Bone Infarction Research Articles

P.095 Holey Spinal Cord - A case of spinal cord stroke secondary to fibrocartilaginous embolism

Background: Fibrocartilaginous embolism (FCE) is a rare reported cause of spinal cord infarction and likely underdiagnosed due to clinical unfamiliarity. FCE can present after a mild trauma and is characterized by back or neck pain along and a rapidly progressive myelopathy. We present a case of FCE and discuss how this clinical entity can break the typical rules of stroke. Methods: Case presentation Results: An otherwise healthy, 56-year-old professional sports coach presented a couple day history of progressive leg paresthesias and mild back pain, followed by unsteady gait and then inability to void. The left leg demonstrated mild weakness, hyperreflexivity, ataxia and an upgoing plantar response. The right leg became spastic and he then developed bilateral impairment of vibration and proprioception at the toes. An initial limited MRI lumbar spine was negative. A repeat MRI spine showed mild diffusion restriction of T10-11 and T11-12 and evidence of a bone infarct L2. He was diagnosed with a spinal cord infarct secondary to FCE. Conclusions: FCE should be considered in the differential for acute myelopathy. It can present with a progressive nature like transverse myelitis and MRI may be non-diagnostic. As more cases are being reported, FCE is becoming better defined and recognized.

Imaging findings of bone infarction in children with systemic lupus erythematosus

To investigate diagnostic value of MRI, X ray and CT for bone infarction in children with systemic lupus erythematosus. Eleven systemic lupus erythematosus children with bone infarction were retrospectively analyzed from January 2015 to January 2017 , and tested by MRI, X-ray and CT. Among them, including 1 male and 10 females aged from 6 to 16 years old with an average of 13 years old. All patients were detected by MRI, 9 patients were detected by X-ray and 3 patients were detected by CT, imaging findings were analyzed. The location of bone infarction involved 60 sits, 30 sites located on metaphyseal-diaphyseal region, 8 located on patella, 21 located on epiphysis, and 1 located on talus. Focus of 11 patients were detected by MRI, the main manifestation showed geographic change, long T1 and T2 signal could seen around focus, and showed double ring sign and three ring sign; 5 of 9 patients by X-ray examination detected focus;2 of 3 patients by CT examination detected focus. No abnormity seen at early stage by X-ray and CT examination, and low density focus around harden edge at chronic stage. MRI could display bone fracture at early stage, X-ray and CT could only display lesion at chronic stage, MRI is the most effective method in diagnosing bone infarction.

Orthopaedic Manifestations of Sickle Cell Disease.

Sickle cell disease (SCD) is an autosomal recessive disorder that results in hemolytic anemia related to abnormal hemoglobin and erythrocyte levels. SCD is characterized by vascular occlusive episodes, visceral sequestration, and aplastic or hemolytic crises. These crises most commonly occur in bone. The orthopaedic manifestations of SCD comprise much of the morbidity associated with this disorder. Osteonecrosis and osteomyelitis are among the most disabling and serious musculoskeletal complications in patients with SCD. Effective management of the bone and joint sequelae requires an accurate diagnosis, an understanding of the pathophysiology of the disease, and knowledge of available medical and surgical treatment alternatives. The major orthopaedic manifestations of SCD are osteonecrosis, osteomyelitis, septic arthritis, and bone infarction. Patients with SCD require close monitoring in the perioperative period because of the risk for vasoocclusive crisis.

Bone Infarctions in a Patient with Systemic Lupus Erythematosus and Anti-phospholipid Syndrome

Bone Infarctions in a Patient with Systemic Lupus Erythematosus and Anti-phospholipid Syndrome

Symptomatic steroid induce multifocal diaphyseal bone infarcts treated with intra-medullary nailing

