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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report a patient with sickle cell anemia who suffered from paraplegia of 18 months duration due to spinal cord compression by a hemopoietic mass. Recovery following removal of the mass was complete.
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PMID:Spinal cord compression by extramedullary hemopoietic tissue in sickle cell anemia. Case report. 115 87

A 10-year-old boy without previous illness was found to have sickle cell disease and pneumococcal meningitis. On completion of 10 days of treatment with penicillin, the only abnormality still present was paraplegia. Movements of the legs recovered gradually, and after 3 months he was walking normally. Paraplegia is rare in sickle cell disease and the prognosis is not always poor.
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PMID:Sickle cell disease, pneumococcal meningitis and paraplegia with recovery. 969 5

A case of paraplegia due to a thoracic epidural abscess in a 6-year-old black girl with sickle cell anemia is reported. MRI and CT scan showed abnormalities involving T6 vertebra which were consistent with osteomyelitis. A laminotomy, associated to antibiotic administration, permitted rapid neurological improvement. Although cultures were negative, the infection was probably due to salmonellae. Salmonellosis is a well-known complication in children with sickle cell disease but spinal localization is unusual. Its pathogenesis is unclear but immunological abnormalities associated to vascular obstructions by abnormal blood red cells have been advocated. The underlying etiology of vertebral abnormalities in these children is difficult to determine, but early diagnosis with modern investigations, as CT scan and MRI, is crucial to promptly begin therapy for osteomyelitis, minimizing the risk of spinal cord compression.
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PMID:[Vertebral osteomyelitis with epidural abscess in a child with sickle cell disease]. 1079 Jun 44

A fifty-year-old male patient presented with bilateral lower limb paraplegia. He was subsequently found to have sickle cell anaemia. The patient showed good response to treatment. Central nervous system complications in sickle cell anaemia are well known. However paraplegia has rarely been documented in sickling disorders. We report this case because of rarity of sickle cell anaemia presenting in old age with paraplegia.
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PMID:Unusual presentation of sickle cell anaemia--paraplegia in a fifty year old man. 1675 80

In apparently healthy individuals, tuberculosis (TB) affects mainly the lungs however, worsening immune status tend to predispose to an increased tendency for extra-pulmonary tuberculosis (EPTB). We report the case of a 22 year old known sickle cell anaemia (HBS) female student with three-month history of bilateral hip pain, weight loss and swelling of the left hip with multiple discharging sinuses, paraplegia and recurrent fever. There was no preceding history of trauma. Full Blood Count (FBC) revealed leukocytosis with neutrophilia, monocytosis, thrombocytosis and Packed Cell Volume (PCV) of 23%. Erythrocyte sedimentation rate (ESR) was 120mm/Hr (Western Green) and retroviral screening was negative. Bacteriologic culture of the discharging sinuses grew Escherichia coli and Staphylococcus aureus, both sensitive to sparfloxacin. Smear microscopy for acid-fast-bacilli (AFB) was negative. Chest X-Ray was reported normal but X-Ray of the pelvis showed loss of L4/L5 disc space and appearances suggestive of avascular necrosis of the femoral heads. Clinical and haematological profile of the patient started to improve by the second month on therapeutic trial of anti-TB regimen. She had nine-month course of therapy and later discharged to physiotherapy clinic. Management of EPTB requires a high index of clinical suspicion and well-equipped laboratory to support the diagnosis. Therefore, this case report highlights the need to upgrade TB-diagnostic facilities in this environment.
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PMID:Case report: extra pulmonary tuberculosis in sickle cell disease. 1731 49

Spinal cord compression due to extramedullary hematopoiesis (EMH) is a rare complication of thalassemia and generally presents as paraparesis with sensory impairment. Complete paraplegia is extremely rare in EMH due to thalassemia although it is known to occur in polycythemia vera and sickle cell anemia. Treatment options mostly include surgery and/or radiotherapy. Whereas cases presenting with paraparesis have been treated with either surgery or radiotherapy with equal frequency and efficacy, almost all reported cases with paraplegia have been treated with surgery with or without radiation therapy. We hereby report a case of thalassemia intermedia with paraplegia treated successfully with radiotherapy.
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PMID:Paraplegia due to extramedullary hematopoiesis in thalassemia treated successfully with radiation therapy. 1740 52

