椎体压缩性骨折四种影像学诊断的一致性研究

目的：对比分析MRI、CT侧位定位像、CT重建图像和X线平片诊断椎体压缩性骨折的一致性。方法回顾性分析2014年1月1日至2014年4月30日,我院同时行胸椎或腰椎MRI和CT检查的患者50例（398个椎体）,以及同时行MRI和X线平片检查的患者70例（480个椎体）,覆盖T4～L5椎体范围,运用半定量分级方法,按椎体压缩程度分为0～3级,分别统计MRI-CT组和MRI-X线平片组中MRI、CT侧位定位像、CT重建图像、X线平片的各级椎体的个数,采用kappa分析得到MRI与CT侧位定位像、CT重建图像诊断压缩性骨折≥1级和≥2级的椎体的一致性,然后分析 MRI 与 X 线平片诊断压缩性骨折≥1级和≥2级的椎体的一致性;两组分别以 MRI 为标准,0级为阴性结果,≥1级为阳性结果,计算侧位 CT 定位像、CT重建图像和X线平片诊断椎体压缩性骨折的敏感性和特异性。结果 MRI与CT侧位定位像诊断≥1级椎体有中等程度的一致性（ k＝0.583）,诊断≥2级椎体有好的一致性（ k＝0.818）;MRI与CT重建图像诊断≥1级和≥2级椎体均有好的一致性（ k＝0.836和0.961）;MRI与X线平片诊断≥1级椎体有中等程度一致性（ k＝0.651）,诊断≥2级椎体有好的一致性（ k＝0.862）;MRI-CT组中CT侧位定位像诊断椎体压缩性骨折的敏感性和特异性分别为55.2%和97.1%,CT重建图像诊断的敏感性和特异性分别为81.6%和98.5%;MRI-X线平片组中X线平片诊断椎体压缩性骨折的敏感性和特异性分别为62.5%和96.7%。结论 MRI和CT重建图像诊断椎体骨折的一致性最高;相对 MRI,CT 重建图像诊断椎体压缩性骨折的敏感性和特异性均较高,而CT侧位定位像和X线平片的特异性较高,敏感性较低。     

Bone health in children and adolescents after allogeneic stem cell transplantation: high prevalence of vertebral compression fractures

BACKGROUND: This cross-sectional study evaluated the overall bone health and the prevalence of vertebral complications after stem cell transplantation (SCT) in prepubertal children and adolescents. METHODS: A total of 44 children and adolescents (median age, 10 years) were evaluated at a median of 3.8 years after SCT for areal bone mineral density (aBMD) with dual-energy X-ray absoptiometry and for vertebral fractures with instant vertebral assessment. Pretransplant and posttransplant medications and nutritional parameters were recorded, and plasma levels of vitamin D, calcium, phosphate, and parathormone were measured. RESULTS: Of the 44 patients, 16 (36%) had a BMD Z-score of <-1.0. The patients with low BMD did not differ from the others with regard to their clinical or biochemical characteristics. Prepubertal patients had better BMD Z-scores at all sites compared with pubertal or postpubertal subjects. This was evident especially at the hip, in which the median aBMD Z-score in prepubertal patients (-0.2; range, -0.5 to +1.7) was found to be significantly higher than in pubertal (-1.1; range, -1.5 to +0.4) and postpubertal (-1.1; range, -2.6 to +0.5) patients (P = .03). Five patients (11%) had a history of peripheral fractures. Nine patients (20%) had vertebral compression fractures, which were asymptomatic in 7 patients. CONCLUSIONS: Approximately one-third of patients who had undergone allogeneic SCT in childhood were found to have a reduced BMD before reaching adulthood. This was due in part to inadequate BMD gain during the pubertal years. The high prevalence of asymptomatic vertebral compression fractures calls for the systematic assessment of spinal health during the posttransplantation follow-up.     

Lymphomatous presentation of childhood acute lymphoblastic leukemia. A subgroup at high risk of early treatment failure.

