Musculoskeletal manifestations in pediatric acute leukemia

BACKGROUND: In children, acute leukemia (AL) at presentation can mimic several orthopaedic pathologies, so that a variable delay of the correct diagnosis is often reported. METHODS: To define more clearly the clinical and radiological musculoskeletal manifestations of leukemia in children, 122 affected children referred from 1984 to 1999 to our Pediatric Onco-Hematologic Clinic were retrospectively reviewed. Average age at diagnosis was 6.6 years (from 7 months to 17 years). Seventy-three (60%) were boys and adolescent boys, 49 (40%) were girls and adolescent girls. One hundred two (83.6%) had acute lymphoblastic leukemia, 20 (16.4%) had acute myeloid leukemia. The mean follow-up was 8.2 years for the 104 survivors and 2.5 years for the 18 nonsurvivors. The chi2 test was used to perform the statistical analyses. RESULTS: At presentation, complaints related to the musculoskeletal system were frequent (38.3%), including pain (34.4%), functional impairment (22.9%), limping (12.3%), swelling (10.6%), and joint effusion (5.7%). At presentation, 40.2% of children had at least 1 radiographic abnormality. In order, they were osteolysis (13.1%), metaphyseal bands (9.8%), osteopenia (9%), osteosclerosis (7.4%), permeative pattern (5.7%), pathological fractures (5.7%), periosteal reactions (4.1%), and mixed lysis-sclerosis lesions (2.5%). Different from previous reports, late radiographic lesions were uncommon (5.7%), probably because of milder newer medication protocols. They included avascular necrosis (3.3%), vertebral collapses (1.6%), and osteolysis (0.8%). CONCLUSIONS: Both clinical and radiological changes had various and no uniform localization. Poor correlation was found between symptoms and radiological lesions. Survival rates in children with AL were 95.8% at 1 year, 89.6% at 3 years, 85.8% at 5 years, and 83.4% at 10 and at 13 years. Radiographic abnormalities (P = 0.400), type of leukemia (P = 0.291), sex (P = 0.245), and white blood cell count at presentation (P = 0.877) were not prognostic factors. The presence of multiple bone lesions did not affect the survival rate (P=0.632). As early diagnosis significantly decreases morbidity and mortality of AL, the orthopaedist should suspect AL in any child with unexplained persistent skeletal pain or radiographic alterations. Accurate history, general physical examination, and complete blood cell count tests should address the suspicion, which is confirmed by a peripheral and/or iliac crest bone marrow biopsy.     

Early renal involvement in acute lymphoblastic leukemia and nonHodgkin's lymphoma in children

Clinical manifestations of kidney disease, particularly renal failure, caused by malignant infiltration in patients with acute lymphoblastic leukemia or nonHodgkin's lymphoma have been described rarely. We report 1 case of acute lymphoblastic leukemia and 3 cases of nonHodgkin's lymphoma in which renal disease was the only or one of the presenting manifestations of malignancy. Of these patients 2 had rapidly progressive renal failure with nephromegaly, 1 presented with bilateral abdominal masses caused by severe nephromegaly and with microscopic hematuria, and 1 had microscopic hematuria without nephromegaly. In all 4 patients kidney biopsy revealed malignant infiltration. In the 2 patients who presented with renal failure kidney function promptly returned to normal after chemotherapy and irradiation of the kidneys. Prompt and correct diagnosis of nephropathy, when it is the only or one of the presenting signs of acute lymphoblastic leukemia or nonHodgkin's lymphoma, is necessary to expedite initiation of specific antitumor therapy.     

High blood pressure and hypertension in children with newly diagnosed acute leukemia and lymphoma

Little has been published on the rate of prehypertension (HBP) and hypertension (HTN) in children with hematologic malignancies. This study was preformed to determine the prevalence and predictors of HBP and HTN in newly diagnosed acute leukemia and lymphoma patients. Retrospectively, blood pressure (BP) values were followed from admission until normalization in 102 children. HBP and HTN were defined as either a systolic or diastolic value ≥ the 90th and 95th percentile BP measurement, respectively. HBP and HTN were identified in 68.6% and 52.9% of children prior to chemotherapy and 78.4% and 67.3% postchemotherapy, respectively. Mean time to BP normalization was 54days. Only ten children (15% of HTN patients) received antihypertensive therapy. Logistic regression determined that the only predictor for HBP and HTN was the estimated glomerular filtration rate (eGFR) at the time of admission—every 10ml/min per 1.73m² increase led to a 16% and 14% decrease in the odds of postchemotherapy HBP (p = 0.02) and HTN (p = 0.03), respectively. A surprisingly high prevalence of BP abnormalities was identified and lower eGFR predicted HBP and HTN in children with newly diagnosed hematologic malignancies. Better recognition and serious consideration for treatment should be given to this cardiovascular abnormality. This study was submitted in abstract form and presented as a poster presentation at the American Society of Nephrology Meeting in St. Louis, Mo, USA, in 2004 (abstract number F-PO630).     

