Cumulative incidence of secondary neoplasms as a first event after childhood acute lymphoblastic leukemia

Context  Little is known about the incidence of secondary neoplasms after 15 to 20 years in children and adolescents who were treated for acute lymphoblastic leukemia. Objectives  To investigate the cumulative incidence of secondary neoplasms in pediatric patients treated for acute lymphoblastic leukemia over 30 years and to characterize late-occurring tumors. Design, Setting, and Patients  Retrospective study of 2169 patients with acute lymphoblastic leukemia treated between 1962 and 1998 at St Jude Children's Research Hospital, Memphis, Tenn, who achieved complete remission and had a median follow-up time of 18.7 years (range, 2.4-41.3 years). Main Outcome Measures  Cumulative incidences of secondary neoplasms in first remission and standard incidence ratios of observed rates compared with rates of cancer development in the general US population. Results  Secondary neoplasms developed as the first event in 123 patients and comprised 46 myeloid malignancies, 3 lymphomas, 14 basal cell carcinomas, 16 other carcinomas, 6 sarcomas, 16 meningiomas, and 22 other brain tumors. The cumulative incidence of secondary neoplasm was 4.17% (SE, 0.46%) at 15 years and increased substantially after 20 years, reaching 10.85% (SE, 1.27%) at 30 years. When meningiomas and basal cell carcinomas were excluded, the overall cumulative incidence was 3.99% (SE, 0.44%) at 15 years and 6.27% (SE, 0.83%) at 30 years, representing a 13.5-fold increase in overall risk compared with the general population. The cumulative incidence of each tumor type at 30 years was 2.19% (SE, 0.32%) for myeloid malignancy, 0.17% (SE, 0.10%) for lymphoma, 3.00% (SE, 0.59%) for brain tumor, 4.91% (SE, 1.04%) for carcinoma, and 0.57% (SE, 0.37%) for sarcoma. Conclusions  The cumulative incidence of secondary neoplasms increases steadily over 30 years after treatment of acute lymphoblastic leukemia. Although the majority of the late-occurring secondary neoplasms are low-grade tumors, the increase in incidence of more aggressive malignant neoplasms is significantly higher than expected in the general population. These results suggest that lifelong follow-up of acute lymphoblastic leukemia survivors is needed to ascertain the full impact of treatment and other leukemia-related factors on secondary neoplasm development.      

High Hb A2 β-Thalassemia Due to a 468 bp Deletion in a Patient with Hb S/β-Thalassemia

We describe a case of Hb S/β-thalassemia (thal) involving a 468 bp deletion that removes the β-globin gene promoter but leaves the coding regions intact. This is the second report of this deletion, and our family study establishes that this deletion causes β⁰-thal with unusually high levels of Hb A₂ and Hb F. As with other genotypes involving deletions of the 5′ region of the β-globin gene, our patient had a mild form of Hb S/β-thal.     

Intensity-modulated stereotactic radiotherapy of paraspinal tumors: a preliminary report

OBJECTIVE: Radioresistant paraspinal tumors may benefit from conformal treatment techniques such as intensity-modulated radiotherapy (IMRT). Local tumor control and long-term palliation for both primary and metastatic tumors may be achieved with IMRT while reducing the risk of spinal cord toxicity associated with conventional radiotherapy techniques. In this article, we report our initial clinical experience in treating 16 paraspinal tumors with IMRT in which the planning target volume was 2 mm or greater from the spinal cord. METHODS: IMRT was administered by using a linear accelerator mounted with a multileaf collimator. Two immobilization body frames developed at Memorial Sloan-Kettering Cancer Center were used for patients with and without spinal implants. During a 30-month period, 16 patients underwent IMRT for metastatic and primary tumors. Eleven patients were treated for symptomatic recurrences after undergoing surgery and prior external beam radiotherapy, and one patient was treated after undergoing radiotherapy for a metastatic pancreatic gastrinoma with overlapping ports to the spine. Four patients with primary tumors were treated after primary resection that resulted in positive histological margins. Twelve patients were symptomatic with pain, functional radiculopathy, or both. Tumoral doses were determined on the basis of the relative radiosensitivity of tumors. Patients with metastatic tumors were administered a median tumoral dose of 20 Gy in four to five fractions and a spinal cord maximum dose of 6.0 Gy in addition to the full tolerance dose administered in previous radiation treatments. The primary tumors were delivered a median dose of 70 Gy in 33 to 37 fractions and a spinal cord maximum dose of 16 Gy. The median tumoral volume was 7.8 cm(3). RESULTS: Of the 15 patients who underwent radiographic follow-up, 13 demonstrated either no interval growth or a reduction in tumor size in a median follow-up period of 12 months (range, 2-23 mo). Two patients, one with a thoracic chondrosarcoma and one with a chordoma, showed tumor progression 1 year after undergoing IMRT. Pain symptoms improved in 11 of 11 patients, and 4 of 4 patients had significant improvement in their functionally significant radiculopathy and/or plexopathy. Pain relief was durable in all patients except the two with tumor progression. No patient showed signs or symptoms of radiation-induced myelopathy, radiculopathy, or plexopathy, including 12 patients with a median follow-up of 18 months. CONCLUSION: IMRT was effective for treating pain and improving functional radiculopathy in patients with metastatic and primary tumors. Although long-term tumor control is not established in this study, high-dose tumoral irradiation can be performed without causing radiation myelopathy in more than 1 year of follow-up.     