Multivariate analyses of the clinical course of 1537 children with acute lymphoblastic leukemia (ALL) identified a subgroup which experienced short remission duration and a high incidence of extramedullary relapse. The patients differed from other ALL patients by the presence at diagnosis of two or more of a constellation of clinical and laboratory features: organomegaly or mass disease, E-rosette positivity, hemoglobin level greater than 10 g/dl, leukocyte count greater than 50,000/microliters, male predominance, and older age. This type of presentation of ALL is referred to as the "lymphoma syndrome" (LS) since such patients exhibit a pattern of several clinical and laboratory features which were observed repeatedly but in differing combinations, and some of which clinically resemble lymphoma. A subsequent database from 2231 patients was analyzed. Patients with a mediastinal mass, massive splenomegaly, or massive adenopathy, alone or in combination, had a worse outcome when the patient also had either leukocytosis, E-rosette-positive lymphoblasts, or a normal or near normal hemoglobin (Hb) level at diagnosis. Similarly, the above three laboratory features alone or in combination did not predict less than 40% disease-free survival (DFS) unless they were accompanied by at least one of the clinical features of mass disease. When at least one clinical feature and at least one laboratory feature were present, the overall DFS was 36% 6 years after diagnosis versus 64% for all other patients. The association of these features with poor prognosis remained significant after adjusting for the level of leukocyte count at diagnosis, age at diagnosis, and sex of the patients. Patients with this recurrent syndrome of features do not represent a homogeneous biologic entity but they constitute a subgroup of patients with ALL having a high risk of treatment failure using current therapies, including failure to achieve remission, early relapse, and increased frequency of relapse in extramedullary sites. They deserve early recognition at diagnosis and selection of treatment strategies appropriate for very high risk ALL.     

The spectrum of metabolic bone disease in lymphoblastic leukemia

Eight patients with childhood acute lymphoblastic leukemia (ALL) and hypercalcemia, osteopenia, or vertebral compression fractures seen at our institution during the last 12 years were evaluated for biochemical evidence of bone disease. Five patients were hypercalcemic, three had abnormal phosphorous levels, and four had elevated alkaline phosphatase values. Parathyroid hormone (PTH) was measured by a polyvalent radioimmunoassay in five patients and these levels were abnormally high in three patients. Four of these five patients also had PTH measured by a midregion-specific radioimmunoassay. One patient had a high PTH value. Two patients had low levels and one patient had a normal PTH level. Although these studies suggest diverse biochemical mechanisms may be contributing to the bone changes and hypercalcemia seen in childhood ALL, ectopic PTH production as well as ectopically produced fragments of PTH may have a role in mediating bone resorption and hypercalcemia.     

Differing complications of hyperleukocytosis in children with acute lymphoblastic or acute nonlymphoblastic leukemia

We assessed the frequency and outcome of complications associated with hyperleukocytosis in children who had received remission induction therapy for either acute lymphoblastic leukemia (ALL) or acute nonlymphoblastic leukemia (ANLL). Among 234 consecutive patients with a leukocyte count of 100 X 10(9)/L or greater at diagnosis, the frequency of early death was significantly higher in those with ANLL (23% v 5% for patients with ALL, P less than .001). The risk of early death increased with increasing leukocyte count, especially when it exceeded 300 X 10(9)/L in patients with ANLL (P less than .001). Intracerebral hemorrhage occurred in eight (11%) of the ANLL patients and accounted for six of the 17 early deaths; in all but two instances, the complication was associated with coagulopathy. Respiratory failure, presumably from pulmonary leukostasis, resulted in six other early deaths in ANLL patients. By contrast, intracerebral hemorrhage occurred in only two of the ALL patients (1.2%); both had normal coagulation studies, but leukocyte counts greater than 400 X 10(9)/L. Severe metabolic derangements from blast cell lysis accounted for three of the eight early deaths among patients with ALL. Leukapheresis or exchange transfusion effectively lowered the leukocyte counts of all 15 patients who received the procedures. Either method may be preferable to emergency cranial irradiation for preventing the complications of hyperleukocytosis in children with acute leukemia.     