Management of pleural effusions in children with malignant lymphoma

PURPOSE: The aim of this study was to determine potential problems in the diagnosis and management of children with pleural effusions and malignant lymphoma as well as the efficacy of thoracentesis. METHODS: The case histories of six children with malignant lymphoma who presented with pleural effusions were reviewed. Thoracentesis was performed using the Seldinger technique. RESULTS: Four of the children presented with symptoms and chest radiograph findings similar to pneumonia. A large mediastinal mass was present in two children. Pleural fluid analysis resulted in a definitive diagnosis of lymphoma in five of the six children. Two of the children had symptoms of reexpansion pulmonary edema after removal of pleural fluid. An empyema developed in one child after thoracotomy and chest tube placement. Reaccumulation of pleural fluid was common before initiating chemotherapy. CONCLUSIONS: Malignant pleural effusions frequently are present in children with non-Hodgkin's lymphoma. They may present with respiratory distress because of the size of the effusion, the mediastinal mass, or both. Management of these pleural effusions is associated with potential complications, some of which are life threatening. Thoracentesis is the initial diagnostic and therapeutic procedure of choice. The use of a Seldinger technique for thoracentesis has proved useful and safe. In patients with large effusions, aggressive removal of the pleural fluid may be followed by reexpansion pulmonary edema.     

Musculoskeletal problems in blind children

At a regional school for the blind, 157 children were examined for musculoskeletal abnormalities. The incidence of scoliosis was 9.5 per cent; of foot deformity, 53.5 percent; and of ligament laxity, 25 per cent. Thirty-six children (23 per cent) had evidence of spastic, flaccid, or athetoid cerebral palsy or other disorders of the central nervous system. The orthopaedist should evaluate blind children carefully and repeatedly for evidence of occult cerebral palsy and progressive deformity of the spine or foot.     

Avascular necrosis in children with acute lymphoblastic leukemia

This study describes the prevalence and demographics of avascular necrosis (AVN) in children with acute lymphoblastic leukemia (ALL). With improving survival of ALL patients on modern chemotherapy regimens, an increasing number of children with AVN will be presenting to orthopaedists. From 1991 to 1996, 202 patients were treated for ALL at a major tertiary pediatric cancer referral center. Eight patients (4.0%) subsequently developed AVN at an average of 30.0 months after beginning chemotherapy. A total of 27 documented joints were involved, with an average of 3.4 joints affected per patient diagnosed with AVN. The subset of patients with high-risk ALL who underwent an aggressive chemotherapy protocol was particularly susceptible to developing AVN. Six of 58 high-risk ALL patients (10.3%) developed AVN at an average of 18.5 months. As ALL patients now frequently survive into adulthood, orthopaedists will be increasingly called on to manage AVN affecting multiple joints in children and young adults.     

Primary malignant lymphoma of the appendix associated with acute appendicitis

Malignant lymphoma comprises 1-4% of the malignant neoplasm of the gastrointestinal tract. Appendiceal lymphomas are extremely rare, reported in 0.015 percent of all gastrointestinal lymphomas. This is a report of a case of localized malignant lymphoma of the appendix associated with the histological features of acute inflammation that presented clinically as acute appendicitis. A three years follow-up after appendectomy alone did not show any evidence of recurrent disease. This case report emphasizes the importance of routine histology examination of the appendectomy specimen.     

Musculoskeletal consequences of near-drowning in children

Nearly drowned children sustaining cardiac or respiratory arrest or altered neurologic status developed anoxic encephalopathy (30%), died in the ensuing days (36%), or were discharged neurologically normal (34%). In encephalopathic children, spasticity was more malignant than in children with other causes of spasticity. The most common musculoskeletal problem was contracture, especially equinus, hip adductor, hamstring, and quadriceps. Hip subluxation or dislocation occurred in 34% as early as 1 month after injury. Scoliosis developed in 18%, with some requiring bracing and surgery. Thirty-one percent ambulated, and the rest were quadriplegic. Independent sitting or better function by discharge was a predictor of ambulation potential. Of the quadriplegics, 66% were dependent sitters.     