Introduction Non traumatic osteonecrosis is the ischaemic death of cellular elements within bone. Etiological factors implicated include long term corticiosteroid use, alcoholism, sickle cell disease, systemic lupus erythematosus, amongst others. Common sites of involvement include the proximal femur, knee, shoulder, ankle. Metaphyseal-diaphyseal lesions have been well described radiolographically, however are commonly considered asymptomatic. There is thus a paucity of literature describing techniques used for symptomatic diaphyseal or metaphyseal lesions not involving the epiphyseal region. Case description Our patient is a 40-year-old woman diagnosed with Arnold-Chiari malformation in 2005 who was then surgically treated with foramen magnum decompression. In 2010 she was treated with 4 months of Dexamethasone 2mg for chemical meningitis. She presented to the Orthopaedic outpatient clinic in 2012, 16 months after ceasing steroid medication, with a 6 month history of difficulty walking due to pain in bilateral groins and bilaterally along her shins, left worse than right. MRI of both hips demonstrated anterior serpiginous lesions within the femoral heads consisted with AVN (Ficat II). MRI of lower legs showed isolated bone infarct in the metaphyseal-dyaphyseal region of her tibias bilaterally. She had bilateral total hip arthroplasties with immediate relief of hip symptoms. Our patient underwent bilateral tibial intramedullary nailing using a Stryker T2 nail with a medial parapatellar approach. At both the 6 week and 5 month follow-up she had no further pain, was non tender to palpation and was very satisfied with result. Results and Conclusions We are unaware of any reports of the development of symptomatic diaphyseal osteonecrosis in patients receiving corticosteroids for the treatment of meningitis. Much of the literature regarding management of osteonecrosis is focused on the treatment of epiphyseal lesions in the femoral head and around the knee. Diaphyseal lesions have been well described radiologically but are often defined as asymptomatic and clinically insignificant. Our use of intramedullary nailing thus illustrates an effective surgical option for the treatment of symptomatic diaphyseal osteonecrosis. Take home message Osteonecrosis must be considered in all patients receiving high dose or long term steroids for any indication. Intramedullary nailing can be a successful method of treating symptomatic diaphyseal osteonecrosis of long bones.

Role of nuclear medicine in the differential diagnosis of bone infarction and osteomyelitis in drepanocytosis

Role of nuclear medicine in the differential diagnosis of bone infarction and osteomyelitis in drepanocytosis

Earlier Development of Limb Ulcers, Digital Bone Infarction and Pulmonary Hypertension in a Patient with SLE- A Rare Case Report

Systemic lupus erythematosus (SLE) is a multiorgan autoimmune disease in which skin is involved in up to 85% of cases. Lower extremity ulcers are an infrequent but disabling complication of SLE. Critical peripheral ischemia (CPI) is also an uncommon but potentially devastating feature of SLE. We reported a case o f SLE who presented with multiple ulcers along with digital infarcts of upper and lower limbs. She had features of digital bone infarct of left hand and right foot. She also had features of pulmonary interstitial involvement with pulmonary hypertension. All of these features were found within three months of disease onset which is very rare. She was treated with intravenous Methylprednisolone followed by oral Prednisolone, Hydroxychloroquinine, Azathioprine, Diuretic, Bosentan, Aspirin and Nifedipine in combinaton. Later warfarin was added after one month. She showed significant improvement after three months of treatment.J Bangladesh Coll Phys Surg 2017; 35(3): 150-154

Role of MRI in the Diagnosis of Different Bone Marrow Lesions in Pediatric Patients with Hematological Malignancies

Background: hematological malignancies are one of the major causes of childhood morbidity;leukemia is the most common of pediatric cancers whereas lymphoma is the third most common childhood malignancy, NHL accounts for approximately 7% of cancers in children younger than 20 years. In patients with hematological malignancies the diagnosis of bone marrow involvement is important to determine prognosis and treatment protocols. MR imaging is the most sensitive imaging modality in the diagnosis of bone marrow infiltration and provides excellent details and additional accurate information about the extent of bone marrow involvement. Aim of the Work: this study aimed to evaluate the role of MRI in the diagnosis of different bone marrow lesions in pediatric patients with hematological malignancies with a trial to highlight the role of different recent modalities in the diagnosis, staging, monitoring management and in post treatment follow up of these patients. Patients and Methods: this study involved 63 patients (37 males and 26 females) with mean age of 9 years (range from 1.5 years: 16 years). They are cases of bone marrow biopsy proven hematological malignancy. They were categorized into two groups, group I: new cases who had bone marrow infiltration, they received no previous chemotherapy, group II: cases under follow up who have bone infarction, with history of chemotherapy or bone marrow transplantation (BMT). The MRI examinations were performed on a superconducting 1.5 T unit. Chi-square test was used to study the association between each 2 variables or comparison between 2 independent groups as regards the categorized data. The probability error at 0.05 was considered significant, while at 0.01and 0.001 were highly significant. Results: the different bone marrow lesions included in this study group of patients showed different MRI diagnostic criteria. A highly significant probability of errors was found in the relation between ADC values of bone infarction, bone marrow infiltration and red marrow.Conclusion: MRI provides an excellent imaging modality for the diagnosis of different bone marrow lesions is pediatric patients with hematological malignancies and can reliably differentiate between them based on the characteristic findings of each.

