Combined chemotherapy and tracheobronchial stenting for life-threatening airway obstruction in a child with endobronchial non-Hodgkin lymphoma

Endobronchial involvement in non-Hodgkin lymphoma is rare even in the presence of advanced disease. A 15-year-old boy presented with progressively worsening dyspnea with occasional hemoptysis for 1 week prior to admission. Three days later, he was intubated due severe dyspnea with complete atelectasis of the right lung. Fiberoptic bronchoscopy disclosed an endobronchial mass almost occupying the right main bronchus. He underwent partial resection of the endobronchial tumor with rigid bronchoscopy. An airway stenting was used in this patient because he had severe tracheal obstruction from the tumor. The compromised airway was alleviated by combined chemotherapy and tracheobronchial stenting.     

MEGADOSE METHYLPREDNISOLONE FOR KASABACH-MERRITT SYNDROME

We report two girls with primary erythrocytosis in whom extensive diagnostic studies revealed no underlying cause. Normal growth of colonies derived from erythroid burst forming units (BFU-E) was observed, and serum erythropoietin concentrations were within or below the normal range. The absence of a rise in serum erythropoietin levels after isovolemic phlebotomy implicated the erythroid marrow as the site of the pathophysiologic abnormality in both patients. Spontaneous resolution of erythrocytosis occurred during the second decade of life. Our experience suggests that primary erythrocytosis may be self-limited in some children. In these cases, the proliferative abnormality may be sufficiently subtle as to not be detected by standard in vitro culture systems, which support the growth of colonies derived from erythroid progenitors.     

TREATMENT OF KASABACH-MERRITT SYNDROME BY MEGADOSE METHYLPREDNISOLONE

The authors have previously demonstrated a favorable effect of high-dose methylprednisolone (HDMP), which can induce differentiation and apoptosis of leukemic cells in children with acute myeloblastic leukemia (AML). Here, they evaluate the effect of short-course HDMP in 2 children with acute myeloblastic leukemia (AML-M2) presented with myeloid tumor (MT). Methylprednisolone (20 or 30 mg/kg/day) was given orally, in a single dose, without using other antileukemic agents. Rapid cytoreduction in MT, peripheral blood, and bone marrow blasts was observed in both children following short-course (4 or 7 days) HDMP treatment, possibly due to HDMP-induced differentiation and apoptosis of leukemic cells. The effects of HDMP should be explored in patients with other subtypes of AML who present with MT.     

PULMONARY HYPERTENSION IN CHILDREN WITH EVANS SYNDROME

Evans syndrome is a rare cause of hemolysis in pediatric patients. The authors describe two severely affected patients who had previously been heavily treated, and who subsequently developed severe pulmonary hypertension. Both patients were successfully managed by a combination of immunosuppression and anti-pulmonary hypertension treatment. The first patient to present, case A, received an allogeneic bone marrow transplant with subsequent cure of both Evans syndrome and pulmonary hypertension and is now on a weaning dose of sildenafil. Case B is being worked up for allogeneic bone marrow transplantation. The authors speculate that the pulmonary hypertension was caused by the underlying immune dysregulation and hemolysis and that Evans syndrome joins the list of other hemolytic anemias that cause pulmonary hypertension, such as sickle cell disease, thalassemia, and paroxysmal nocturnal hemoglobinuria. However, they suggest a vasculitic process as the main cause.     

OBSTRUCTIVE JAUNDICE: An Unusual Initial Manifestation of Intra-Abdominal Non-Hodgkin Lymphoma in a Child

Obstructive jaundice is an unusual manifestation of non-Hodgkin lymphomas in children. Although surgical drainage is one of the initial treatment choices in some cases, usually lymphomatous masses rapidly response to chemotherapy and jaundice decreases due to regression of the mass, without any surgical procedure. The authors report the case of a 16-year-old girl who presented with biliary obstruction due to a neoplasm involving the duodenum. Histological examination of the specimen, which was taken from the mass by endoscopic biopsy, revealed Burkitt lymphoma infiltrating the duodenum. Chemotherapy including cyclophosphamide was started immediately. In a few days, jaundice decreased rapidly by the shrinkage of the mass. Neither surgery nor percutaneous drainage were needed. In conclusion, biliary tract obstruction due to non-Hodgkin lymphoma can be effectively treated with chemotherapy alone without any surgical procedure.     

