Bone marrow edema syndromes of the hip: MRI features in different hip disorders

The objectives of this study were to describe the essential magnetic resonance imaging (MRI) features of bone marrow edema syndromes affecting the hip joint. In addition, to evaluate the role of MRI in the assessment of hip joint involvement in different clinical settings that may share similar clinical findings. Thirty-four patients who complained of hip pain were studied consecutively. Of these, 21 were men (61.8%) and 13 were women (38.2%). After clinical assessment of possible hip disease, plain radiograph and MRI study of both hips were performed. The literature was searched using keywords: bone marrow edema, hip, and MRI. All patients had antalgic gait and limping. Initial clinical examination revealed painful limited internal and external rotation of the affected hip/hips suspect for hip disease. Unilateral hip involvement was identified in 31 patients (91.2%), and bilateral hip involvement was found in three patients (8.8%), with a total of 37 hips evaluated by MRI. The final diagnoses in our patients were: reactive arthritis (1), transient osteoporosis (7), avascular necrosis (10), osteoarthritis (2), tuberculous arthritis (4), septic arthritis (2), osteomyelitis (2), sickle cell anemia (2), lymphocytic leukemia (1), and femoral stress fracture (3). Bone marrow edema affecting the hip is neither a specific MR imaging finding nor a specific diagnosis and may be encountered in a variety of hip disorders due to different etiologies. MR imaging is the modality of choice when clinical examination is suspect for hip disease and plain radiographs are normal or equivocal. Early diagnosis and treatment is important in many of the disorders. The literature is reviewed regarding bone marrow edema of the hip.     

Effectiveness of extracorporeal shock wave therapy in bone marrow edema syndrome of the hip

There is no gold standard for treatment of bone marrow edema syndrome of the hip (BMESH). Usually, treatment is conservative, owing to the favorable and self-limiting prognosis. In musculoskeletal disorders, the effectiveness of extracorporeal shock wave therapy (ESWT) has been widely recognized and recent research supports its use in the treatment of the first stages of avascular osteonecrosis of the proximal femur and in other conditions where bone marrow edema is present. On this basis, we performed a prospective study to evaluate the effectiveness of ESWT in normalizing the symptoms and imaging features of BMESH. Twenty consecutive symptomatic patients underwent two treatments of high-energy ESWT and were followed-up at 2, 3 and 6 months, with a final clinical follow-up at mean 15.52 ± 1.91 months. Patients underwent magnetic resonance imaging of the hip and were evaluated according to the Harris hip score. The mean improvement in HHS over the course of the study was of 58.5 ± 14.9 points (p < 0.0001), and the mean edema area reduced from 981.9 ± 453.2 mm² pre-treatment to 107.8 ± 248.1 mm² at 6 months. ESWT seems to be a powerful, non-pharmacological tool that produces rapid pain relief and functional improvement and aids the normalization of the vascular and metabolic impairments which characterize BMESH.     

Treatment of Chediak-Higashi syndrome by allogenic bone marrow transplantation: report of 10 cases

Chediak-Higashi syndrome is a rare condition characterized by susceptibility to bacterial infections, defective natural killer activity, and episodes of macrophage activation known as accelerated phases. Chemotherapy can induce transient remission of the accelerated phase, but relapses become less and less sensitive to treatment and ultimately lead to death. Allogenic bone marrow transplantation (BMT) has been proposed as a curative treatment for Chediak-Higashi syndrome. We report the outcome of BMT in 10 such children. Seven received marrow from an HLA-identical related donor and three from an HLA-nonidentical related donor. Three patients died, two from a new accelerated phase after rejection of transplanted bone marrow and one from cytomegalovirus (CMV) pneumonia. Six of seven recipients of HLA- identical marrow and one of three recipients of HLA-nonidentical marrow are alive and well without treatment 1.5 to 13 years after transplantation (median, 6.5 years). No manifestations of accelerated phases have occurred in these seven patients, and significant natural killer activity is detectable. Interestingly, BMT prevented recurrence of accelerated phases in patients with limited numbers of donor-type leukocytes after transplantation. Ocular and cutaneous albinism were not corrected after transplantation. None of the patients developed serious toxic reactions to the BMT conditioning regimen or have long- term sequelae. These results show that HLA-identical BMT is an acceptable curative treatment for Chediak-Higashi syndrome, whereas HLA- nonidentical BMT remains an experimental approach.     