Elevated serum transaminase activity at diagnosis is associated with rapidly progressing disease in children with acute lymphoblastic leukemia

The authors study whether quantitative measurement of serum glutamate pyruvate transaminase (SGPT) activity might serve as an indicator of liver involvement at diagnosis of acute lymphoblastic leukemia (ALL) and could be used as a parameter in predicting the prognosis of individual patients. The series consisted of 123 children with newly diagnosed untreated ALL. The mean follow-up time was 69 months (range, 22-140 months). The SGPT activity at diagnosis was significantly associated with the duration of event-free survival (P less than 0.0001). For the 13 patients with SGPT activity greater than 40 IU/l the 5-year event-free survival was only 9%. All 11 patients with the lowest SGPT activities (less than 8 IU/l) remain in primary remission (24 to 81 months). The relative risk of relapse or death for children with SGPT activities of less than 8 IU/l, 8-40 IU/l, and greater than 40 IU/l were 0.2 (95% confidence limits 0.09-0.45), 1, and 4.8 (2.2-10.7), respectively (P less than 0.001). The SGPT activity at diagnosis was not associated with clinically assessed liver enlargement. The authors speculate that high SGPT activities at diagnosis are associated with a rapidly progressing, fulminant form of ALL.     

Bony morbidity in children treated for acute lymphoblastic leukemia.

PURPOSE: Corticosteroids are widely used in the treatment of acute lymphoblastic leukemia (ALL). To determine the frequency of corticosteroid-associated bony morbidity in children with ALL, we retrospectively evaluated the incidence of fractures and osteonecrosis (ON) on two consecutive pediatric ALL protocols. PATIENTS AND METHODS: One hundred seventy-six consecutive children were treated for ALL between 1987 and 1995 at the Dana-Farber Cancer Institute and Children's Hospital. Prednisone was used as the corticosteroid during postremission therapy from 1987 to 1991, and dexamethasone was used from 1991 to 1995. Medical records for all patients were reviewed to assess the occurrence of fractures and ON. RESULTS: With a median follow-up of 7.6 years, the 5-year cumulative incidence (CI) +/- SE of any bony morbidity for the 176 patients was 30% +/- 4%, with a 5-year CI of fractures of 28% +/- 3% and of ON of 7% +/- 2%. With multivariate analysis, independent predictors of bony morbidity included age 9 to 18 years at diagnosis (P <.01), male sex (P <.01), and treatment with dexamethasone (P =.01). Dexamethasone was associated with a higher risk of fractures (5-year CI, 36% +/- 5% v 20% +/- 4% with prednisone; P =.04), but not ON (P =.40). The 5-year event-free survival for the 176 patients was 79% +/- 3%. CONCLUSION: Children treated for ALL had a high incidence of fractures and ON. Older children, boys, and patients receiving dexamethasone were at increased risk for the development of bony morbidity. Future studies should attempt to minimize corticosteroid-associated bony morbidity without compromising clinical efficacy.     

The clinical and biological correlates of coagulopathy in children with acute leukemia

We determined the clinical and biological correlates of coagulopathy in a large series of patients with untreated childhood acute leukemia. Twenty-five of 805 children with acute lymphoblastic leukemia (ALL) (3.1%) and 27 of 195 with acute myeloid leukemia (AML) (13.8%) met any two of three requirements for a coagulation disorder: fibrinogen level less than 150 mg/dL; fibrin degradation products greater than 10 micrograms/mL; and prolongation of prothrombin time (PT) greater than 12 seconds, activated partial thromboplastin time (PTT) greater than 45 seconds, or thrombin time (TT) greater than 18 seconds. Patients with ALL complicated by abnormal coagulation were more likely to be boys and to have a T-cell immunophenotype, a high leukocyte count, a mediastinal mass, leukemic involvement of the CNS, hepatosplenomegaly, and L2 blast cell morphology. These features were highly interrelated, with only T-cell markers and CNS involvement achieving independent significance in a multivariate logistic regression model. Hepatomegaly, blast cell morphological subtype (French-American-British [FAB] M3, M4, and M5) and age less than 2 years were each associated with coagulopathy in patients with AML, although age failed to retain importance after logistic regression analysis. The presence of coagulopathy at diagnosis of ALL did not influence the rate of remission induction (P = .55). By contrast, only 14 of 27 children with coagulopathy at diagnosis of AML achieved a complete remission (CR), compared with 129 of 168 other patients who lacked this complication (P = .003). After multivariate analysis, coagulopathy remained independently associated with failure to attain remission in AML (P = .02). Fatal hemorrhagic complications arising in the CNS or lungs accounted for nine of the 13 induction failures in this group. The presence or absence of coagulopathy had no discernible influence on treatment outcome among patients with either ALL or AML who attained a CR. Laboratory evidence of a coagulation defect may be useful in identifying patients with AML who have a greater risk of induction failure and, hence, require close surveillance and intensive replacement therapy to prevent fatal hemorrhagic complications.     