Obstructive jaundice: an unusual initial manifestation of intra-abdominal non-Hodgkin lymphoma in a child

Obstructive jaundice is an unusual manifestation of non-Hodgkin lymphomas in children. Although surgical drainage is one of the initial treatment choices in some cases, usually lymphomatous masses rapidly response to chemotherapy and jaundice decreases due to regression of the mass, without any surgical procedure. The authors report the case of a 16-year-old girl who presented with biliary obstruction due to a neoplasm involving the duodenum. Histological examination of the specimen, which was taken from the mass by endoscopic biopsy, revealed Burkitt lymphoma infiltrating the duodenum. Chemotherapy including cyclophosphamide was started immediately. In a few days, jaundice decreased rapidly by the shrinkage of the mass. Neither surgery nor percutaneous drainage were needed. In conclusion, biliary tract obstruction due to non-Hodgkin lymphoma can be effectively treated with chemotherapy alone without any surgical procedure.     

Clitoromegaly and abdominal tumour as leading signs of a mediastinal non-Hodgkin lymphoma

A 2-year-old girl was admitted with clitoromegaly, mediastinal tumour and enlarged kidneys. A seventh cranial nerve palsy was associated with an increased cell count in cerebrospinal fluid. The diagnosis of a lymphoblastic lymphoma was confirmed by a cervical lymph-node biopsy.Six years after diagnosis and treatment according to the BFM protocol the girl remains in first complete remission.     

CLINICAL COURSE OF CHILDREN WITH IMMUNE THROMBOCYTOPENIC PURPURA TREATED WITH INTRAVENOUS IMMUNOGLOBULIN G OR MEGADOSE METHYLPREDNISOLONE OR OBSERVED WITHOUT THERAPY

The authors compared the prognosis in 50 children with acute immune thrombocytopenic purpura (ITP) who received intravenous immunoglobulin G (IVIG), megadose methylprednisolone (MDMP), or no therapy. Twenty-six children were observed with no therapy, 12 children received IVIG, and 12 children received MDMP. The percentage of the patients whose platelet counts increased at a level of > 20 &#50 10 9 /L and > 50 &#50 10 9 /L at 3 days after starting therapy was significantly higher in both IVIG and MDMP groups than in the no therapy group ( p < .01), but there was no significant difference at 10 and 30 days after initiation between the 3 groups ( p > .05 in each comparison). This result suggested that therapy does not increase the rate of recovery but shortens the duration of thrombocytopenia in the first days. Management decision in ITP is made on clinical condition rather than on platelet count and no treatment options is to be preferred even in the face of mucosal bleeding. If the patient has extensive bleeding and the decision is to treat, both IVIG and MDMP are equally effective in providing a safe platelet level early on.     

Dural sinus thrombosis in children with cancer

Dural sinus thrombosis (DST) has been reported in association with cancer in both adults and children. We describe the seven patients seen with this complication in our centre between 1981 and 1995. Diagnosis was confirmed by either cerebral CT scanning, MRI or angiography. Median age was 13 years (range 8–15). Six patients were boys. Six children were being treated for non-Hodgkin lymphoma and one for neuroblastoma. Presenting symptoms were seizures and transient neurologic deficit, often preceded by headaches. The probable cause of DST was found in two cases. Tumour localisation in the central nervous system (CNS) probably caused DST in one patient who was treated for Ki 1 lymphoma. Dehydration in combination with a poor general condition seemed to be the cause of DST in the patient with neuroblastoma. In five children with stage III or IV non-Hodgkin lymphoma (three lymphoblastic lymphoma; two Burkitt's lymphoma), etiology remained unknown. In these children, DST occurred early in the course of therapy. The median interval between start of chemotherapy and onset of symptoms was 19 days (range 8–40). No child had received L-asparaginase. Prognosis was favourable, with symptoms completely disappearing without therapy within 1 to 5 days. The incidence of DST in patients with advanced stage non-Hodgkin lymphoma during induction and consolidation was calculated to be below 3%. We conclude that DST is rarely diagnosed in children with cancer. Occurrence during the initial phase of therapy for non-Hodgkin lymphoma is associated with a benign prognosis. Med. Pediatr. Oncol. 29:296–302, 1997. © 1997 Wiley-Liss, Inc.     