10例Budd-Chiari综合征的临床分析

Ｂｕｄｄ－Ｃｈｉａｒｉ综合征是由于肝静脉流出道阻塞所引起的肝窦扩张,淤血伴有肝肿大,大量腹水,下肢浮肿的临床综合征［１］。我院４年来先后收治了 １０例,本文作一临床分析,目的是为了提高对Ｂｕｄｄ－Ｃｈｉａｒｉ综合征的认识及诊断水平。 临床资料 一、病例选择１０例均为住院患者,时间从１９９５年６月一１９９８年２月,部分患者曾多次住院,其中男性５例,女性５例,年龄２６－７５岁,平均５３．１０岁,入院后被误诊为肝硬化或其他疾病者３例。所有病例经临床表现,彩色多普勒超声,特别是磁共振血管显影（ＭＲＡ）等明…     

Bone marrow transplantation in Shwachman-Diamond syndrome

Shwachman-Diamond syndrome is a rare autosomal recessive disorder characterized by exocrine pancreatic dysfunction, metaphyseal dysostosis and bone marrow dysfunction with a predilection towards severe hematologic complications. Allogeneic bone marrow transplantation has been used as a therapeutic approach for SDS patients with serious hematologic abnormalities with mixed results. There is some concern that these patients may be more susceptible to early (<100 days) transplant-related complications than other transplant groups. We report a patient who received a matched allogeneic transplant without developing serious early transplant-related complications, but eventually died from relapse of his disease. Although experience is limited, a review of the reported cases suggests patients with SDS may be transplanted without significant short-term morbidity and mortality.     

10例Dubin-Johnson综合征患者临床特征分析

Dubin-Johnson综合征(Dubin-Johnson syndrome,DJS)是一种临床表现为慢性间歇性/持续性高胆红素血症,由位于染色体10q24区的ATP结合盒C亚家族转运体2(ATP-binding cassette subfamiliy C member 2,ABCC2)基因突变,导致多耐药相关蛋白2(multidrug resistance protein 2,MRP2)功能障碍或缺失引起的常染色体隐性遗传病[1-2],其在临床中少见且容易误诊。通过分析总结DJS病例资料,以期加强对该病的认识。     

Allogeneic bone marrow transplantation in the treatment of leukemia: analysis of 10 cases]

Ten leukemic patients were treated with allogeneic bone marrow transplantation (BMT). The diagnosis were ANLL in 6 cases, CML in 3 and ALL in one. Pretransplant immuno suppressive measures including total body irradiation cyclophosphamide and daunorubicin were given. All the patients were infused with health stem cell preparation, so that the hemopoietic function was restored. Graft versus-host disease of grade I to II was present in 5 of the patients. Leukemia recurred 76 days after BMT in one patient who received the procedure during a relapse of the disease, while in the remaining 9 patients disease-free survival from 1 to 23 months has been observed.     

缓解性血清阴性的对称性滑膜炎伴可凹陷性水肿综合征21例分析

目的　进一步了解缓解性血清阴性的对称性滑膜炎伴可凹陷性水肿(RS3PE)综合征的临床和实验室特点,减少漏诊和误诊。　方法　回顾性分析21例RS3PE综合征患者的临床资料,包括症状、体征、实验室检查及疗效,并复习相关文献。　结果　21 例患者中男性18 例,年龄57~81岁,平均69 2岁,其中≥60岁19例。均表现为突发双手、足背凹陷性水肿及多关节炎,近端指间关节炎和掌指关节炎各17例(各81 0%),腕关节炎12 例(57 0%),膝关节炎和踝关节炎各8 例(各38%)、肘关节炎4 例(19 0%)。14 例外周血白细胞(10 0 ~ 14 3)×109/L,血沉平均(90±38)mm/1 h, C反应蛋白平均(836±122)μg/L,1例抗核抗体1∶40 阳性,其余均阴性,类风湿因子、抗可提取核抗原抗体及抗双链DNA抗体、人类白细胞相关抗原B27均阴性。21例患者手足、骶髂关节X线均未见异常。8例合并恶性肿瘤,7例死于肿瘤转移。1例合并急性肾盂肾炎和反应性噬血细胞综合征。小剂量泼尼松和慢反应药治疗后临床症状缓解,但合并肿瘤者易复发,手术或化疗后缓解。合并感染者经抗感染治疗未再复发。　结论　RS3PE综合征是异质性临床症候群,小剂量泼尼松和     

抗磷脂抗体综合征合并急性心肌梗死10例分析

目的通过对抗磷脂抗体综合征(APS)引起的急性心肌梗死病例的分析提高对本病的认识。方法回顾性分析本院274例APS患者的临床资料。结果 274例APS患者中,合并急性心肌梗死者10例,发生率为3.6%,10例患者年龄为(45±13)岁,男女比为1:4,其中原发性APS和继发性APS各5例,8例患者在诊断急性心肌梗死后才明确APS的诊断。经激素、免疫抑制剂和抗栓治疗后2例(20%)死亡。结论 APS患者合并急性心肌梗死少见,预后差,治疗措施主要为抗血小板聚集和抗凝。对发生急性心肌梗死的年轻女性需考虑APS。     

低增生型骨髓增生异常综合征的骨髓病理观察

目的：评估骨髓活检在骨髓增生异常综合征尤其是低增生型病例时的诊断价值。方法：对比分析５３例骨髓增生异常综合征的患者的骨髓涂片与切片的诊断结果。结果：骨髓活检病理检查对于诊断骨髓增生异常综合征,尤其在低增生型骨髓增生异常综合征患者有很大帮助。结论：骨髓活检是确诊低增生型骨髓增生异常综合征的必要手段。     