AN UNUSUAL CASE OF SPINAL MANIFESTATIONS OF SICKLE CELL DISEASE

Sickle cell disease (SCD) results from the presence of abnormal beta globin chains within hemoglobin. Overtime, the disease produces various spinal abnormalities as a result of chronic anemia which include marrow hyperplasia, reversion of yellow marrow to red marrow, and occasionally extramedullary hematopoiesis. Vaso-occlusion may manifest as ischemia and infarction whereas superimposed infection may lead to osteomyelitis. There is paucity of literature illustrating the spinal manifestations of the SCD in a single case. Hence, in the present work, we report the case of a 30-year-old male with SCD who presented with almost all the manifestations of the disease in the spine. Key words: Bone infarction, Magnetic resonance imaging, Sickle cell disease, Spinal manifestations

20 Degree Post Traumatic Ankle Valgus and Distal Lateral Tibial Osteonecrosis Treated with Staged Deformity Correction and Total Ankle Arthroplasty

Category: Ankle, Ankle Arthritis Introduction/Purpose: The purpose of this review is to present a case of post-traumatic ankle valgus and distal lateral tibial osteonecrosis successfully treated with staged deltoid repair, opening wedge tibial osteotomy, fibular lengthening, syndesmotic fusion and total ankle arthroplasty. Methods: Initial surgery consisted of ankle joint arthrotomy and deltoid imbrication. The second surgery consisted of a tibial opening wedge osteotomy with autogenous cortical fibular bone graft superior to the area of osteonecrosis to correct the 20 degree ankle valgus. Fibular lengthening osteotomy and fusion of the distal syndesmosis were also performed. CT scan confirmed bony consolidation at the distal tibiofibular syndesmosis as well as union of the allograft opening wedge. The final surgery was total ankle joint replacement with bone grafting of the area of osteonecrosis. Results: After 5 years of follow up the patient has progressed out of his AFO to full weightbearing. He reports no ankle pain, improved function and range of motion and is ambulating independently with no assistive devices. Conclusion: We successfully treated a case of distal lateral tibial osteonecrosis, and a 20 degree ankle valgus with staged deformity correction and ankle replacement. Radiographs demonstrate a well seated and positioned implant. We believe that with proper alignment that total ankle arthroplasty is a safe treatment option in the face of bone infarction.

Skeletal involvement in type 1 Gaucher disease: Not just bone mineral density

Gaucher disease is characterized by multi-organ infiltration of phospholipid-laden macrophages. Bone involvement is characterized by typical deformities, osteopenia/osteoporosis, pathological fractures, and bone marrow infiltration (avascular osteonecrosis, infarction). Estimation of skeletal disease includes bone quality that contributes substantially to bone strength. We studied 23 type 1 Gaucher patients (median age 22years, range 3–73) on Enzyme Replacement Therapy from 2months to 26years (median 7years); 4 patients had pathological fractures, 10 bone infarctions, 6 avascular osteonecrosis. We noninvasively assessed bone quality by trabecular microarchitecture and macroscopic geometry, using two innovative dual-energy X-ray absorptiometry tools: Trabecular Bone Score (TBS) and Hip Structural Analysis (HSA). Bone quality parameters distinguished the patients with skeletal complications. TBS was significantly lower in patients with avascular osteonecrosis (p=0.049) and pathological fractures (p=0.024), while it could not identify those with bone infarctions. Among HSA parameters, the Cross Sectional Area of the intertrochanteric region and the Buckling Ratio of the narrow neck allowed the distinction of patients with avascular osteonecrosis. BMD was low in 11 patients (50%); neither BMD nor HSA were associated with pathological fractures. The combined evaluation of bone quality and bone quantity is useful to identify GD patients with more severe skeletal involvement.