The role of local radiation in the treatment of non-hodgkin lymphoma in children

Eighty-one patients between 1 and 15 years of age with non-Hodgkin lymphoma were seen at Memorial Sloan-Kettering Cancer Center (MSKCC) in an 8-year period ending December 1973. There was no statistically significant difference among the survival distributions for site or histologic type. The patients also were divided into three groups according to the chemotherapeutic regimen employed in their care, and further subdivided as to whether they received “curative” radiotherapeutic attempts. Children treated with multiple agent chemotherapeutic regimen (LSA-2) did significantly better than children of the LSA-1 or nonprotocol (NP) group. Children with Stage I and Stage II disease did significantly better than children with Stage III and Stage IV disease. Radiation therapy as employed in this study prevented recurrence of local disease. All patients in whom the disease recurred died within a year from the appearance of recurrence. However, no statistically significant association between radiation and survival could be shown in this series.     

Use of gemcitabine,oxaliplatin, and anti-CD20 therapy in children and adolescents with non-Hodgkin lymphoma unfit for intensive therapy

Multiagent immunochemotherapy affords excellent outcomes in pediatric non-Hodgkin lymphoma (NHL); however, scarce data exist for patients unfit for intensive treatment. Rituximab, gemcitabine, and oxaliplatin (R-GemOx) is well tolerated and efficacious in elderly adults with NHL; however, its use has not been described in pediatrics. In this retrospective, single-center study, six children with mature B-cell NHL and significant comorbidities received anti-CD20 therapy with GemOx (rituximab or obinutuzumab or ofatumumab with gemcitabine and oxaliplatin [R/O-GemOx]). R/O-GemOx was well tolerated and resulted in complete response in two of three patients with newly diagnosed NHL and one of three patients with primary refractory NHL. R/O-GemOx is a viable treatment option for children with NHL who cannot tolerate intensive therapy.     

Childhood non-Hodgkin malignant lymphomas: A clinicopathologic retrospective study

Between 1968 and 1975, 44 evaluable children under 16 years of age with the histologic diagnosis of non-Hodgkin malignant lymphoma (ML) were treated at the Istituto Nazionale Tumori of Milan. Histologic diagnoses were reclassified as follows: 13 lymphoblastic (others) ML, 15 convoluted cell type lymphoblastic ML, 9 Burkitt type ML, and 7 immunoblastic ML. Only 36% of the patients had stage I and II disease. At diagnosis 25% showed malignant cells in the bone marrow smears. Bone marrow infiltration was particularly frequent in the convoluted cell type lymphoblastic ML and in the lymphoblastic (others) ML subgroups. Burkitt type ML frequently was associated with abdominal lesions and subsequently a high incidence of central nervous system involvement. Patients with stage I and II ML were encountered mostly in the immunoblastic ML subgroup. After 1973 more intensive chemotherapy plus radiotherapy seems to have slightly improved the survival of the patients, except in the Burkitt type ML subgroup.     

Tumor lysis syndrome in children with non-Hodgkin lymphoma

This study was designed to evaluate the incidence of tumor lysis syndrome (TLS) among children with non-Hodgkin lymphoma and to define whether renal involvement can be associated with higher incidence of TLS after chemotherapy. Medical charts of 59 patients were reviewed. TLS was diagnosed using laboratory and clinical criteria. Renal involvement was diagnosed based on ultrasound and CT scan findings. Laboratory TLS occurred in 7 patients (11.85%) and clinical TLS was observed in 7 patients (11.85%) as well. In 5 out of 14 TLS patients, hemodialysis was required to correct electrolyte abnormalities and TLS related death was reported overall in 3 patients. Sex, age, pretreatment LDH, and initial WBC count were not associated with higher incidence of TLS after chemotherapy, but a significant correlation was found between pretreatment renal involvement at imaging studies and development of TLS after chemotherapy (p = .027). The results indicate that despite all preventive measures, tumor lysis syndrome still occurs in children with non-Hodgkin lymphoma following chemotherapy. Patients who have evidence of renal involvement at imaging studies are more likely to develop TLS after chemotherapy.     