Extramedullary hematopoiesis (EMH) occurs in patients with various hematologic disorders involving a chronic increase in the production of red blood cells, and is often associated polycythemia vera and sickle cell anaemia, but is less common with thalassemia especially with hemoglobin E-beta thalassemia. Spinal cord compression due to EMH is a extremely rare complication of thalassemia and may present with paraparesis or paraplegia with or without sensory impairment. Treatment options mostly include surgery and/or radiotherapy. Whereas cases presenting with paraplegia have been treated with either surgery or radiotherapy with equal frequency and efficacy, almost all reported cases with paraplegia have been treated with surgery with or without radiation therapy. We hereby report a case of hemoglobin E-beta thalassemia with paraplegia treated successfully with radiotherapy.
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PMID:Extramedullary hematopoiesis and paraplegia in a patient with hemoglobin e-Beta thalassemia. 2063 44

The phenomenon of erythrocyte sickling observed in sickle cell anaemia is responsible for ischaemia and tissue infarction compromising several organs and systems including the mouth and face. This brief paper reports the case of a 17- year-old female with a complicated sickle cell anaemia, hypertension and paraplegia (after an ischaemic stroke at the age of six years). Oral examination revealed the absence of tooth 12, fractures of teeth 11, 21 and 22 (from trauma), active caries lesions in the enamel of teeth 36, 37 and 46, mucosal pallor, and a smooth tongue. Oral radiographs revealed bone rarefaction and trabecular bone coarsening. Dental surgeons and physicians should be aware of the general and oral abnormalities that can be present in individuals with sickle cell anaemia to allow for preventive measures and implementation of effective treatment options.
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PMID:Oral considerations in the management of sickle cell disease: a case report. 2297 72

Sickle cell disease (SCD) is reported to be the most common genetic disorder affecting Nigerians. Children with SCD are at a high risk of neurological morbidity. The main objective of this study was to determine the pattern of adverse neurological outcomes among a cohort of Nigerian children with SCD. All children with SCD seen in the Department of Paediatrics, University College Hospital, Ibadan, Nigeria, over a period of 2 years were carefully evaluated for symptoms and signs of neurological complications, defined as clinical outcomes referable to the central nervous system. Of the 214 children evaluated, 187 were diagnosed with Hb SS disease and 27 with Hb SC disease. Neurological complications were identified in 78 (36.4 %) of the cases. The most common complications were headache (17.8 %), seizure (9.3 %) and stroke (8.4 %). Other less frequent complications included bacterial meningitis (2.8 %), spontaneous visual loss (1.4 %), paraplegia (0.9 %) and transient ischaemic attacks (0.9 %). Neurological complications occurred more frequently in children with sickle cell anaemia than in those with Hb SC disease (P = 0.002, 95 % CI 1.450-82.870). Adverse neurological events are common in Nigerian children with SCD, with a significantly higher risk in Hb SS than Hb SC disease. Stroke represents a major underlying cause of symptomatic epilepsy in SCD. Institution of primary preventive measures for stroke in SCD will significantly reduce the burden of stroke and epilepsy associated with SCD in Nigeria.
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PMID:Adverse neurological outcomes in Nigerian children with sickle cell disease. 2312 67

We have performed allogeneic hematopoietic cell transplantation (allo-HCT) using a reduced intensity conditioning regimen for curative management of advanced myelofibrosis (MF). However, allo-HCT is rarely considered for elderly or patients with severe comorbidities due to high transplantation-related mortality. In those patients, an alemtuzumab-based non-myeloablative (NMA) conditioning regimen followed by stem cell transplantation could be a possible treatment that has been tried in sickle cell anemia showing stable mixed chimerism and improvement of the disease. However, it is uncertain whether this regimen can provide durable donor-dominant chimerism also in patients with MF. We planned a two-stage allo-HCT in four patients - initially aimed at mixed chimerism with NMA conditioning and then reinforced with additional stem cell infusion if graft failure occurred. In one case with extensive extramedullary hematopoiesis, causing blindness and paraplegia, we achieved stable complete donor-chimerism and complete molecular response with disappearance of bone marrow fibrosis after allo-HCT. Although this NMA regimen failed to achieve durable donor-chimerism, additional stem cell infusion showed a possible role for stable long-term chimerism with good clinical outcomes. Although it leaves room for further improvement, allo-HCT using an NMA conditioning regimen may be worth consideration for advanced MF patients with severe comorbidity, otherwise no appropriate treatment option is available.
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PMID:Experiences of allogeneic hematopoietic cell transplantation following non-myeloablative conditioning regimen in severely comorbid patients with myelofibrosis: case series with a patient presenting with extensive extramedullary hematopoiesis. 3265 47


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