Abstracts of the 57th Annual Scientific Meeting of the British Society for Haematology, 27-29 March 2017, Brighton, UK.

Background: Sickle cell anaemia (SCA) is associated with frequent episodes of vaso-occlusive pain crises, but many patients also experience chronic daily pain, which may be due to avascular necrosis of joints, bone infarction, osteomyelitis or due to intractable chronic pain without obvious pathology2 or neuropathic pain3. Not all patients experience a significant chronic pain burden4. The impact of chronic sickle cell pain on the brain is not yet clear; a recent resting-state fMRI study showed a correlation between functional connectivity of the default-mode network and number of hospitalizations for pain5. In other chronic pain conditions, reduced grey matter volume has been noted in various cortical regions involved in pain processing such as the anterior6 and posterior7-9 cingulate cortex (ACC and PCC, respectively), primary motor cortex9 (M1), primary somatosensory cortex5,6 (S1) and insula cortex. The aim of this study is to investigate the relationship between daily pain and a priori selected grey matter regions. Methods: As part of the baseline assessments for the Prevention of Morbidity in Sickle Cell Anaemia (POMS2b) phase-II trial, 52 children and adults recorded baseline daily pain and underwent neuropsychological testing and MRI in the 14 days prior to randomisation. Daily pain was recorded on a numerical scale 0-10 for 14 days. Patients were split into Low-Pain group (50% of recorded days in pain). Neuropsychological assessment was carried out using: Wechsler full-scale IQ (FSIQ), working memory index (WMI) and processing speed index (PSI), Delis-Kaplan Executive Function System Tower and Sorting subtests. Patients underwent MRI on a 3T Siemens Prisma that included T2-weighted sequences to diagnose silent cerebral infarction and a 3D T1-weighted MPRAGE. Cortical parcellation was carried out using Freesurfer. A model was fitted to the imaging data variables to investigate differences between low-pain and highpain groups, controlling for age, gender and presence of SCI and socioeconomic status10 (Index of Multiple Deprivation decile by UK postcode). Results: Five children were excluded from analysis (poor quality data or braces artefact). The final sample was 47 children and adults with SCA. There was a significant correlation with percentage of days in pain and age (r = 0.53, p = 0.0001). In the low-pain group, 11 patients reported no pain during the recorded days. High-pain patients had significantly lower WMI and a trend for lower FSIQ, PSI, Sorting-correct sorts and Sorting-free sorting description. High-pain patients had significantly reduced cortical thickness in the mean cortex of the left and right hemispheres, and significantly thinner cortex in the bilateral precuenus, bilateral PCC, left M1 and right caudal ACC. There was a trend for positive correlation between average pain and right-hemisphere rostral ACC thickness. Conclusion: This study is the first to describe structural brain abnormalities in patients with high burden of sickle cell pain. These patients are part of a 6-month randomised controlled trial of overnight respiratory support with cognitive and pain endpoints as well as repeat MRI; this study may provide structural biomarkers that may parallel amelioration of chronic daily pain.

Multifocal Osteonecrosis in Systemic Lupus Erythematosus

Clinical Image: A 55 years old African male known case of SLE, Hypertension and atrial fibrillation was admitted in Internal medicine unit with acute ischemic stroke. He was on 10 mg prednisolone, azathioprine 50 mg BD and hydroxychloroquine 200 mg once daily and was in remission. His last SLE flare was 6 months back. On day 3, he complained of left knee pain. Left knee and lower thigh were tender without any swelling or erythema. Radiographs of left knee joint showed serpiginous sclerosis (A) along the distal femur and proximal tibia suggestive of bone infarcts. No periosteal reaction was detected and joint space was preserved. MRI lower limbs showed bilateral involvement of distal femur and proximal tibia with intramedullary lesion (B) having serpiginous borders and characteristic double line sign consisting of high signal intensity inside and a low intensity peripheral rim (C ) on T2WI /STIR and TIWI (D) (Figure 1). His pain was controlled with simple analgesics (NSAIDs, acetaminophen). He was pain free on follow up visit in 4 weeks.