CLINICAL CHARACTERISTICS AND TREATMENT RESULTS OF PEDIATRIC B-CELL NON-HODGKIN LYMPHOMA PATIENTS IN A SINGLE CENTER

The aim of this study was to evaluate and compare the clinical characteristics of the B-cell non-Hodgkin lymphoma (NHL) patients and therapeutic efficacy of modified NHL BFM-90 and NHL BFM-95 protocols in the authors’ center. From January 1993 to December 2003, 61 newly diagnosed children with B-NHL were enrolled to the study. The patients were stratified by risk factors and treated either with a modified B-NHL BFM-90 or BFM-95 protocols. The use of 1 or 3 g/m² of methotrexate instead of 5 g/m²/24 h was the only important modification in BFM-90 protocol. Sixty-one children (12 girls, 49 boys) with a median age of 6.5 years (range: 2.5–16) were treated in the center. There were 14 patients in stage II, 28 in stage III, and 19 in stage IV. The most common initial primary tumor sites were abdomen, head, and neck. Forty-five patients were treated with modified B-cell BFM-90 and 16 patients were treated with B-cell BFM-95 regimens. The 5-year overall survival (OS) for all patients was 85.8%, and event-free survival (EFS) was 82.8%. The 5-year OS rates in modified BFM-90 and in BFM-95 protocols were 85.2 and 87.5%; the 5-year EFS rates in these 2 protocols were 84.6 and 70%, respectively (p >.05). Factors associated with lower EFS by univariate analysis were bulky disease, risk groups, and LDH level ≥ 500 IU/L. By multivariate analysis only LDH level was significant. In conclusion, the treatment results in this study were similar to those of BFM group.     

Varicella Vaccine in Children with Acute Lymphoblastic Leukemia and Non-Hodgkin Lymphoma

To determine the safety and efficacy of varicella vaccine, 17 children with acute lymphoblastic leukemia (ALL) and 2 children with non-Hodgkin lymphoma (NHL) receiving chemotherapy in remission were immunized with live attenuated varicella vaccine (Oka strain). Rash occurred in 7 children 7-53 days (median 24 days) after vaccination. There were 10-80 (median 20) erythematous vesicles and low-grade fever. All children required no specific treatment. There was no spread of varicella to their susceptible siblings. The control group comprised 92 ALL and 25 NHL patients receiving chemotherapy in remission who were nonvaccinated and susceptible to varicella.

Over a risk duration of 80 person-years, one child of the vaccinated group developed varicella of mild degree, whereas in a risk duration of 120 person-years, 14 g the control group developed varicella. One patient died, though prompt antiviral therapy, especially acyclovir, was given to all of them. Herpes zoster of mild degree was observed in one child of the vaccinated group 65 days after vaccination. Two children of the control group deueloped disseminated herpes zoster. With acyclovir therapy, there was no mortalily. The incidence of varicella in the vaccinated group was less than that of the control group. The diffence is statistically significant (p =.0222), and the side effects of the vaccine were acceptable. Thus we conclude that the vaccine can be safely used in children with ALL or NHL under chemotherapy and can effectively protect such children from varicella.     

Discitis following lumbar puncture in non-Hodgkin lymphoma

Discitis in children is a rare disorder of intervertebral disc and vertebral end plate. Infection or trauma, like lumbar puncture, may be the possible causes. Low-back pain and gait disturbance are the main symptoms. The most appropriate diagnostic procedure is MRI. Treatment is mainly empirical. Here a case with non-Hodgkin lymphoma is discussed. Treatment consisted of strict bed rest and antibiotics. Safe and sterile technique is important in patients with invasive procedures like intrathecal chemotherapy. Although discitis is a self-healing condition, it might cause vertebral osteomyelitis. In this regard, physicians should be aware of this probable complication after lumbar puncture and manage it earlier in children with cancer.