Evidence for hypomethylation in two children with acute lymphoblastic leukemia and leukoencephalopathy

BACKGROUND: The prognosis of patients with acute lymphoblastic leukemia (ALL) in childhood has improved with intensive chemotherapy. In particular, central nervous system (CNS) leukemia has been well controlled by the presymptomatic administration of intrathecal methotrexate (MTX), high dose systemic MTX, and irradiation. However, the prolonged intrathecal administration and/or the administration of high doses of systemic MTX, especially when combined with irradiation, can lead to leukoencephalopathy (LE), a serious CNS complication of such prophylaxis. Because the mechanisms by which MTX causes this complication have not been elucidated, the authors investigated the transmethylation status of the cerebrospinal fluid (CSF) in two children with ALL and LE to investigate the pathophysiology of that disorder. METHODS: The levels of S-adenosylmethionine (SAM) and S-adenosylhomocysteine (SAH) were measured in the CSF of 2 children with ALL and LE, 7 children with ALL only who were undergoing presymptomatic administration of MTX, and 18 reference children in whom diagnostic lumbar puncture was indicated for other reasons. A sensitive, high performance liquid chromatography (HPLC) method was used with fluorescence detection. RESULTS: The concentrations of SAM in the CSF were lower in the patients with ALL during treatment with MTX compared with the reference children. The SAM levels in the 2 patients with both ALL and LE were slightly lower than the levels in the 7 patients with ALL only. The SAH concentrations in the CSF were higher in the patients with ALL and LE compared with the patients with ALL only and the reference children. The mean concentration of SAH in the CSF was similar in the reference children to that found in the 7 patients with ALL only. The SAM-to-SAH ratios were lower in the 2 patients with ALL and LE and in the 7 patients with ALL only compared with the reference children. The ratios in the patients with ALL and LE were still lower than in those with ALL only, thus providing supporting evidence of hypomethylation in the 2 patients with ALL and LE. CONCLUSIONS: The data suggest that the treatment of children with ALL using MTX causes subclinical hypomethylation and that progressive hypomethylation in the CNS, as evidenced in the 2 patients with ALL and LE, may be responsible for the demyelination in the LE induced by MTX.     

Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy

Cerebrospinal fluid (CSF) amino acid concentrations were measured in 45 children with acute lymphoblastic leukemia (ALL). Central nervous system (CNS) disease was absent in 34 and present in 11 (Groups L and M, respectively) at diagnosis. Thirty-two otherwise healthy children with febrile convulsions were studied for comparison. Results from this study show that glutamine levels at Day 0 were significantly higher in patients than in controls. Patients in Group M had elevated glutamine levels compared to Group L. In comparison, at Day 14, concentrations of glutamine and asparagine decreased, while glutamic acid amounts increased significantly in Group L. Glutamine levels fell at Day 42 in Group M, which may have resulted from more intensive treatment. From this study we hypothesise that higher baseline glutamine levels are indicative of a greater risk for CNS leukemia. Large-scale prospective trials are required to confirm increased baseline CSF glutamine levels in ALL patients, to identify glutamine as a marker for CNS disease and to clarify underlying mechanisms regulating glutamine in ALL.     