急性白血病患儿的灵性需求及干预

目的改善急性白血病患儿负性情绪及其应对方式。方法将100例急性白血病患儿随机分为对照组与观察组各50例,在常规治疗的基上,对照组给予常规心理护理,观察组根据患儿灵性需求实施针对性干预。连续干预1个月后评价效果。结果干预后观察组患儿焦虑及抑郁评分、应对方式评分显著优于干预前及对照组(均P<0.01)。结论急性白血病患儿存在多方面灵性需求,针对性灵性护理可有效改善其心理状态及应对方式。     

Burkitt's lymphoma revealed by acute intussusception in children]

STUDY AIM: Burkitt's lymphomas are rarely revealed by acute intestinal intussusception in children. The study aim was to report eight cases. PATIENTS AND METHODS: Between 1988 and 1999, eight children, seven boys and one girl (mean age: 6 years) were hospitalized for an acute abdominal syndrome. Abdominal ultrasonography showed intestinal intussusception (n = 8) primitive tumor (n = 2), mesentivic lymph nodes (n = 2) and liver nodes (n = 1). Enema (n = 6) confirmed presence and irreductibility of the intestinal intussusception. A laparotomy was performed on emergency in seven patients and found the primitive tumor in 6. The procedure consisted in disinvagination (n = 4) and intestinal resection for ischaemia (n = 2). One patient was not operated on and the diagnosis was performed through ultrasonography guided tumoral puncture. RESULTS: According to the Murphy classification, there were 2 stage II, 3 stage III and 3 stage IV patients. With LMB protocol chemotherapy, a complete remission was observed following the first cure. All the children were alive at the time of this study with a follow-up longer than one year after the complete remission. CONCLUSION: Abdominal sonography is the most efficient examination for the diagnosis of intestinal intussusception and sometimes of the primitive lesion. In the absence of sonographic intestinal impair, thanks to ultrasonography guided tumoral puncture, diagnosis may be made and chemotherapy started. If the lymphoma is not visualized with ultrasonography, an emergency laparotomy is necessary for the diagnosis of the lymphoma and the intestinal resection in case of necessity. Burkitt's lymphoma is very sensible to chemotherapy.     

Musculoskeletal manifestations of Lyme disease in children

Lyme disease, caused by a tick-transmitted spirochete, has significant musculoskeletal manifestations in children as well as in adults. A series of 23 children with Lyme disease is examined. Acute arthritis was present in five cases, with the knee being involved in three of these cases. Twelve cases (52%) presented with arthralgias, and 10 cases (43%) presented with myalgias. Neurological manifestations were present in 39% of the cases. Five cases have demonstrated recurrent polyarthralgias. Lyme disease should be considered in the differential diagnosis of arthralgias and arthritis in children.     

Necrotizing fasciitis in two children with acute lymphoblastic leukemia.

Necrotizing fasciitis is a severe, soft tissue infection, and is an unusual condition in children. The cornerstone of therapy is prompt, aggressive surgical treatment. Despite vigorous treatment, mortality rates are high. We report the occurrence of necrotizing fasciitis in two children during the granulocytopenic phase of induction chemotherapy for acute lymphoblastic leukemia. The diagnosis and treatment of necrotizing fasciitis in these two children was made more difficult by their underlying disease and its chemotherapy. The successful treatment of their infections relied on a multimodality approach. Aggressive surgical debridement was the mainstay of therapy. Adjuvant therapy was vital to the successful outcomes and included meticulous wound care, intravenous hyperalimentation, appropriate antibiotics, and granulocyte transfusions.     

Renal function during and after treatment for acute lymphoblastic leukemia in children

Renal function tests (cystatin C, serum and urine creatinine, creatinine clearance, serum and urine ₂-microglobulin, microalbuminuria, osmolality) were performed in 21 children at the diagnosis and during the treatment for acute lymphoblastic leukemia (ALL) (group I) and in 37 children (group II) treated for ALL 3.9±3.7 years before the study. The results were compared to 20 healthy children. Mean values of renal tests were in normal range at all points of analysis in groups I and II compared to the control group. Transitory higher cystatin C values (but in normal range) were observed after methotrexate administration and after the end of treatment. Deteriorated renal function was observed in one child during the treatment (after each protocol) and in five children treated previously for ALL. In conclusion, combined treatment for ALL is not associated with severe or long-term impairment of renal function.     

A case of primary malignant lymphoma of the brain associated with acute hydrocephalus

A case of primary cerebral malignant lymphoma associated with hydrocephalus is reported. The patient was a 54 year-old male who enjoyed good health until the onset of headache and vomiting 4 weeks before admission. His consciousness was alert and neurological examination revealed severe papilloedema with retinal hemorrhage. No lymph node or abdominal tumor enlargement were noted. CT scan and MR images revealed no abnormal lesion except mild ventriculomegaly. CSF study revealed mild elevation of protein and sugar and cell count was 66/3. CSF cytology revealed atypical lymphoid cell with irregular nuclear contour and large nucleolus. Immunological marker studies of the tumor cell revealed increasing of anti J-5 (CD10), anti B-4 (CD19) and OKT-IA1. The patient was treated by a whole brain irradiation and chemotherapy after V-P shunt. It is 12 months since the operation, and the patient's condition is still good.