Pigmented Macrophages Research Articles

Brain lesions in chronic granulomatous disease

In chronic granulomatous disease (CGD) enzyme-deficient neutrophils and mononuclear cells lack the respiratory burst required for biocidal activity. Recurrent infections lead to granulomas in various organs but brain lesions are rare. In the present case, a 23-year-old male with numerous infections since early childhood died of overwhelming pulmonary aspergillosis. He first began to experience neurological deficits at the age of 17. Computerized tomography and magnetic resonance imaging revealed fleeting white matter lesions that were interpreted as multiple sclerosis (MS). At post mortem, three types of brain lesions were found: (1) Pigmented macrophages in perivascular spaces and the leptomeninges similar to those reported previously. They contained fine, golden-brown, lipofuscin-like material whose chemical composition included a sulfur peak by X-ray analysis. (2) Focal, well-demarcated, "burnt out" white matter lesions with loss of both myelin and axons and intense sclerosis. (3) Diffuse areas of mild pallor in the centrum ovale which spared the U fibers. The pigmented macrophages are characteristic of those seen in the periphery in CGD. The origin of the discrete, destructive white matter lesions is unclear. They may have resulted from: (i) earlier activity by CGD macrophages; (ii) previous infections due to sepsis or embolism; or (iii) possibly post-infectious encephalomyelitis. The more diffuse, mild, white matter lesions are attributed to edema. Evidence for MS, progressive multifocal leukoencephalopathy, or human immunodeficiency virus encephalitis was lacking. This case is presented to alert us to look more carefully for brain lesions in CGD, characterize them and to help determine their cause.

Clinical and histopathological aspects of Kaposi's sarcoma in Africa: Relationship with HIV serology

From 1983 to 1987, 45 Kaposi's sarcomas (KS) were diagnosed at the University Hospital in Bangui; 37 cases were easily classified as either endemic or AIDS-related KS on clinical grounds and HIV serology. Moreover, probably due to the stage at which patients consulted and lesions were sampled, noticeable histopathological differences were observed between the two clinical presentations. But for 8/45 which we classified as "borderline KS", strong discrepancies occurred between clinical aspects, patient evolution, HIV serology and histopathology. In two cases, HIV-positive patients had typical endemic non-evolutive KS and have survived 15 and 36 months.

Chronic Inhalation Studies in Mice. II. Effects of Long-Term Exposure to 2R1 Cigarette Smoke on (C57BL/Cum × C3H/AnfCum)F<sub>1</sub> Mice<xref ref-type="fn" rid="FN1">2</xref><xref ref-type="fn" rid="FN2">3</xref>

Standardized exposure conditions with Kentucky reference 2R1 cigarettes were used to expose 2,053 (C57BL/Cum x C3H/AnfCum)F1 female mice (nose only) to fresh, whole cigarette smoke. In addition, 1,014 mice were sham-exposed, and 449 mice were held as shelf controls. The protocol entailed exposing mice to smoke (or sham-exposure) on a daily basis, 5 days/week, for 110 weeks and observing remaining mice until death. A large number of animals was used so that the smoke generation and animal-holding systems could be tested and evaluated and yet provide significant numbers of animals for exposure to cigarette smoke for a major portion of their lifetime. Deposition of smoke particulates was estimated to be about 125-200 micrograms total particulate matter/lung/day. The only lung cancers observed were diagnosed as alveolar adenocarcinomas (AAC). A total of 19 of 978 smoke-exposed mice and 7 of 651 sham-exposed mice were observed with AAC. The difference between the smoke-and sham-exposed groups was not statistically significant at P less than or equal to .05, but the data suggested that the tumors occurred with a shorter latency in the smoke-exposed group (P = .10). The data were analyzed by various methods, including analysis of subsets of the population of animals. A significant increase in the incidence of lung cancer was observed in one subset; however, this difference was not found in the population as a whole or as a result of any other analyses. Under these exposure conditions, 2R1 cigarette smoke would seem to have weak carcinogenic activity in mouse lung tissue. Other changes associated with smoke exposure were increased incidence of pigmented alveolar macrophage accumulation, otitis media, and head and neck fibrosarcomas. However, the incidence of nephritis, hematopoietic cancers (e.g., leukemias, lymphosarcomas, and reticulum cell sarcomas), and pulmonary congestion was significantly higher in the sham-exposed animals.

Ocular Histopathologic and Biochemical Studies of the Cerebrohepatorenal Syndrome (Zellweger's Syndrome) and its Relationship to Neonatal Adrenoleukodystrophy

The eyes of three infants with cerebrohepatorenal disease (Zellweger's syndrome) who died demonstrated ganglion cell loss, gliosis of the nerve fiber layer and optic nerve, optic atrophy, and changes resembling those of retinitis pigmentosa in the retina and pigment epithelium. Ultrastructural examination showed bileaflet inclusions identical to those seen in neonatal adrenoleukodystrophy in the pigment epithelium and in pigmented macrophages, but these were absent in the cornea. Biochemical analysis of tissues demonstrated an excessive amount of very-long-chain fatty acids in the ocular tissues, an abnormality also found in adrenoleukodystrophy. These histopathologic and biochemical results demonstrated that the cerebrohepatorenal syndrome and neonatal adrenoleukodystrophy are similar in regard to ocular abnormalities and the presence of saturated very-long-chain fatty acids.

Anatomic and Histologic Changes of Early Emphysema

Most systems of classifying emphysema grade the lesions according to type, distribution, and extent of involvement from barely visible onward. Clinical and anatomic evidence of progression in these lesions suggests the existence of earlier stages. Thirty-nine lungs were studied. All appeared grossly normal but contained widespread macroscopic foci of early centrilobular and panlobular emphysema. Numerous abnormal brownish pigmented alveolar macrophages were found in the adjacent, otherwise intact appearing parenchyma. They were not found in normal lungs. Identical pigmented macrophages were found in sputum specimens obtained from apparently healthy cigarette smokers. Frequency of occurrence appeared related to the number of cigarettes consumed. The brownish pigment resembled tobacco but could not be identified as such. In heavy smokers, many of these cells also contained iron particles, suggesting the possibility of tissue damage. Such cells are thought to produce proteases. Their occurrence prior to evidence of bronchiolar-alveolar destruction may be of particular significance in the various alpha 1 -antitrypsin deficiency states.

Albinism Associated with Hemorrhagic Diathesis and Unusual Pigmented Reticular Cells in the Bone Marrow: Report of Two Cases with Histochemical Studies

Abstract 1. A description is given of two unrelated albinos with hemorrhagic diathesis and peculiar, pigmented reticular cells in the bone marrow. 2. On examining hemostasis, the only consistent abnormal laboratory finding was a prolonged bleeding time, so that the hemorrhagic disorder fell into the group of so-called pseudohemophilias. 3. Unusual reticuloendothelial cells in the bone marrow were packed with blackish or greenish blue granules or particles. According to the histochemical study, the substance in the cytoplasm was probably of a lipid nature. These cells could not be identified with similar cells of this kind which have so far been described. 4. The combination of the above described congenital abnormalities (albinism, pseudohemophilia and unusual pigmented macrophages in the bone marrow) in two unrelated patients suggests that a common syndrome is present.