Expression of a novel surface antigen MKW in childhood acute leukemia has prognostic significance

A monoclonal antibody (MoAB) has been developed which reacts with a previously unidentified hematopoietic cell surface protein called MKW. This MoAB (anti-MKW) does not cluster with antibodies in any of the known cluster groups of differentiation. Blast cell expression of MKW was studied in 196 consecutively diagnosed children with acute lymphoblastic leukemia (ALL), 69 children with previously untreated acute myeloblastic leukemia (AML) and four children with secondary AML. MKW expression, clinical, laboratory and cytogenetic features at diagnosis, and treatment response and duration were examined for significant correlations. MKW was expressed on blasts from 12.8% of children with ALL and 24.6% of children with de novo AML. The expression of MKW appears to be more common in patients with secondary AML (three of four) than de novo AML (17 of 69). In patients with AML, the expression of MKW was correlated with an elevated initial leukocyte count (p = 0.0005) and poorer disease-free survival (p = 0.04). In patients with ALL, the expression of MKW was associated with a lower hemoglobin level (p less than 0.05) and a lower complete remission rate (p = 0.02). At a median follow-up of 4.6 years ALL patients with greater than or equal to 50% MKW+ blasts had a poorer event-free survival (EFS) than both MKW+ patients with 25-49% positive blasts (p = 0.03) and MKW+ patients (p = 0.0001). The disease-free survival was also poorer for ALL patients with greater than or equal to 50% MKW+ blasts (p = 0.02). In Cox regression analysis, the expression of MKW had an independent prognostic significance in children with ALL. As MKW is a unique cell surface antigen and its expression has prognostic significance in acute leukemias in children, further study in a larger series of patients is warranted.     

Vertebral fractures following stereotactic body radiotherapy for spine metastases

Stereotactic body radiotherapy has emerged as one of the preferred treatments for patients with spine metastases, with the potential for long‐term control from lesion irradiation. Post‐treatment vertebral compression fractures are a known complication of this therapy, contributing to worsening pain and reduced quality of life, sometimes requiring surgical intervention. This review explores the current knowledge of post‐radiotherapy fractures, in terms of the rates and associated predictive factors. A search of databases including Medline, Embase and the Cochrane Library was conducted using keywords such as ‘vertebral compression fracture’, ‘stereotactic body radiotherapy’ and ‘spine metastases’. The search was limited to published studies up to March 2019, reporting clinical outcomes including both the post‐treatment fracture rate and statistical identification of associated risk factors. Rates of post‐treatment fractures ranged from 4 to 39%. A variety of factors were found to increase the risk, including the appearance of lytic vertebral disease, degree of pre‐existing compression, spinal malalignment, increased dose per fraction and a Spinal Instability Neoplastic Score >6. This knowledge can enable clinicians to counsel patients when considering management options for spine metastases, maintaining the balance between local tumour control and the risk of subsequent fracture.     

Anterior Intervertebral Disc Herniations in Children Unrecognised Chronic Trauma to the Spine

Five new cases of Anterior Intervertebral Disc Herniations are reported. Anterior intervertebral disc hemiation is a common complication of chronic spinal trauma. It is often accompanied by vertebral fractures which present as localised, mild platyspondyly or as frank vertebral body fractures. Two groups of children are prone to develop the disease. I-Healthy boys active in sport and II -Children with osteoporosis. The differential diagnosis includes discitis, tuberculosis, eosinophilic granuloma and specifically Scheuermann's disease.     

Expression of the multidrug transporter P-glycoprotein is highly correlated with clinical outcome in childhood acute lymphoblastic leukemia: results of a long-term prospective study

The improved cure rate in childhood ALL may be attributed largely to the effective multidrug regimens currently applied in well-designed clinical trials. However, in a minority of patients with ALL, chemotherapy failure remains a leading cause of cancer related death, most probably due to cellular drug resistance. The better-known mechanism of such resistance is mediated by P-glycoprotein (P-gp). In a long term prospective study (mean time of follow-up: 65 months) the multidrug efflux pump P-gp was examined immunocytochemically in leukemic cells of 102 protocol-treated children with de novo acute lymphoblastic leukemia (ALL) and of 37 children with relapsed ALL. Fourteen percent expressed P-gp at initial diagnosis and 35% were P-gp positive at relapse. The patients being P-gp positive at initial diagnosis had a higher rate of leukemic relapse than the P-gp negative patients (P = 0.02). In the relapsing patients, those who were P-gp positive had a 2.18-fold greater risk for leukemic death than those who were P-gp negative. Paired analysis on diagnostic and relapsed samples from 20 patients did not support the hypothesis of P-gp mediated expression being a chemotherapy induced phenomenon. The cumulative event free survival for de novo ALL patients was significantly higher in the P-gp negative patient group. Multivariate analysis showed that P-gp expression is independent of other known risk factors. In conclusion we strongly advise that tests for P-gp in leukemic blasts should be conducted for every child with ALL, since this parameter selects a subgroup of patients with increased risk for leukemic relapse.     

