Eosinophilic granuloma bones

Eosinophilic granuloma bones (synonym Taratynova disease) is a rare disease characterized by the formation in bone marrow granuloma, rich eosinophilic white blood cells. Eosinophilic granuloma bones occurs mostly in children of preschool age, less often in adolescence. Etiology and pathogenesis still remain unclear.
When eosinophilic the granuloma bones observed the proliferation of endothelial tissue and violation of intracellular enzyme processes. Microscopic examination of eosinophilic the granuloma bones define a limited granulomatose-osteolytic process, expressed hyperplasia reticular cells, clusters eosinophilic white blood cells, sometimes large foam cells containing significant amounts of cholesterol, neutral fats, phosphatides.
Core topic eosinophilic granuloma bones - single or multiple isolated pockets in the flat and tubular bones. Most often affects the bones of the cranial vault, ribs, femur, the bone pelvis, vertebrae.
The clinical picture. The initial symptoms are pain and swelling in the area of the lesion. On the skull swelling becomes soft edge of the bone defect palpable as crateriform thickening. With the defeat of the long bones is determined club-shaped thickening. The skin above them is usually unchanged.
In the initial period of the disease on radiographs visible defects of bones round or oval. In the future, merging, the centers acquire polycyclic shape and form a honeycomb pattern.
General condition of the patient satisfactory. Sometimes children complain of pain, aggravated by movement of a headache with the defeat of the skull bones.
The disease develops slowly, has non-malignant chronic. For large lesions possible pathological fractures and education false joints.
The diagnosis is made on the basis of a pain in the area of bone tumor, like formation, the x-ray pictures and microscopic examination of material obtained by puncture, biopsy or surgery.
Differential diagnosis of eosinophilic granuloma bones is true tumors (osteogenic sarcoma, Ewing sarcoma, and others), tuberculosis of bones and joints, osteomyelitis, bone cysts.
Treatment of eosinophilic granuloma bone surgery and radiotherapy.
The prognosis is usually favorable and with appropriate treatment begins recovery. In rare cases, the disease progresses.

Eosinophilic granuloma bones in children is a relatively rare disease. She has a number of features and is currently a separate nosological unit.
Mostly affects children of school age and young men. The disease is flowing benign, which is confirmed by the description of the cases of spontaneous recovery. The earlier trauma occurs, eosinophilic granuloma bones is not important. Affecting both flat and tubular bones; from the flat bones of the most frequently - skull bones, and of tubular - femoral and humeral with the localization process in their proximal. Distal extremities (arms, legs) are affected very rarely. Eosinophilic granuloma bone may be singular and plural.
The clinical picture. The first symptom is usually mild soreness in the area of bone lesion. If the process is localized in the lower limbs, the patient starts to limp. At the same time or later determined thickening bone at the site of the lesion with moderate swelling of soft tissues without inflammatory changes; in some cases there is atrophy of muscles. The tumor skull has a mild consistency; Sometimes on the site of the tumor can determine crater edge of the bone defect. Marked malaise, decreased appetite, and a slight increase in body temperature, however, these symptoms is variable. The disease is chronic, for months. Sometimes when General good condition lesions in bone found by chance or after the occurrence of a pathological fracture. In blood may be moderate leukocytosis (15 000-17 000 cells per 1 mm3 the blood), eozinofilia (to 10-15%), but quite often the picture of blood normal. Biochemical analysis of blood and urine analysis does not show deviations from the norm.
The diagnosis. Poverty anamnestic data, unclear clinical symptoms and diversity of the x-ray pictures are always difficult diagnosis of eosinophilic granuloma bones. Therefore, there are cases when you set the erroneous diagnosis of myeloma with a predominance of eosinophilic cells, osteomyelitis with eosinophilic reaction, bone cysts, sarcoma, illness Hand-shyullera-Christian, tuberculosis, syphilis and other Diagnosis to be confirmed only by histological examination of material obtained by scraping the affected hearth or by aspiration.
Treatment. Usually apply surgery or x-ray treatments. When isolated foci eosinophilic granuloma bone ends recovery after scraping granuloma from the affected part of the bone. Bone defect in the majority of cases is filled in a few months, the full restoration of bones occurs in the course of a year or more. Some authors after scraping large lesions eosinophilic granuloma produce transplantation of healthy bone a defect or fill the cavity of the bone autostroitel or freeze-dried samostroykas (M. Century Volkov). When multiple tumors, eosinophilic granuloma bone shown radiotherapy, which gives excellent results (C. A. Reinberg). Described cases of spontaneous cure of this disease.
Forecast eosinophilic granuloma bones favorable.