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1/13. Increased sister chromatid exchange in bone marrow and blood cells from Bloom's syndrome.

    Bone-marrow cells from a patient with Bloom's syndrome cultured for 48 h in the presence of BudR exhibited a striking increase in the number of sister chromatid exchanges (SCEs) in comparison to that in the marrow cells of a patient with treated polycythemia vera (PV). Thus, it appears that an increased incidence of SCE in Bloom's syndrome occurs in various differentiated types of cells, not just blood lymphocytes, and constitutes the syndrome's most characteristic cytogenetic feature. In contrast, the incidence of SCE was not increased in marrow cells and lymphocytes of the particular PV patient studied here, whose cells did exhibit increased numbers of chromatid and chromosome gaps and breaks, presumably as result of the patient's earlier treatment. An increased frequency of SCE was demonstrated in Bloom's syndrome lymphocytes using both a technique based on BudR incorporation and one based on labeling with tritated deoxycytidine. This observation constitutes evidence against the increase of SCE being due to an unusual reaction to BudR. By conventional cytogenetic techniques, chromosome instability, including chromatid and chromosome breaks, but no homologous chromatid interchanges were also recognized in Bloom's syndrome bone-marrow cells incubated in vitro (without BudR) for either 1.k or 16 h. This observation points to the existence of chromosome instability in vivo.
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2/13. Photoinduced dermal pigmentation in patients taking tricyclic antidepressants: histology, electron microscopy, and energy dispersive spectroscopy.

    Two patients had been taking long-term tricyclic antidepressant therapy. Each developed a blue to slate-gray hyperpigmentation in sun-exposed areas. On histologic examination there were refractile golden brown granules free in the dermis along collagen bundles. Similar pigment was present in macrophages and along the basement membrane zone. The granules stained for melanin, but not for iron, and were bleached by the permanganate method. Electron microscopy showed varying size and shaped electron-dense granules within lysosomes and free in the dermis, which, in unstained sections, showed a less dense peripheral halo. This peripheral halo was also evident on light microscopy. Energy dispersive spectroscopy showed these granules to be rich in copper and sulfur (elements present in tyrosinase and pheomelanin, respectively). We believe that this represents a drug-melanosome complex, which is most likely caused by chronic photoactivation.
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keywords = macrophage
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3/13. Polymorphic light eruption occurring in common variable hypogammaglobulinaemia, and resolving with intravenous immunoglobulin therapy.

    A 55-year-old woman with a past history of lower respiratory tract infections presented with a photosensitive eruption. Polymorphic light eruption (PLE) was diagnosed on the basis of the temporal relationship to sun exposure and the diagnosis was supported by positive monochromator irradiation tests in the ultraviolet A wavelength spectrum. Investigation of the patient's immune status identified low levels of all immunoglobulin (Ig) subtypes consistent with common variable hypogammaglobulinaemia. Intravenous Ig replacement therapy, instituted to minimize risks from bacterial infections, was commenced and over the ensuing months resulted in a complete resolution of the PLE. PLE is considered to represent a type IV hypersensitivity reaction directed against a cutaneous autoantigen induced by exposure to ultraviolet light. In PLE, nonspecific immunomodulatory mechanisms of intravenous Ig may be active, such as a reduction in the synthesis of cytokines and a blockage of the IgG Fc receptors on macrophages.
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ranking = 509.64899581644
keywords = macrophage
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4/13. Photodistributed blue-gray pigmentation of the skin associated with long-term imipramine use.

    A 72-year-old white woman presented with progressively increasing slate-gray pigmentation of the face and extensor aspect of the forearms, which she had been suffering from for 8 years. She had been taking imipramine for approximately 30 years. Her other medications included ranitidine and anacin. physical examination revealed slate-gray hyperpigmentation of the skin photodistributed on the face (Figs 1, 2) and extensor aspects of the forearms. Relative sparing of the skin folds, mucous membranes, sclerae, teeth, and nails was noted. The remainder of the physical examination revealed no abnormalities. skin biopsy specimens from the right cheek and right forearm were obtained. Histologic examination revealed collections of variably sized, round to ovoid, yellow-brown globular deposits in the upper and mid dermis (Fig. 2). The deposits were identified within macrophages and free within the dermis. The epidermis was unremarkable and free of deposits. The deposits stained for melanin with a Fontana-Masson stain, but did not stain for iron.
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keywords = macrophage
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5/13. Actinic superficial folliculitis.