Percutaneous vertebral augmentation in metastatic disease: state of the art

Improvements in diagnosis and treatment have prolonged cancer survival, with a consequent increase in the incidence of spinal metastases and vertebral compression fractures with associated axial pain, progressive radiculomyelopathy, and mechanical instability. Pain relief in malignant vertebral compression fractures is key to achieving a better quality of life in patients under palliative care. The gold standard for pain relief is nonsteroidal anti-inflammatory drugs and opioids. Nonresponsive cases are then treated with radiotherapy, which may require 2-4 weeks to take effect and in most cases does not provide complete pain relief. Percutaneous vertebroplasty and percutaneous kyphoplasty can in particular give relief in patients with vertebral body compression fractures that do not cause neurological deficits but severely compromise quality of life because of intractable pain.     

Skeletal morbidity in childhood acute lymphoblastic leukemia.

PURPOSE: Treatment for acute lymphoblastic leukemia (ALL) in childhood results in a reduction in bone mineral density (BMD). Whether there is a recovery of this lost bone mass in survivors of ALL is not known. We sought to determine if changes in BMD are common long-term sequelae in children with ALL. METHODS: Bone mineral densitometry of the lumbar spine and femoral neck was performed on 106 patients. The results were compared with those of age-matched normal controls. The effect of treatment was examined in those with low BMD compared with the remainder of the study group. RESULTS: When data were tested with respect to age, sex, and age and sex, no difference was observed in BMD between survivors of childhood ALL and controls. In the subgroup of patients with low BMD, the difference was not related to age, age at diagnosis, or years since diagnosis. Low BMD of the spine was not explained by radiotherapy (RT), methotrexate (MTX) dose, or corticosteroid dose. Low BMD of the femur was not explained by RT. However, those with low femoral BMD were more likely to have received high-dose MTX or higher-dose corticosteroids compared with the remainder of the group. CONCLUSION: It appears that survivors of childhood ALL as a whole recover normal BMD. However, those patients who received a total MTX dose of greater than 40000 mg/m(2) or a total corticosteroid dose of greater than 9000 mg/m(2) may not recover normal BMD and therefore should be screened for decreased BMD of the femoral neck.     

Fluoride supplementation status, fractures and osteopenia in children with acute lymphoblastic leukemia

Children with acute lymphoblastic leukemia (ALL) experience fractures on the basis of osteopenia related to chemotherapy administered for the maintenance of remission. It is likely that corticosteroids are the main cause of bone mineral loss in this circumstance. Because fluoride has been used as a therapeutic intervention in osteoporosis, including that induced by corticosteroid therapy, we explored the prospect that children with ALL who received fluoride supplementation (in drinking water or from other sources) may be relatively protected from iatrogenic skeletal morbidity. Children who completed therapy according to the Dana Farber Cancer Institute protocol 87-01 (n=35) were assessed by skeletal radiology and bone densitometry every 6 months from diagnosis. In addition, their families completed a questionnaire relating to fluoride supplementation. There was no correlation between such fluoride supplementation and either the prevalence of fractures or the severity of osteopenia. This outcome may reflect the mainly appendicular location of the fractures in this group of children. These findings, together with a consideration of the risk benefit ratio of fluoride administration to children at large, suggest that such intervention is unlikely to be beneficial in limiting skeletal morbidity during the treatment of ALL in childhood.     

