Bone marrow fibrosis occurs in association with a number of pathological states. Despite the extensive fibrosis that sometimes characterizes renal osteodystrophy, little is known about the factors that contribute to marrow accumulation of fibrous tissue. Because circulating cytokines are elevated in uremia, possibly in response to elevated parathyroid hormone levels, we have examined bone biopsies from 21 patients with end-stage renal disease and secondary hyperparathyroidism. Bone sections were stained with antibodies to human interleukin1a (IL-1a), IL-6, IL-11, tumor necrosis factor-a (TNF-a) and transforming growth factor-ß (TGF-ß) using an undecalcified plastic embedding method. Intense staining for IL-1a, IL-6, TNF-a and TGF-ß was evident within the fibrotic tissue of the bone marrow while minimal IL-11 was detected. The extent of cytokine deposition corresponded to the severity of fibrosis, suggesting their possible involvement in the local regulation of the fibrotic response. Because immunoreactive TGF-ß and IL-6 were also detected in osteoblasts and osteocytes, we conclude that selective cytokine accumulation may have a role in modulating bone and marrow cell function in parathyroid-mediated uremic bone disease.

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The immunohistological staining with polyclonal antibodies of bone biopsies from patients with osteitis fibrosa due to secondary hyperparathyroidis111 was tested in methyl tnethacrylate embedded bone samples. The etnbedding medium preserved the expression of several antigens. Type I collagen was lightly stained in the bone marrow fibrous tissue and stained intensely in non-mineralized osteoid seams and in woven bone. Type 111 collage11 stained throughout the fibrous tissue of the bone [narrow as well as in non-mineralized osteoid matrix. Intense immunostaining for basement membrane type IV collagen was evident within the fibrous mat-row tissue next to capillaries and sinusoids. Non-collagenous glycoproteitls, sitch as fibronectin and latninin, appeared evenly distributed throughout the fibrous bone marrow tissue. After decalcification, mineralized trabecular bone matrix became strongly positive for type I collagen and fibronectin. Light, patchly distributed itnmunostaining was observed for tenascin. The results frotn the present study provide insight into the routine ~tti-lization of i~n~nunohistocl~etnical staining in plastic embedded bone biopsies as a reliable procedure in the investigation of several co~nponetlts of the bone and bone liiarrow in nor~nal and pathological conditions. (The J Hi.stotecl71zol20: 253, 1997)

The prognostic significance of the additional abnormalities to the t(15; 17) remains controversial. We report a case of promyelocytic leukemia (APL) in a ten-year-old boy. Classical and molecular cytogenetic (FISH) studies of a bone marrow sample obtained at diagnosis revealed the presence of trisomy of chromosome 11 as an additional chromosomal abnormality to the t(15; 17). The presence of the translocation t(15; 17), the cytogenetic marker of APL, is usually associated with good response to treatment with ATRA. In this case, although the patient had risk factors associated with good prognosis, he evolved and died quickly. So it seems that the presence of the trisomy 11 may be associated with disease progression and the poor outcome. To our knowledge, this is the first reported case of t(15; 17) associated with trisomy of chromosome 11 in a child with APL.

OBJETIVO: Descrever os achados gerais do linfoma em pacientes abaixo de 20 anos de idade e por subtipo histológico. MATERIAIS E MÉTODOS: Estudo retrospectivo do arquivo digital de tomografia computadorizada do Centro de Controle do Câncer do Hospital Universitário Pedro Ernesto da Universidade do Estado do Rio de Janeiro, no período de março de 2003 a julho de 2005. Dos 22 casos - 16 do sexo masculino e 6 do sexo feminino, com média de idade de 11,5 anos -, 12 eram do subtipo Hodgkin e 10 eram não-Hodgkin. RESULTADOS: Dos achados gerais, verificamos as linfonodomegalias mediastinais como o mais freqüente (59%), com predomínio no grupo Hodgkin (75%), seguido por hepatoesplenomegalia (50%) e linfonodomegalias cervicais e retroperitoneais (27,3%). No subtipo Hodgkin houve predomínio do acometimento linfonodal, em sucessivas cadeias, seguido pela hepatoesplenomegalia (50%). Verificamos um caso de massa tonsilar unilateral, opacidade pulmonar em "vidro-fosco" e nódulos renais. No subtipo não-Hodgkin houve predomínio extranodal caracterizado por hepatoesplenomegalia (50%), espessamento de alça intestinal (40%), derrame pleural (30%), nódulo pulmonar (20%), ascite (10%), derrame pericárdico (10%) e lesões ósseas mistas (10%). CONCLUSÃO: A tomografia computadorizada é de grande valia no diagnóstico, estadiamento e seguimento do linfoma, com achados de alerta como massa linfonodal, notadamente mediastinal, hepatoesplenomegalia, massa unilateral na tonsila e espessamento parietal de alça intestinal.

Given discrepancies between methods for diagnosing hyposplenism, the purpose of this study was to evaluate the effect of the spleen size on the correlation between the methods, and to propose a model for improving the interpretation. Patients with renal allografts were included, in whom the spleen was assessed using Doppler ultrasound, scintiscan, and the presence of Howell-Jolly bodies (HJBs) in peripheral smears. In 35 subjects, scintiscan and HJBs were normal (Group 0); 20 had an abnormal result in both methods (Group 1); 34 had discordant results with HJBs present (Group 2); and 14 had discordant results with decreased spleen uptake (Group 3). There was no association between HJBs and scintiscan. The patients of Groups 1 and 2 had smaller spleens. The patients with smaller spleen had more hematological evidence of hyposplenism and exhibit smaller discrepancies between the methods than patients with larger spleen. The spleen can tip the balance from a normal to impaired function provided that the spleen size is below the critical mass required to maintain splenic function. A mild impairment of phagocytic function and slight dyserythropoiesis along with a small spleen would result in decreased take up of radiocolloid or the appearance of HJBs in blood smears.

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