Langerhans cell histiocytosis,☆☆,,★★

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CLASSIFICATION AND NOSOLOGY

The term histiocytosis identifies a group of disorders that have in common the proliferation of cells of the mononuclear phagocyte system and the dendritic cell system.14 The individual entities are diagnosed on the basis of certain symptoms, signs, and laboratory information that, when taken together, fulfill generally agreed-on criteria for the diagnosis of that particular disorder. The various histiocytoses of childhood are listed in Table I. Histiocytes (macrophages) and dendritic cells

SIGNS AND SYMPTOMS

Langerhans cell histiocytosis can occur in persons ranging in age from newborn to elderly; the peak incidence occurs between 1 and 4 years. Reliable data on the annual incidence are difficult to gather, but the frequency in the pediatric age range has been estimated at 2 to 5 per million per year.19 In most studies LCH occurs almost equally in male and female subjects, with a small preference for male subjects. The disease is probably underdiagnosed, the bone lesions being symptomless, painful

DIAGNOSTIC STEPS

The Writing Group of the Histiocytic Society, an international body, outlined in 1987 the morphologic, immunohistochemical, and clinical criteria required for the diagnosis of LCH and the other histiocytic disorders in children.2 The various entities were grouped as follows: LCH as class I, the histiocytoses of mononuclear phagocytes other than Langerhans cells as class II, and malignant histiocytic disorders as class III. The Histiocyte Society2 also published confidence levels for the

THERAPY

Treatment of patients with LCH depends on the extent of disease. The number of organs involved and the implications of that involvement are of governing importance. When patients are assigned to one of the two categories described above (Table III), the prognosis is excellent for those with restricted presentations. It is guarded to ominous for those with extensive LCH, especially for patients with organ dysfunction.

FOLLOW-UP AND SEQUELAE

The sequelae and late effects of treatment are dependent on the varied “natural history” of LCH itself and on the original signs and symptoms that dictate the intensity of treatment. Comparing morbidity among series has always been hampered by the lack of uniformly accepted schedules and methods of evaluation. This problem has largely been overcome by the standardized criteria that make accurate comparisons possible3 (, ).

Late sequelae of the disease and its treatment seem to be more frequent

PROGRESS IN BASIC RESEARCH

Although the cause is still unknown, investigative research in three major fields has advanced the understanding of LCH and has provided new insights to stimulate further biologic and clinical investigations of the disease.

SUMMARY AND CONCLUSIONS

The first major stride toward understanding LCH was taken when ultrastructural studies identified the proliferating cells as part of the Langerhans (dendritic) cell system.16, 17 Another step forward was the definition of the morphologic, immunohistochemical, and clinical criteria needed for the diagnosis of LCH.2, 3 Meanwhile, modern imaging studies have disclosed lesions that were not previously visible, especially those in the brain and the pituitary gland. These advantages have had a major

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    From the Department of Pediatric Hematology-Oncology, Sophia Children's Hospital, Erasmus University Rotterdam, Rotterdam, The Netherlands, and the Department of Radiation Oncology, University of Pennsylvania, Philadelphia

    ☆☆

    Reprint requests: R. Maarten Egeler, MD, PhD, Sophia Children's Hospital, Erasmus University Rotterdam, Pediatric Hematology-Oncology, Dr. Molewaterplein 60, 3015 GJ Rotterdam, The Netherlands.

    THE JOURNAL OF PEDIATRICS 1995;127:1-11

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    0022-3476/95/$3.00 + 0 9/18/64029

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