Prevalence of autoantibodies in the course of Gaucher disease type 1: A multicenter study comparing Gaucher disease patients to healthy subjects

ObjectivesType 1 Gaucher disease may be related to the presence of autoantibodies. Their clinical significance is questioned.Primary endpoint was to compare the prevalence of autoantibodies in type 1 Gaucher disease patients with healthy subjects, seeking correlations with autoimmune characteristics. Secondary endpoints were to determine whether patients with autoantibodies reported autoimmunity-related symptoms and if genotype, splenectomy or treatment influenced autoantibodies presence. MethodsType 1 Gaucher disease patients and healthy volunteers were included in this national multicenter exploratory study. Autoantibodies presence was compared in both groups and assessed regarding to genotype, splenectomy, Gaucher disease treatment and autoimmunity-related symptoms. ResultsTwenty healthy subjects and 40 type 1 Gaucher disease patients were included. Of the studied group: 15 patients undergone splenectomy, 37 were treated either with enzyme replacement therapy (34) or with substrate reduction therapy (3), 25 were homozygous/heterozygous for the N370S mutation.In type 1 Gaucher disease group (studied group), 52% had positive autoantibodies versus 26% in control group. Antiphospholipid antibodies were more frequent in the studied group (30% vs. 5%), but without correlation to thrombosis, osteonecrosis or bone infarcts. In the studied group, antinuclear antibodies were more frequent (25% vs. 16%). None of the patients with autoantibodies had clinical manifestations of autoimmune diseases. Autoantibodies were not correlated with treatment, genotype, or splenectomy, except for anticardiolipid, more frequent in splenectomized patients. ConclusionsIn type 1 Gaucher disease, autoantibodies were more frequent compared to a healthy population. However, they were not associated with an increased prevalence of clinical active autoimmune diseases.

The Role of Whole-Body Magnetic Resonance Imaging (WB-MRI) in Patients with Paroxysmal Nocturnal Hemoglobinuria (PNH)

▪INTRODUCTION: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal blood disorder of the hematopoietic stem cells characterized by chronic, uncontrolled complement-mediated intravascular hemolysis resulting in thrombophilia with thromboembolic events (TEs) and cytopenia due to bone marrow failure. PNH is the most vicious acquired thrombophilic state and TEs represent the leading cause of morbidity and mortality in PNH. PNH clone size correlates with the possibility to acquire a thrombosis but thromboses are also present in patients (pts) with small clones. Early detection of TEs is critically to improve the outcome in PNH with the availability of the terminal complement inhibitor eculizumab.METHODS: Between Dec 2013 and Jan 2016 whole-body magnetic resonance imaging (WB-MRI) scans at 1.5 Tesla (T) (Siemens MAGNETOM® Avanto) were performed in 37 pts (51% (19/37) females; median age 44 years (range 24-73 years), median D-dimer 0.23 mg/l (range 0.2-5.1 mg/l) with either PNH (n=23) or AA/PNH-syndrome (n=14). The protocol included a cranial time-of-flight magnetic resonance angiography (TOF-MRA), an arterial and venous contrast-enhanced MRA (CE-MRA), and a T1-weighted contrast-enhanced fat-suppressed gradient-echo sequence (radial volumetric interpolated breath-hold examination (VIBE)). Median clone size of GPI-deficient granulocytes (FLAER) was 88% (range 2.6-100%) and all pts were treated according to the German PNH guidelines, including the use of the terminal complement inhibitor eculizumab (70% (26/37)), with eculizumab being initiated in 23 pts prior to MRI. In the remaining pts eculizumab was initiated post MRI for reasons other than TEs. In 64% (24/37) of the pts no TEs were documented prior to the MRI scans. Two pts had a possible clinical history of TEs, including pulmonary embolism (PE) or deep venous thrombosis (DVT). The remaining pts (29% (11/37)) had a history of venous thromboses (e.g. DVT (5/11), portal venous thromboses (4/11), or vena caval thromboses (2/11)). One pt even had a myocardial infarction and two had a cerebral venous sinus thrombosis or a thalamic infarction. Six pts (16%) had more than just one documented TE in their medical history.RESULTS: In pts with the history of TEs we did not observe a progression of the existing TEs. There was no evidence of PE throughout observation time. In pts under chronic eculizumab therapy and prior TEs, additional bone infarcts involving the lower extremities were seen in one pt. In a second pt the diagnosis of a bilateral renal infarction was made. In two pts with no history of TEs bone infarcts of the lower extremities were seen (clone size >50%). In another pt a previously undiagnosed left renal infarction was observed. Moreover, a clinical non-critical occlusion of the right posterior tibial artery was identified in one pt.CONCLUSIONS: In contrast to previous studies there was no evidence for silent major TEs (e.g. PE) at the time of analysis. We were able to identify previously undiagnosed renal and bone infarctions (overall unknown prevalence) in our cohort of PNH pts. This is the first description of bone infarctions as possible thromboembolic complications in PNH. WB-MRI scans seem to be a novel and feasible method for the assessment of the whole vascular status of a PNH pt and allow the detection of previously unidentified vascular complications. This might directly affect treatment as eculizumab is indicated in PNH pts with TEs. Furthermore, in pts with existing TEs WB-MRI scans might be implicated to document their impact prior to treatment, especially in pts with major TEs or in pts with high disease acitivity and suspicion for TEs (e.g. recurrent abdominal pain or persisting dyspnea). DisclosuresDührsen:Amgen: Honoraria, Research Funding; Roche: Honoraria, Research Funding; Alexion Pharmaceuticals: Honoraria, Research Funding. Röth:Alexion Pharmaceuticals: Honoraria, Membership on an entity’s Board of Directors or advisory committees, Research Funding; Novartis: Honoraria.