T cell receptor gene rearrangements in B-precursor acute lymphoblastic leukemia correlate with age and the stage of B cell differentiation

Children with ALL diagnosed at less than 2 years of age have a poor prognosis when compared with older children. In an effort to identify biologic features of ALL in children less than 2 that might explain this difference, we performed extensive immunophenotypic and molecular genetic analyses on a series of patients. For comparison purposes patients were divided into four groups: CALLA- (CD10-) infants less than 2 years of age at diagnosis (n = 10), CALLA- children greater than 2 years of age at diagnosis (n = 10), CALLA+ infants (less than 2 years, n = 21) and CALLA+ children (older than 2 years, n = 21). No immunophenotyping differences in CALLA- or CALLA+ subgroups were identified when cases less than 2 were compared with cases greater than 2 years of age at diagnosis. The most interesting results were in the CALLA- group where 94% of the samples expressed the B cell antigen CD19 but 27% co-expressed CD7. Double labeling experiments confirmed leukemic blast cells co-expressed CD19 and CD7. The double-labeled cells represent either leukemic conversion of a precursor cell which has not yet committed to B or T cell lineage or aberrant expression of these antigens. Molecular genetic studies demonstrated that all samples, regardless of the patients' age or immunophenotype, had rearrangement of the Ig heavy chain gene. The most striking molecular results were in CALLA- patients; in patients less than 2 at diagnosis neither the beta- nor the gamma-chain gene of the T cell receptor (TCR) was rearranged, whereas DNA from 5 of 10 patients over the age of 2 demonstrated beta- or gamma-chain TCR gene rearrangements. The percentage of CALLA+ cases under the age of 2 years with rearrangements in TCR genes is less than that found in CALLA+ cases over the age of 2 years. The finding of no TCR rearrangements in CALLA- ALL and a decreased number of gamma-TCR rearrangements in CALLA+ cases under the age of 2 suggest that age may affect TCR gene rearrangements in lymphoblasts. The molecular differences in TCR gene rearrangements do not appear to correlate with the response to therapy.     

Kyphoplasty in the treatment of osteolytic vertebral compression fractures as a result of multiple myeloma.

PURPOSE: We prospectively evaluated the safety and efficacy of kyphoplasty in the treatment of osteolytic vertebral compression fractures resulting from multiple myeloma. The principle symptoms in multiple myeloma result from bone destruction, especially the spine. Kyphoplasty is a new technique that involves the introduction of inflatable bone tamps (IBT) into the vertebral body. The purpose of the IBT is to restore the vertebral body back toward its original height, while creating a cavity that can be filled with highly viscous bone cement. PATIENTS AND METHODS: Fifty-five consecutive kyphoplasty procedures were performed in 18 patients with osteolytic vertebral compression fractures resulting from multiple myeloma. Cement leakage and any complications were recorded. Early objective analysis was made by comparing preoperative and latest Short Form 36 Health Survey scores. Height restoration was estimated by measuring vertebral height on lateral radiographs. RESULTS: The mean age of patients was 63.5 years, mean duration of symptoms was 11 months, and mean follow-up was 7.4 months. There were no major complications related directly to use of this technique. On average, 34% of height lost at the time of fracture was restored. Asymptomatic cement leakage occurred at two (4%) of 55 levels. Significant improvement in SF36 scores occurred for Bodily Pain (23.2 to 55.4, P =.0008), Physical Function (21.3 to 50.6, P =.0010), Vitality (31.3 to 47.5, P =.010), and Social Functioning (40.6 to 64.8, P =.014). CONCLUSION: Kyphoplasty was efficacious in the treatment of osteolytic vertebral compression fractures resulting from multiple myeloma. Kyphoplasty is associated with early clinical improvement of pain and function as well as some restoration of vertebral body height.     

Acute respiratory failure and pulmonary thrombosis in leukemic children

Acute respiratory failure (ARF) in an 11-year-old child with pre-T acute lymphoblastic leukemia (ALL) at the beginning of induction therapy was observed, connected with a pulmonary thrombosis and not with an infective origin. A systematic search for this pathology identified six other children with the same pulmonary complication, five of whom where in the early phase of acute nonlymphoblastic leukemia (ANLL) and one in induction therapy for ALL in marrow relapse. At the beginning of the symptomatology, all children presented severe hypoxia and hypercapnia, with no or minimal chest radiograph abnormalities and no clear hemodynamic involvement. In all patients the arteriography and nuclear imaging studies confirmed the diagnosis. The causes of the thrombi could be connected with neoplastic emboli after cell lysis and/or with the vascular damage resulting from antiblastic therapy. Intravenous urokinase treatment and respiratory assistance had been successfully carried out in six of seven children.