同步骨髓涂片与骨髓活检在2761例血液疾病诊断中的对比分析

目的探讨骨髓涂片和骨髓活检同步对比分析在血液病诊断中的意义。方法收集1989—2009年沈阳军区总医院2761例血液疾病患者,骨髓样本采用骨髓抽吸、活检同步法取材,骨髓涂片与活检染色后,行增生程度及细胞形态学观察。结果在急性白血病(AL)、再生障碍性贫血(AA)、原发性骨髓纤维化(PMF)、骨髓转移癌(MCBM)、多发性骨髓瘤(MM)、慢性淋巴细胞白血病(CLL)、慢性粒细胞白血病(CML)、骨髓增殖性疾病(MPD)及恶性淋巴瘤的诊断符合率,骨髓活检高于骨髓涂片(P<0.05);对于白血病的分型、特发性血小板减少性紫癜(ITP)的诊断符合率,骨髓涂片高于骨髓活检(P<0.05)。骨髓活检在监测PMF和继发性骨髓纤维化疾病的骨髓增生程度及纤维化程度占显著的优势。结论在血液疾病的诊断中骨髓活检和骨髓涂片各具优缺点,两者的结合对提高疾病的诊断率有重要的价值。     

Gold induced marrow suppression: a review of 10 cases

We have reviewed 10 cases of gold sodium aurothiomalate (GSTM) induced marrow suppression. All had biopsy proven marrow hypoplasia/aplasia. Anemia, neutropenia and thrombocytopenia were observed in 7 of the 10 patients. In 2 patients, hypoplastic marrow was associated with suppression of 2 of the formed elements of the blood, while in one patient, there was isolated neutropenia. Nine of the 10 patients survived. Bone marrow recovery occurred within an average of 5.4 months. A response was seen in 3 with antithymocyte globulin. One patient required an allogeneic bone marrow transplant. A combination of corticosteroids and androgens was associated with recovery in 5. Blood and platelet transfusions and antibiotics were instituted only when clinically indicated. We conclude that the prognosis of GSTM induced marrow suppression is better than previously reported.     

Bone marrow mast cell content in preleukemic syndrome

Bone marrow mast cell content was evaluated by a semiquantitative method in 22 marrow specimens from 20 patients with preleukemic syndrome and was compared with 21 marrow specimens from iron-deficient control subjects. Results indicate a statistically significant increase of bone marrow mast cell content in patients with preleukemic syndrome in comparison to control subjects (p < 0.0005). Two of 20 preleukemic patients converted to acute myeloblastic leukemia and conversion was accompanied by a significant decrease of bone marrow mast cell content. Our findings indicate that bone marrow mast cell content can be reproducibly quantitated and represents an additional morphologic criterion for diagnosis of the preleukemic syndrome.     

Bone marrow transplantation does not correct the hyper IgE syndrome

Congenital immunodeficiency in hyper IgE syndrome is characterised by a markedly raised IgE level, recurrent staphylococcal skin infection and pneumatoceles. Standard treatments include anti-staphylococcal antibiotics. We report a severely affected patient in whom successful bone marrow transplantation was followed by reappearance of the immunodeficiency. We conclude that bone marrow transplantation does not cure the immunological features of the hyper IgE syndrome. Bone Marrow Transplantation (2000) 25, 1303-1305.     

Etiology of acute pulmonary edema during liver transplantation : a series of cases with analysis of the edema fluid

STUDY OBJECTIVES: To describe the clinical features of a group of patients who acutely developed pulmonary edema during orthotopic liver transplantation and to determine the nature (transudate vs exudate) of the edema. DESIGN: Retrospective review of clinical records and radiographic studies. SETTING: Operating room and ICU of a tertiary-care medical center hospital. PATIENTS: End-stage liver disease patients undergoing orthotopic liver transplantation under general anesthesia. Interventions and measurements: Pulmonary edema fluid obtained from seven patients within 15 min of first appearance was analyzed for protein content and compared with the protein content of a simultaneously obtained plasma sample. Hemodynamic data, fluid administration totals, and length of postoperative intubation and ICU stay were also collected. RESULTS: Eight patients were identified. Six of the seven patients whose edema fluid was analyzed had edema fluid/plasma protein ratios > or =0.75, characteristic of increased permeability pulmonary edema (the one other patient had a ratio of 0.73). Hemodynamic monitoring at the time of onset of the edema effectively ruled out a cardiogenic etiology. One patient died intraoperatively; at autopsy, the cause of death was determined to be pulmonary fat embolization. In the other seven patients, production of edema fluid resolved within 6 h of admission to the ICU. The duration of ventilatory support ranged from 23 to 96 h, with a mean of 49 h. CONCLUSIONS: The most likely cause of the reaction is transfusion-related acute lung injury (TRALI). An incidence of TRALI that is higher than previously reported in this population indicates that other elements, such as reperfusion of the newly implanted liver, may be contributing factors.