Management of Severe Chronic Pain with Methadone in Pediatric Patients with Sickle Cell Disease

IntroductionPatients with sickle cell disease (SCD) experience acute painful events; some patients develop chronic pain requiring daily short acting opioids, and frequent emergency department (ED) visits, and inpatient hospitalization. From the Cooperative Study of Sickle Disease (Platt, NEJM 1991), only 1% of patients with SCD experience more than 6 pain events per year. Methadone is a synthetic opioid used in chronic pain, but there is a paucity of data on its use and effectiveness in the pediatric SCD population. We hypothesized that methadone can be used safely in pediatric patients with SCD with severe chronic pain and would reduce ED visits and hospitalizations.MethodsWe conducted a retrospective cohort study (IRB approved) of 16 pediatric patients with SCD who received methadone for chronic pain management, indicating having more than 5 pain events per year. These patients were among 1100 total patients with SCD at our center. Primary SCD providers escalated doses to maximal effect and when clinically stable, doses were weaned. Follow-up started at time of methadone initiation and ended when a patient was weaned or transitioned to adult care; the 1 year period before treatment initiation was defined as the baseline period. We calculated descriptive statistics to characterize the study population. We compared clinical outcomes (ED visits or hospitalization for pain) for the baseline period and 1 year post methadone initiation using paired T test. To evaluate methadone dosing and dependency, we identified the mean dose, percentage of time at highest dose, and wean of methadone. We assessed withdrawal and safety by reviewing outpatient encounters, EKG screening for QTc > 450, eGFR by cystatin C to determine either a decrease in eGFR by 20% or an eGFR <80, and microalbumin/creatinine urinary excretion.ResultsAmong the 16 participants, 10 (62.5%) were male and the genotypes of each patient were: 14 SS, 1 S/β0-thalassemia, 1 SC. The mean age was 15.5 (±2.8) years at time of methadone initiation and there was a medium follow-up of 2.1 years. Clinically, 13 (81.3%) had previous cholecystectomy, 6 (37.5%) had previous splenectomy, 7 (43.8%) had radiographic evidence of infarction of a long bone within the year prior to initiation of methadone, 10 (62.5%) were receiving chronic transfusion during methadone, and 10 (62.5%) received psychosocial counseling for depression and/or anxiety. The average number of ED visits per month was 0.31 (±0.27) in the baseline period and were 0.28 (±0.28, p=0.658) and 0.31 (±0.25, p=0.921) in the 1 year post and entire follow-up periods. The rate of hospitalizations per month decreased from 0.35 (±0.19) in the baseline period to 0.19 (±0.17, p=0.016) and 0.22 (±0.21, p=0.05) in the 1 year post and entire follow-up periods. The average initial starting dose was 12.5 mg and the mean highest dose was 26.6 mg. In 14 (87.5%) patients the final dose was less than the highest dose and 4 (25.0%) patients were weaned completely off of methadone. For the entire cohort, each patient spent a median of 183 days at their highest dose, a mean of 34% of the total time treated with methadone. No patients experienced signs consistent with opioid withdrawal during the follow-up period. The most common subjective complaints were sedation (12%), nausea and/or vomiting (24%) and constipation (6%). We found no documented new abnormal QTc intervals, changes in eGFR, or new onset proteinuria during the follow up period.ConclusionMethadone use in pediatric patients with SCD is well-tolerated and may lead to improved pain control as evidence by significant reduction in hospitalizations. Additionally, when followed closely, methadone can be used safely without concern for physiologic dependency; this is supported by our ability to wean patients off methadone entirely or weaning to a lower dose than their highest dose in the majority of the cohort. Hematologists should consider methadone as a part of chronic pain management in patients with SCD and severe chronic pain. DisclosuresLebensburger:ASH: Research Funding; NHLBI K23: Research Funding.

