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"Child`s Health" 1 (52) 2014

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Lymphomatoid granulomatosis of the lungs and mediastinum in child of two years

Authors: S.V. Veselyy, I.P. Zhurilo, V.K. Litovka, K.V. Latyshov — Donetsk National Medical University named after М.Gorky; Regional Children’s Clinical Hospital, Donetsk

Categories: Pediatrics/Neonatology

Sections: Specialist manual

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Information about lymphomatoidgranulomatosis in children are in work. In the classification of the World Health Organization lymphomatoidgranulomatosis is included as a proliferative disease of mature B-cells with an uncertain malignant potential. This disease is quite rare. Lymphomatoidgranulomatosis is almost always by lesions of the lung accompanied. Other ekstragonad area (skin, brain, bone marrow, kidney, liver) are often affected. Lymphatic nodes are rarely detected. Infiltrate composed from small T-cells and different amounts of EBV-positive B-cells, they are sometimes polymorphic. Angiosentrical and angiodestruktion option of growth occurs frequently, it may be accompanied by the appearance of focus of infarction. Lymphomatoidgranulomatosis has degree of differentiation (Grade) are classified as follows. Grade I  at high magnification in the same field of view is less 5 EBV-positive B-cells, necrosis is determined rare. Grade II  at high magnification in the same field of view is the 5-20 EBV-positive large B-cells, necrosis is determined more often. Grade III  there are large number of EBV-positive large B-cells. They form in some areas the confluent layers, the different T-cells numbers, and frequent necrosis. B-cells are clonal cells when Grade II and Grade III. This subtype should be considered as a variant of diffuse B-largecells lymphoma. Congenital or acquired immunodeficit is usually determined in patient's lymphomatoidgranulomatosis. The pathogenesis of lymphoproliferative diseases (lymphogranulomathosis, leukemia, and lymphoma) is a violation of T-cell immunity against Epstein-Barr virus in most cases. This leads to B-cell proliferation and, eventually, tumor formation. Lymphomatoidgranulomatosismanifested with the release of mucous cough sputum, cyanosis, shortness of breath, chest pain, emaciation. Symmetrical bilateral disease of the lungs in posteriorbazal segments often detected during radiography. Rales in the lower lung fields are determined by auscultation. Diagnosis of lymphomatoidgranulomatosis determine significantly before the operation difficult. The diagnosis can verify after needle biopsy or surgery.

Only one child with lymphomatoidgranulomatosis was in the clinic of pediatric surgery in the last thirty years. Patient K., 2 years 3 months, was transferred to the first surgical department 15.07.2013 with a suspected tumor of the chest cavity from the right. The child suffers from a primary combined immunodeficit. The phenomenon of recurrent necrotizing stomatitis, candidiasis of the oral mucosa appeared with one year of age. The girl observed treated by a neurologist at the progressive disease of the central nervous system (encephalitis) with symptoms of tetraparesis since November 2012. Radiography of the chest was made 10.07.2013. The X-ray revealed tumor of the chest cavity from the right. 12.07.2013 the patient was hospitalized in intensive care, and then transferred to the surgical department.

The general condition of the child is heavy. Skin is clean, pale. Fever up to 38,5?C occurs intermittently. The patient holds his head badly, not sitting. There is paresis of the facial nerve on the right. There is tetraparesis, more pronounced on the right. Hard breathing listened in the lungs, to the right a few weakened. Heart sounds muted, rhythmic. The pathology is not detected by abdominal palpation. Urine output is normal. There is a tendency to constipation. The pathological changes in the liver, spleen, pancreas, kidneys during ultrasonography were not identified. During the ultrasound of the chest cavity in the subclavian area on the right, below the subclavian vein, cystic formation wrong round, size 36/43 mm, revealed. Helical computed tomography (12.07.2013): the structure of an oval-shaped, homogeneous, with clear margins, size 2,8/3,2/3 sm in the upper mediastinum on the right is rendered. Tumour proliferates in paramediastinum (on the level of Th2-Th5). Hearth seal 0,6/0,5 sm in the upper lobe of the lung found. In the root zone the structure of soft-tissue density with clear margins, size 1,3/1,2 sm, visualized, adjacent to the vessels. Conclusions: the inflammatory process may be neoplastic. The girl was examined at the regional tuberculosis clinic  the data in favor of tuberculosis have not been identified. Biochemical analyses of blood (bilirubin, transaminases, glucose, urea, creatinine, prothrombin, potassium, sodium, chloride) are in the normal range. In the analyses of blood: ESR  30 mm/h, the other indicators unremarkable. Concerning immunodeficit patient specific treatment received. Puncture biopsy to verify the diagnosis could not be held. The clinical diagnosis was determined inflammatory pseudotumor of the chest cavity (mediastinum and lung) on the right (Mezenhimoma?, Lymphoma?).

24.07.2013 the operation is performed: right thoracotomy, removal of the tumor of the upper lobe of right lung. Immunohistochemistry was received (06.08.2013): lymphomatoidgranulomatosis, Grade II. The postoperative period was uneventful. Sutures are removed, the wound healed by first intention. 10.08.2013 the child was discharged home. Further treatment in Minsk (Belarus) is planned.



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