Hodgkin's disease and non-Hodgkin's lymphoma in children and young adults: A clinicopathologic study of 127 cases

One hundred twenty-seven cases of non-Hodgkin's lymphoma and Hodgkin's disease in children and young adults at the University of Wisconsin Hospital between 1969 and 1980 have been reviewed. Nodular sclerosing was the most frequent histologic type in patients with Hodgkin's disease. Malignant lymphoblastic lymphoma (MLLB) was the most common type of non-Hodgkin's lymphoma. The relationship of the histological pattern to age and sex as well as clinical behavior and survival are discussed.     

Combined therapy in children and adolescents with classical Hodgkin's lymphoma: A report from the SFCE on MDH-03 national guidelines

Hodgkin's lymphoma (HL) in children and adolescents is highly curable, but children are at risk of long-term toxicity. The MDH-03 guidelines were established in order to decrease the burden of treatment in good-responder patients, and this report should be considered a step toward further optimization of treatment within large collaborative trials. We report the therapy and long-term outcomes of 417 children and adolescents treated according to the national guidelines, which were applied between 2003 and 2007 in France. The patients were stratified into three groups according to disease extension. Chemotherapy consisted of four cycles of VBVP (vinblastine, bleomycin, VP16, prednisone) in localized stages (G1/95 pts/23%), four cycles of COPP/ABV (cyclophosphamide, vincristine, procarbazine, prednisone, adriamycin, bleomycin, vinblastine) cycles in intermediate stages (G2/184 pts/44%) and three cycles of OPPA (vincristine, procarbazine, prednisone, adriamycin) plus three cycles of COPP in advanced stages (G3/138 pts/33%). Radiation therapy of the involved field was given to 97% of the patients, with the dose limited to 20 Gy in good responders (88%). With a median follow-up of 6.6 years, the 5-year event-free survival (EFS) and overall survival (OS) were 86.7% (83.1–89.7%) and 97% (94.5–98.1%), respectively. EFS and OS for G1, G2, and G3 were 98% and 100%, 81% and 97%, and 87% and 95%, respectively. Low-risk patients treated without alkylating agents and anthracycline had excellent outcomes and a low expected incidence of late effects. Intensification with a third OPPA cycle in high-risk group patients, including stage IV patients, allowed for very good outcomes, without increased toxicity.     

Secondary malignancies in a child with Hodgkin's disease: Peripheral T-cell lymphoma and myelodysplastic syndrome evolving into acute nonlymphoblastic leukaemia

Hodgkin's disease (HD) has been linked to an increased risk of second malignant neoplasms (SMN), especially non-Hodgkin's lymphoma (NHL) and acute nonlymphoblastic leukaemia (ANLL). The mutagenic property of cytotoxic therapy as well as defective immunity have been implicated as playing a major role in the development of SMN in patients previously treated for HD. We report a case of a 14-year-old girl with HD who developed two different second malignancies within a latent period of 28 months following HD diagnosis. The patient presented initially with bilateral cervical and supraclavicular as well as mediastinal and paraaortic lymphadenopathy. She was staged as IIIA, nodular sclerosing type HD, and was given eight alternative cycles of MOPP-ABVD followed by “mantle” field radiotherapy to a total dose of 3.3 Gy plus 0.4 Gy to the upper mediastinum. Within 8 months following the completion of therapy, a period of myelodysplasia and progressive severe immune deficiency, considered as a result of initial treatment, occurred. Eighteen months after HD diagnosis while the patient was continuously neutropenic and heavily immunocompromised, a peripheral T-cell lymphoma of the angiocentric immunoproliferative lesion type (AIL) Grade III, appeared in both lungs within and beyond the radiation field, with no evidence of HD in biopsy specimens. After institution of a new chemotherapy regimen. (L17M), a satisfactory response regarding NHL lesions was noted. However, 10 months later the myelodysplastic syndrome (MDS) accompanied by complex chromosomal abnormalities evoluted to frank ANLL with a rapid fatal course. This case supports the hypothesis that combined modality treatment accompanied by severe immunodeficiency may result in the development of multiple second malignancies even within a very short latent period, especially in a subgroup of HD patients who may be at particularly increased risk for second cancers. © 1996 Wiley-Liss, Inc.     

Treatment of Hodgkin's disease in children with alternating mechlorethamine,vincristine, procarbazine,and prednisone (MOPP) and adriamycin,bleomycin, vinblastine,and dacarbazine (ABVD) courses without radiotherapy

Since the introduction of mechlorethamine, vincristine, procarbazine, and prednisone (MOPP) chemotherapy, children with Hodgkin's disease (HD) have been treated with chemotherapy alone. The occurrence of side effects related to irradiation (especially secondary solid tumors) is less likely to occur. Alkylating agents in the MOPP chemotherapy combinations are, however, known for their late effects, i.e., gonadal dysfunction and secondary malignancies. Combination with non-alkylating and non-cross-resistant drugs (as in the adriamycin, bleomycin, vinblastine, and dacarbazine [ABVD] combination) may give superior treatment results and possibly a decrease in the occurrence of side effects. From 1988 to 1993 all children presenting with HD were treated with alternating MOPP and ABVD courses (3 × MOPP, 3× ABVD). Twenty-one children (7 females, 14 males), ages 5–18 years (median 14 years) were included; their clinical stages were 1, 7 patients; II, 8 patients; III, 5 patients; IV, 1 patient. Their pathology revealed 2 lymphocytic predominance, 17 nodular sclerosis, 1 mixed cellularity. In 1 patient only cytology was done and thus histopathologic subclassification was not possible. Two children have relapsed; disease-free survival is 90%. Analysis of toxicity revealed no decrease in cardiac function by ultrasound examination and no pulmonary effects noted by carbon monoxide diffusion. In 1 of the 10 children tested, mild hypogonadism was noted. No secondary tumors occurred. From this small population of children with HD we conclude that treatment with MOPP/ABVD for 6 cycles without radiotherapy may be adequate. The occurrence of gonadal dysfunction may be less frequent than with 6 cycles of MOPP. However, more patients and further follow-up are needed. Med. Pediatr. Oncol. 29:23–27, 1997. © 1997 Wiley-Liss, Inc.     

Correlation of ribonucleic acid polymerase chain reaction, acid dissociated p24 antigen, and neopterin with progression of disease: A retrospective, longitudinal study of vertically acquired human immunodeficiency virus type 1 infection in children

Objective: We investigated the relationship between cell-free viral load, neopterin, age-adjusted CD4 ⁺ cell concentration, and clinical events in 49 children with vertically acquired human immunodeficiency virus type 1 infection.Study design: Viral load was measured by quantitating viral ribonucleic acid in serum by polymerase chain reaction and measurement of immune complex dissociated p24 antigen in serum and plasma. Children were followed for an average of 2 ½ years, with an average of 6 samples per child. Medical records were reviewed for weight, CD4 ⁺ cell count, and clinical events.Results: High virus copy number in serum was predictive of a decrease in weight-for-agezscore during the subsequent 6 months. High viral load, low CD4 ⁺ cell count, and high neopterin level were correlated with encephalopathy. High viral load correlated with opportunistic infections. All of these relationships held regardless of treatment status, although viral load decreased significantly after treatment was begun.Conclusions: Measurements of viral load were useful prognostic indicators for poor weight gain. Elevated serum virus levels and neopterin values and low CD4 ⁺ cell counts were all associated with encephalopathy. (J Pediatr 1997:130:898-905)