Intraosseous lipoma - A rare cause of ‘cystic’ lesion of calcaneum

Intraosseous lipoma is an uncommon benign bone neoplasm with most of them detected incidentally on multimodality imaging while being evaluated for an unrelated pathology. Long and cancellous bones are commonly involved. This lesion can present a diagnostic challenge to those uninitiated in its appearence because they can be often mistaken for other benign or malignant bone lesions such as enchondroma, fibrous dysplasia, osteoblastoma, bone infarct, bone cyst and chondrosarcoma. Here we report a case of symptomatic calcaneal lipoma in a 50 year old lady who presented with heel pain. Plain X-ray suggested cystic lesion of calcaneum. MRI revealed the presence of intralesional fat and histopathological examination confirmed the diagnosis. We present this report for its rarity and the clinching MRI features of this lesion, the excellent prognosis and near negligible recurrence rates, so that misdiagnosis leading to unnecessary workup, biopsy and treatment may be avoided, especially in asymptomatic patients.

Skeletal involvement in Gaucher disease: An observational multicenter study of prognostic factors in the Argentine Gaucher disease patients.

Patients with Gaucher type 1 (GD1) throughout Argentina were enrolled in the Argentine bone project to evaluate bone disease and its determinants. We focused on presence and predictors of bone lesions (BL) and their relationship to therapeutic goals (TG) with timing and dose of enzyme replacement therapy (ERT). A total of 124 patients on ERT were enrolled in a multi-center study. All six TG were achieved by 82% of patients: 70.1% for bone pain and 91.1% for bone crisis. However, despite the fact that bone TGs were achieved, residual bone disease was present in 108 patients on ERT (87%) at time 0. 16% of patients showed new irreversible BL (bone infarcts and avascular osteonecrosis) despite ERT, suggesting that they appeared during ERT or were not detected at the moment of diagnosis. We observed 5 prognostic factors that predicted a higher probability of being free of bone disease: optimal ERT compliance; early diagnosis; timely initiation of therapy; ERT initiation dose ≥45 UI/kg/EOW; and the absence of history of splenectomy. Skeletal involvement was classified into 4 major phenotypic groups according to BL: group 1 (12.9%) without BL; group 2 (28.2%) with reversible BL; group 3 (41.9%) with reversible BL and irreversible chronic BL; and group 4 (16.9%) with acute irreversible BL. Our study identifies prognostic factors for achieving best therapeutic outcomes, introduces new risk stratification for patients and suggests the need for a redefinition of bone TG. Am. J. Hematol. 91:E448-E453, 2016. © 2016 Wiley Periodicals, Inc.

