journals Major Artery Occlusion: a Rare Complication of Sickle Cell Disease

1970 ◽  
Vol 2 (1) ◽  
pp. e2010007
Author(s):  
Adnan Agha
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Internal Carotid ◽  
Rare Complication ◽  
Artery Occlusion ◽  
Left Internal Carotid Artery ◽  
Cell Disease ◽  
Vessel Occlusion ◽  
Major Vessel ◽  
Haemolytic Anemia

Abstract: Sickle cell disease is hereditary hemoglobinopathy which causes haemolytic anemia, vaso-occlusive crisis, ischemic injuries and many other morbidities like cerebral infarction.  In this report, we describe a case of a young patient with sickle cell disease presenting with right-sided weakness and slurring of speech with examination confirming right-sided hemiparesis with motor aphasia. On further investigation, she was found to have frontotemporal infarction.  On magnetic resonance imaging with angiography, she was found to have absent circulation in left internal carotid artery probably secondary to sickle cell disease.  Major vessel occlusion is rare complication of sickle cell disease that one must bear in mind.

Extramedullary hematopoiesis of the paranasal sinuses in sickle cell disease

2005 ◽  
Vol 132 (6) ◽  
pp. 954-956 ◽  
Author(s):  
William O. Collins ◽  
Ramzi T. Younis ◽  
Monica T. Garcia
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Paranasal Sinuses ◽  
Extramedullary Hematopoiesis ◽  
Cell Disease

scholarly journals Acute compartment syndrome in a patient with sickle cell disease

2020 ◽  
Vol 102 (9) ◽  
pp. e1-e2
Author(s):  
E Cochrane ◽  
S Young ◽  
Z Shariff
Keyword(s):  
Sickle Cell Disease ◽  
Compartment Syndrome ◽  
Sickle Cell ◽  
Rare Complication ◽  
The United States ◽  
Acute Compartment Syndrome ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
The United Kingdom ◽  
Considerable Morbidity

Haemoglobin SC (HbSC) disease accounts for 30% of cases of sickle cell disease in the United Kingdom and the United States. Unlike other sickle cell carriers, who are relatively asymptomatic, people with HbSC disease have a combination of genotypes with the potential to cause considerable morbidity due to intracellular water loss. Patients can present with acute pain, acute chest syndrome, proliferative retinopathy, splenic and renal complications, or stroke. We present a young man with HbSC disease who developed acute compartment syndrome. This is only the second report of this syndrome in a patient with HbSC disease. This is a very rare complication in HbSC disease, but it can have serious implications.

scholarly journals Orbital compression syndrome: a rare complication of sickle cell disease in children

2019 ◽  
Vol 6 (2) ◽  
pp. 890
Author(s):  
Prateek Kumar Panda ◽  
Srikant Kumar Padhy
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Surgical Intervention ◽  
Rare Complication ◽  
Red Blood Cell Transfusion ◽  
Uneventful Recovery ◽  
Cell Disease ◽  
Compression Syndrome

Vasoocclusive crises in sickle cell disease commonly involve bone marrow of the long bones and vertebrae. Involvement of bones with less marrow space, including the bones of the orbit, is reported rarely in the literature. The present case is a 6-year-old boy, a known case of sickle cell disease, who presented with acute onset right upper and lower eyelid swelling, restriction of extraocular movement and pain. USG orbit showed a retroorbital hemorrhage compressing on the orbit, probably due to orbital bone infarction. The child was managed successfully with red blood cell transfusion and intravenous antibiotics, without steroids and surgical intervention. This report highlights the importance of maintaining a high index of suspicion in patients with known sickle cell disease presenting with pain, orbital swelling and restriction of ocular movement. Timely red blood cell transfusion only may be sufficient for uneventful recovery, without steroids or surgical intervention. Expedient diagnosis of orbital compression syndrome in children with sickle cell disease is crucial because this is a potentially sight-threatening entity.

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