    Actinic superficial folliculitis was first described in 1985, and since then only three reports have been published. Clinically and histopathologically this disease is very particular and has been suggested to be considered as an entity. We report on a 30-year-old man who presented with an extensive superficial follicular pustulosis on his back, shoulders and upper chest after exposure to intense heat and subsequent sweating on a sunny day. The pustules arose within 24-36 h afterwards. histology and immunohistochemistry revealed subcorneal pustules, suppurative folliculitis and an infiltrate consisting of T cells, macrophages and neutrophils around the hair follicle, sebaceous glands and small vessels. To the best of our knowledge this is the fourth report on actinic superficial folliculitis and the first on which a characterization of the inflammatory infiltrate has been performed. Because of the impressive, unique symptoms and the characteristic histology we agree with those who have suggested that actinic superficial folliculitis is a new entity.
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keywords = macrophage
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6/13. Photosensitivity and acute liver injury in myeloproliferative disorder secondary to late-onset protoporphyria caused by deletion of a ferrochelatase gene in hematopoietic cells.

    Late-onset erythropoietic protoporphyria (EPP) is a rare complication of myelodysplastic syndrome (MDS) but has not been described in association with a myeloproliferative disorder (MPD). EPP is normally an inherited disorder characterized by photosensitivity that starts in early childhood and results from overproduction of protoporphyrin secondary to ferrochelatase (FECH) deficiency. Severe liver disease occurs in 1% to 2% of patients. Here we report that severe photosensitivity and cholestatic liver disease in a patient with a myeloproliferative disorder was caused by excess protoporphyrin production from a clone of hematopoietic cells in which one FECH allele had been deleted. Our observations suggest that the usual explanation for the association of late-onset EPP with MPD and MDS is acquired somatic mutation of one FECH allele in bone marrow and show for the first time that the consequent overproduction of protoporphyrin may be severe enough to cause acute liver damage.
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keywords = bone
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7/13. rothmund-thomson syndrome in two siblings.

    rothmund-thomson syndrome, a rare autosomal recessive disorder, is characterized by photosensitivity; specific skin changes including poikiloderma, atrophy, and telangiectases; juvenile cataracts; short stature; and bone defects. We describe two siblings with this syndrome.
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8/13. quinidine-induced lichenoid and eczematous photodermatitis.

    We report 6 cases of photodistributed rashes due to oral administration of quinidine: 4 cases with a lichen-planus-like eruption and 2 with an eczematous dermatitis. The casual relationship between the drug and the eruption was established mainly by means of circumstantial evidence. It was further strengthened by an in vitro challenge test in 4 cases and by a rechallenge in 2 patients. The in vitro challenge test used was the macrophage migration inhibition test, which can be of diagnostic aid whenever a rechallenge of the patient is contraindicated. quinidine-induced lichenoid eruption in a photodistribution seems to be more common than previously reported. physicians should be aware of this type of drug eruption.
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keywords = macrophage
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9/13. Lichenoid eruption due to hydrochlorothiazide. Diagnostic aid of macrophage migration inhibition factor (MIF) test.

    The case of a lichenoid photosensitive eruption induced by hydrochlorothiazide is described. The macrophage migration inhibition factor test identified hydrochlorothiazide as the offending drug out of the several suspected drugs taken by the patient. It is suggested that an allergic reaction towards hydrochlorothiazide was involved in the development of the photosensitive lichenoid eruption in this case.
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ranking = 2548.2449790822
keywords = macrophage
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10/13. rothmund-thomson syndrome. A case report, phototesting, and literature review.

    rothmund-thomson syndrome is a rare hereditary syndrome with developmental defects. Characteristics of this syndrome, based on a review of 107 reported cases in the literature, are (in descending order) as follows: early onset of poikiloderma, short stature, absence or sparseness of eyebrow and/or eyelash hair, familial juvenile cataracts, small hands and bone defects, sunlight sensitivity, hypogonadism, defective dentition, nail abnormality, hyperkeratosis, and mental retardation. Recently we encountered a 25-year-old white woman who had developed this syndrome but without juvenile cataracts, hypogonadism, or mental retardation. She had developed a basal cell epithelioma, which has not previously been described in this syndrome. Phototesting with monochromatic radiation and with a solar simulator showed photosensitivity in the ultraviolet A range but not in the ultraviolet B range. This case may represent an example of the Thomson type. The case is described and the literature reviewed.
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