Nerve Transfers for Treatment of Isolated Axillary Nerve Injuries

BACKGROUND:

The most common neurological defect in traumatic anterior glenohumeral dislocation is isolated axillary nerve palsy. Most recover spontaneously; however, some have persistent axillary neuropathy. An intact rotator cuff may compensate for an isolated axillary nerve injury; however, given the high rate of rotator cuff pathology with advancing age, patients with an axillary nerve injury are at risk for complete shoulder disability.

OBJECTIVE:

To review reconstruction of the axillary nerve to alleviate shoulder pain, augment shoulder stability, abduction and external rotation to alleviate sole reliance on the rotator cuff to move and stabilize the shoulder.

METHODS:

A retrospective review of 10 patients with an isolated axillary nerve injury and an intact rotator cuff who underwent a triceps nerve branch to axillary nerve transfer was performed. Patient demographics, surgical technique, deltoid strength, donor-site morbidity, complications and time to surgery were evaluated.

RESULTS:

Ten male patients, mean age 38.3 years (range 18 to 66 years), underwent a triceps to axillary nerve transfer for isolated axillary nerve injury 7.4 months (range five to 12 months) post-traumatic shoulder dislocation. Deltoid function was British Medical Research Council grade 0/5 in all patients preoperatively and ≥3/5 deltoid strength in eight patients at final follow-up (14.8 months [range 12 to 25 months]). There were no complications and no donor-site morbidity.

CONCLUSION:

A triceps to axillary nerve transfer for isolated axillary neuropathy following traumatic shoulder dislocation improved shoulder pain, stability and deltoid strength, and potentially preserves shoulder function with advancing age by alleviating sole reliance on the rotator cuff for shoulder abduction and external rotation.     

Characterization of actions taken during the delivery of medication therapy management: A time-and-motion approach

Objectives

To characterize actions performed by pharmacists and support staff during provision of medication therapy management (MTM) and to compare actions performed according to practice characteristics.

Planning and managing a complex multisite study in the age of technology

A 2‐year long, multisite research study that evaluated cardiopulmonary resuscitation skill decay among nursing students was conducted at 10 schools of nursing across the United States. The study was conducted in two phases and required carefully timed sessions for skill performance. Multisite studies in nursing education need to be carefully planned. Time delays should be anticipated with processes and Institutional Review Board protocols across sites. All team members were trained and consistently supported during the entire study. While challenges and obstacles were identified, innovative solutions were implemented that assisted the research team to successfully complete the study. The use of new and existing technology allowed the team to surmount many of the challenges encountered in this study. The purpose of this article is to describe the logistics, processes, challenges, and lessons learned related to conducting a complex multisite study.     

The utility of traditional prehospital interventions in maintaining thermostasis

Objective. Hypothermia can have a negative effect on the metabolic and hemostatic functions of patients with traumatic injuries. Multiple methods of rewarming are currently used in the prehospital arena, but little objective evidence for their effectiveness in this setting exists. The purpose of this study was to assess the relative effectiveness of traditional prehospital measures in maintaining thermostasis in trauma patients. Methods. Participating helicopter and ground ambulance ALS units were prospectively randomized to provide either routine care only (passive or no warming) or routine care (passive warming) in conjunction with active warming (either reflective blankets, hot pack rewarming, or warmed IV fluids). A total of 174 trauma code patients, aged >14 years, who met inclusion criteria were prospectively enrolled by prehospital providers. Patients who received a non-assigned intervention or who had incomplete temperature data were dropped from the analysis. A total of 134 patients were included in the final analysis. Results. Patients who received hot pack rewarming showed a mean increase in body temperature during transport (+1.36°F/0.74°C), while all other groups (no intervention, passive rewarming, reflective blankets, warmed IV fluids, warmed IV fluid plus reflective blanket) showed a mean decrease in temperature during transport [-0.34 to -0.61°F (-0.2 to -0.4°C); p < 0.01]. In addition, the hot pack group was consistent, with every patient who received hot pack warming showing an increase in body temperature during transport, while in all other groups there were patients who had both increases and decreases in temperature. The intervention groups did not differ significantly on exposure to precipitation, transport unit temperature, total prehospital time, initial vital signs, amount of fluid administered, Injury Severity Score, or Glasgow Coma Score. Conclusions. Most traditional methods of maintaining trauma patient temperature during prehospital transport appear to be inadequate. Aggressive use of hot packs, a simple, inexpensive intervention to maintain thermostasis, deserves further study as a potential basic intervention for trauma patients.