Differentiating Rheumatoid Arthritis Related Musculoskeletal Pain in Patients With Concurrent Sickle Cell Disease: A Case Series and Literature Review

Rheumatoid arthritis (RA) can occur concurrently in patients with sickle cell disease (SCD). Both conditions can have similar musculoskeletal symptoms during painful exacerbations. SCD tends to be frequently labelled as the causative factor of painful events by both patients and providers alike. It is important to distinguish between these two conditions causing painful exacerbations to guide treatment plans. Our review of literature showed that the concurrent presentation of RA and SCD is not well described. We present a case series of eight patients with concurrent RA and SCD. We conducted a retrospective review of their charts to make observations about differences in musculoskeletal symptoms, biomarkers and radiological changes. Generally, RA affects smaller joints compared to SCD which affects larger joints. Radiological differences include bone infarcts and avascular necrosis in SCD versus erosive arthritis in RA. In terms of biomarkers, we are better guided using C-reactive protein (CRP) in conjunction with lactate dehydrogenase (LDH). Our case series demonstrates certain indicators which help make a clearer distinction between the two usually painful conditions. J Med Cases. 2016;7(8):334-340 doi: http://dx.doi.org/10.14740/jmc2602e

Results of treatment of lymphoblastic lymphoma at the children cancer hospital Egypt – A single center experience

IntroductionLymphoblastic lymphoma (LBL) and acute lymphoblastic leukemia (ALL) are neoplasms of immature B or T-cell precursors. They are considered as a unique biological entity in the 2008 World Health Organization Classification of Hematologic Neoplasm. Both entities are arbitrarily separated by a cut-off point of 20–25% of blast cells in the bone marrow. Treatment of LBL has evolved over time from conventional high-grade NHL schedules to ALL-derived protocols.The aim of this work is to report the clinical characteristics, overall survival (OS), event free survival (EFS), and common chemotherapy toxicities of lymphoblastic lymphoma (LBL) patients during a 5.5year period.Patients and methodsA Retrospective review of patient’s charts diagnosed and treated as LBL during the period between July 2007 and end of December 2012 was done. Patients were treated according to St. Jude Children Research Hospital ALL Total Therapy XV protocol, standard risk arm.ResultsThis study included 77 patients. T-cell LBL patients were 67, while 10 were of B-cell origin. The median age at diagnosis was 9years (95% CI: 7–10). The majority were males 54/77. Stage III patients were 51, stage IV 13, stage II 11 and stage I 2 patients. Two patients were excluded from analysis as they died before receiving chemotherapy. Complete remission post induction chemotherapy was seen in 22 patients considered early responders, and partial remission in 55 considered late responders. With a median follow up duration of 47months (95% CI: 38–56), the 4year overall survival and event free survival were 86.45% (95% CI: 73.78–94.09) and 82.18% (95% CI: 69.25–90.61) respectively.Twelve patients died during the study period; 2 early deaths before starting chemotherapy from disease progression, 2 in CR due to chemotherapy related toxicity and 8 from disease progression. All the relapsed patients were T-cell, had advanced disease at presentation (6 with stage III; 2 with stage IV). Two patients (2.6%) had isolated local, BM, and CNS relapse each, while 1 (1.3%) had both local and CNS relapse. Disease recurrence was local in 3 patients (3.9%), and systemic in 5 (6.4%), while it was early in 6 (7.8%), and late in 2 (2.6%) patients. Median time to disease progression was 20months (range 5–39months). All relapsed patients did not survive salvage chemotherapy. The most common chemotherapy toxicities were cerebral venous thrombosis (20%), followed by bone infarcts (10.6%), and avascular necrosis (AVN) of head of femur (9.3%). One patient developed secondary acute myeloid leukemia after 3years of FU with unfavorable cytogenetic abnormalities.ConclusionResults of treatment of LBL on the St Jude’s total therapy XV study are comparable to most of the similar reported studies. Outcome of relapsing patients is extremely poor, hence there is a need to identify biologic or clinical prognostic factors including minimal residual tumor to better evaluate chemotherapy response. Steroid induced AVN, and cerebral vascular thrombosis were the main chemotherapeutic adverse events.