Cytoreductive procedures in the early management in cases of leukemia and hyperleukocytosis in children.

Cytoreduction for hyperleukocytosis before the initiation of primary therapy may reduce morbidity and mortality from blast cell lysis in children with acute lymphoblastic leukemia (ALL) and from leukostasis in children with acute nonlymphoblastic leukemia (ANLL) or chronic myelogenous leukemia (CML). The clinical features of 35 children (23 with ALL, 5 with ANLL, and 7 with CML) who underwent cytoreduction before the institution of definitive therapy were studied. Twelve children had exchange transfusions and 23 underwent leukaphereses. The cytoreductive procedures were equally effective in removing peripheral leukocytes (median decrease, 60%) and produced no complications. Ten children required additional cytoreduction because of further leukocyte increase before chemotherapy became effective. Three children with ALL who had renal insufficiency and metabolic derangement prior to leukapheresis subsequently required additional therapeutic measures. Three children with respiratory symptoms attributable to leukostasis improved after cytoreduction, and there were no episodes of intracerebral hemorrhage. These observations demonstrate the safety and efficiency of exchange transfusion and leukapheresis, and provide support for the role of cytoreduction in the early management of cases of hyperleukostasis and leukemia in children.

Mediastinal nonlymphoblastic lymphomas in children: a clinicopathologic study.

The records of 25 pediatric patients with mediastinal nonlymphoblastic lymphoma (NLBL) were reviewed. These patients comprise approximately 5% of all patients with non-Hodgkin's lymphoma (NHL) in the pediatric age group. There were 15 females and ten males. The median age was 13.5 years (range, 2 to 19). Most patients presented with symptoms attributable to a large mediastinal mass, and superior vena cava syndrome was a common feature. Disease was localized to the supradiaphragmatic area in 17 patients (71%) at diagnosis. Pathologic review revealed 22 of these lymphomas to be diffuse histiocytic type in the Rappaport classification, and 20 were large-cell immunoblastic type in the Working Formulation. Treatment regimens were not uniform, but included multiagent chemotherapy in 23 patients and radiation to the mediastinum in 20 patients. Twenty-three patients (92%) attained a complete remission (CR). Of these, 17 (74%) remain disease-free 13 to 65 months from diagnosis (median, 43 months). No CNS relapses have been observed. Mediastinal NLBL in the pediatric age group has distinctive clinicopathologic features that warrant special consideration in the design of treatment protocols.

Ossifying renal tumor of infancy.

Ossifying renal tumor of infancy is a rare lesion, with only 2 cases reported in the literature. We report an additional case, and review its presentation and histological findings.

Chronic obstructive airways disease after bone marrow transplantation.

The clinical course, serial pulmonary function studies, lung histopathologic findings, and treatment in two patients after bone marrow transplantation for acute monoblastic leukemia or aplastic anemia are presented. The course in one patient has been slowly progressive for 2 years and characterized by chronic obstructive airways disease and recurrent pneumothoraces. Histopathologic changes were nonspecific, characterized by chronic interstitial pneumonitis and interstitial fibrosis. In the second patient there was insidious onset of disease with increasing dyspnea on exertion and rapid clinical deterioration; he died within 4 months of severe obstructive airways disease. Necrotizing bronchitis and bronchiolitis characterized the lung findings. Neither patient responded to conventional bronchodilator therapy, and prednisone was the only agent to produce subjective, though transient, improvement. Symptomatic obstructive airways disease associated with chronic graft-versus-host disease is emerging as a potentially major cause of morbidity and mortality after marrow transplantation.

Presence of intracytoplasmic IgG in the lymphocytic infiltrates of the minor salivary glands of patients with primary Sjögren's syndrome.

The nature of the minor salivary gland B cell infiltrates in 13 patients with primary Sjögren's syndrome was studied utilizing an immunoperoxidase staining technique. A group of 6 patients without primary Sjögren's syndrome undergoing lip biopsy for evaluation of dry mouth served as controls. The patients with primary Sjögren's syndrome, in contrast to the control group, were found to have a large percentage of cells containing intracytoplasmic IgG. This finding points to the minor salivary gland as a site of localization of the activated B cell in patients with primary Sjögren's syndrome.

