Journal of clinical pathology
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Patients without spleens are at increased risk of overwhelming infection. Recently, greater efforts, including the publication of national guidelines, have been made to improve the management of asplenic individuals. In theory, risks of serious sepsis can be reduced by good advice, immunisation, and antibiotic prophylaxis. In practice, such preventive measures might not be followed or may fail. A study of recent cases of overwhelming postsplenectomy infection (OPSI) was undertaken to examine specific associated factors and to determine whether currently recommended preventive measures are being followed. ⋯ Currently accepted best practice for managing asplenic patients is not being followed. Some OPSI cases may still be preventable but many asplenic individuals remain unrecognised. The compilation of asplenic patient registers might help to implement agreed policies with audit necessary to evaluate compliance. More is needed to ensure optimal management for this cohort of the population.
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Case Reports
Unusual manifestations of type II cryoglobulinaemia associated with Waldenström's macroglobulinaemia.
Cryoglobulinaemia in association with Waldenström's macroglobulinaemia is relatively common, ranging from 8% to 18% of cases; however, < 5% have symptoms or complications. We describe a patient with a history of cutaneous, peritoneal, and fallopian tube vasculitis related to type II cryoglobulinaemia associated with Waldenström's macroglobulinaemia. ⋯ However, after the third course of chemotherapy, the patient presented with septic shock and died. Even though cryoglobulinaemia is a model of systemic vasculitis, peritoneal and fallopian tube vasculitis associated with type II cryoglobulinemia has not been described previously.
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Aggressive angiomyxoma of pelvic parts is a distinctive soft tissue tumour that chiefly involves the vulvar and perineal region of female patients. Several previous reports have demonstrated oestrogen receptor (ER) and/or progesterone receptor (PR) positivity in this neoplasm. The aim of this study was to confirm whether ER and/or PR positivity is present in aggressive angiomyxoma. We also wished to ascertain whether positivity may be found in the stromal cells of normal vulval skin and in other lesions at this site that can cause diagnostic confusion with aggressive angiomyxoma. ⋯ All cases of aggressive angiomyxoma of pelvic soft parts in female patients exhibited positivity for ER and/or PR. Because of its propensity to occur in female patients during the reproductive years, it is possible that aggressive angiomyxoma is a hormonally responsive neoplasm. However, dermal fibroblasts in normal vulval skin and stromal cells in a variety of vulval lesions can also be positive. ER or PR immunoreactivity cannot be used to distinguish aggressive angiomyxoma and its histological mimics.
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A case of myelomatous involvement of the dura mater is reported. The patient presented with blurring of vision in the right visual field and left sided facial numbness. ⋯ The infiltration of the dura mater is likely to have arisen by spread from contiguous bone lesions, contrasting with the pattern of spread seen in myelomatous involvement of the leptomeninges, which probably occurs through haematogenous seeding of the meninges. Leptomeningeal involvement is associated with a very poor prognosis; however, this patient had a favourable response to combined chemotherapy and cranial radiotherapy, suggesting that myelomatous involvement of the dura mater should be considered as a distinct complication of myeloma.