Malignant histiocytosis X: a distinct clinicopathologic entity

Article Abstract:

Langerhans cells are specialized cells that participate in the initiation of an immune response. These and similar cells are also found in other epithelia within the body. The pathological proliferation of Langerhans cells is called Langerhans cell histiocytosis. This condition, most often benign, is also called histiocytosis X. Langerhans cells contain a characteristic structure in their cytoplasm, the Birbeck granule. These granules are sometimes called X-bodies, hence the name "histiocytosis X". (The condition should not be confused with histiocytosis, which involves cell types other than Langerhans cells.) Generally, histiocytosis X follows a chronic, indolent course. Some pathologists are unsure if the condition can ever be truly malignant. In contrast to classic histiocytosis, which affects men and women with equal frequency, this disease affects males versus females six to one. The authors present data from 31 cases of histiocytosis X; among them are cases which support the existence of malignant histiocytosis X as a distinct disease. In 19 cases, the disease followed an indolent course, and examination of the involved tissues revealed cells with a benign appearance. The lymph nodes were the most commonly involved tissue, followed by bone. Skin, liver, and lung were also involved in some of these patients. In three patients of the 31 patients, the microscopic appearance of the cells was benign, but the disease followed an aggressive course, resulting in death between one and seven months. Conversely, two other patients had a benign course of disease, despite malignant appearance of cells in the involved tissues. The one patient for whom long-term follow-up is available has survived 18 years. Seven patients were seen in which the malignant appearance of the cells was matched by a malignant disease course. These patients all died after periods ranging from eight days to two years. The authors suggest that this last group of patients represents a distinct syndrome, malignant histiocytosis X. The observations made in this study also indicate that the pathological appearance of cells are imperfect indicators of the prognosis of histiocytosis X. (Consumer Summary produced by Reliance Medical Information, Inc.)

Physiological aspects, Development and progression, Prognosis, Reticuloendotheliosis, Langerhans cells

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Lymphomas in University College Hospital, Ibadan, Nigeria

Article Abstract:

Burkitt's lymphoma is a rare and sporadic form of lymphoma in the United States. However, in parts of the world where malaria is endemic, Burkitt's lymphoma is common. Indeed, in Nigeria, Burkitt's lymphoma is the most common childhood cancer. The authors provide a review of lymphoma cases seen at a major university hospital in Ibadan, Nigeria. A total of 508 cases of lymphoma were seen between 1974 and 1989. The single most common type was Burkitt's lymphoma, which accounted for 211 cases; 95 percent of these cases involved children. Although Burkitt's lymphoma is a high-grade tumor, it is also particularly sensitive to chemotherapy, and fortunately, enjoys a better prognosis than other high-grade lymphomas. Eighty-five other high-grade lymphomas were seen, as were 117 low-grade lymphomas. Ninety-five of the 508 cases were Hodgkin's disease; 32 percent of these involved children. Lymphomas were more common among males than females; this preponderance of males was most striking for Hodgkin's disease. Among the patients with Hodgkin's disease, males outnumbered females by 4.6 to 1. If only the children are considered, the preponderance of males is even higher. The peak incidence in Hodgkin's disease was found to be between the ages of 11 and 25 years. In the United States, Hodgkin's disease actually has two peaks of incidence, an early one similar to the peak in Nigeria and a later peak, which occurs after 50 years of age. The second peak was not observed in this study. This probably does not represent any intrinsic difference in Hodgkin's disease between the two nations, however, but rather a difference in demographics. In the US, more than 30 percent of the population is over 45 years of age. By a 1986 population estimate, only 9 percent of the Nigerian population is older than 45 years. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Hodgkin's disease, Demographic aspects, Nigeria, Nigerians, Burkitt's lymphoma, Burkitt lymphoma, Ibadan, Nigeria

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A clinicopathologic study of malignant lymphomas of the nose, paranasal sinuses, and hard palate, including cases of lethal midline granuloma

Article Abstract:

Lethal midline granuloma is a mixed bag of diseases with common symptoms, that includes the slowly progressive ulceration of the nose and paranasal sinuses and often the destruction of soft tissues, cartilage, and bone. The syndrome may result from malignant lymphoma, infection, collagen vascular disease, and disease of uncertain cause, such as Wegener's granulomatosis and idiopathic midline destructive disease. To help tease apart the complicated factors which play a role in the differential diagnosis of lethal midline granuloma, 20 cases of malignant lymphoma of the nose, paranasal sinuses, and hard palate were observed for distinctive histopathological features. Eleven of the 20 were lymphomas of the large cell type, and 13 of 20 had features suggesting T cell lymphoma. Histological material was available for immunocytochemical staining in only 10 cases; 8 of these were definitively T cell phenotypes. Unfortunately, it is difficult to distinguish such tumors from inflammatory cells due to other causes of lethal midline granuloma. The presence of inflammatory cells and necrosis may mask the presence of cancerous cells. Biopsies must be sufficiently large that a representative area is sampled. The T cell phenotype is of diagnostic value in most, though not all cases, as is the so-called angiocentric lymphoma. The angiocentric growth pattern is a circular region of lymphoma growth surrounding a blood vessel; these infiltrates often destroy the blood vessel and may thereby cause a wider area of necrosis. The differential diagnosis of lymphoma from other possible causes of lethal midline granuloma is especially important since the lymphoma may well be radiosensitive, whereas some other causes, such as Wegener's granulomatosis, are more responsive to chemotherapy and steroids. (Consumer Summary produced by Reliance Medical Information, Inc.)

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