CT demonstration of fat tissue in malignant renal neoplasms: atypical Wilms' tumors.

It has been implied that the recognition of fat in a renal tumor suggests that the lesion is benign. Several authors have suggested tht angiomyolipoma is the only common renal tumor containing mature adipose tissue. Other neoplasms may contain mature fat that can be identified with current diagnostic imaging techniques. Under discussion is our experience with Wilms' tumor containing a predominance of adipose tissue.

Resolution of longstanding protein-losing enteropathy in a patient with intestinal lymphangiectasia after treatment for malignant lymphoma.

In 1956 we evaluated a patient who had a debilitating disease of a 2 yr duration, characterized by recurrent vomiting, diarrhea, cachexia, massive edema, hypoproteinemia, and dilated intestinal lymphatics. During our initial evaluation of this patient, we observed that 42% of her circulating protein pool was lost into her gastrointestinal tract daily, whereas normal gastrointesinal loss of protein does not exceed 1.6%. Her disease appeared to represent a classic example of intestinal lymphangiectasia. She was treated symptomatically for 13 yr with essentially no change. In 1969 the patient developed a stage IV diffuse, undifferentiated (non-Burkitt's) malignant lymphoma. Using immunoperoxidase staining, the neoplastic cells were found to contain cytoplasmic IgMKappa, suggesting that the lymphoma had a monoclonal B-cell origin. She was successfully treated with cyclophosphamide, vincristine, and prednisone. Shortly after the initiation of this systemic combination chemotherapy, her serum protein concentration returned to normal, her edema resolved, and she was cured of gastrointestinal symptoms. Moreover, repeat studies revealed that her protein loss had fallen to only 2%. The simultaneous cure of both the intestinal lymphangiectasia and lymphoma with combination chemotherapy suggests new relationships between these conditions as well as new possibilities for the treatment of acquired forms of intestinal lymphangiectasis associated with overwhelming gastrointestinal protein loss.

Diffuse large cell lymphomas (reticulum cell sarcomas, histiocytic lymphomas). Correlation of morphologic features with functional markers.

Electron microscopic findings in 15 cases of diffuse large cell lymphoma were correlated with other morphologic features, surface immunotype and cytoplasmic immunoglobulin content. Immunologically, the cases were: B cell, 8; null, 4; T cell, 2; and H cell (true histiocytic), 1. Ultrastructurally, all B cell and three null lymphomas were characterized by an abundance of polyribosomes and segments of rough endoplasmic reticulum. Concentric rough endoplasmic reticulum was observed in 4 cases of B cell lymphoma containing cytoplasmic immunoglobulin and in a null lymphoma. In 1 case of B cell lymphoma, the diastase-sensitive, periodic-acid-Schiff-positive cytoplasm showed evidence of widely dispersed monoparticulate glycogen granules. The two T cell lymphomas contained hyperlobulated or single round nuclei, and abundant smooth to rough endoplasmic reticulum. One null lymphoma appeared to share the ultrastructural features of T cell convoluted nuclei and the cytoplasmic organelles of myeloid precursor cells. The H cell lymphoma had features of monocytic-macrophagic differentiation. The large cell lymphomas, a morphologically and functionally heterogeneous group, were represented predominantly in this series by neoplasms with follicular center cells or early plasma cells.

Hodgkin's disease occurring in a patient with the Wiskott-Aldrich syndrome.

Hodgkin's disease, nodular sclerosing, developed in a 16-year-old man with the Wiskott-Aldrich syndrome. Two brothers and two nephews had documented Wiskott-Aldrich syndrome and had died of infectious complications in childhood. While the patient reported here had lifelong thrombocytopenia and recurrent upper respiratory infections, he had no severe infection prior to the development of Hodgkin's disease. Skin test sensitization with dinitrochlorobenzene was unsuccessful. No antibodies were found after immunization with pneumococcal polysaccharides. Platelet aggregation studies were abnormal in the patient, his mother, and one of his nephews. A complete response of short duration occurred after treatment with nitrogen mustard, vincristine, procarbazine, and prednisone. On recurrence, he proved unresponsive to further chemotherapy or radiation therapy. Infection with four different fungi was found at autopsy. This patient is the third recorded case of Hodgkin's disease associated with the Wiskott-Aldrich syndrome.