RESUMO
Systemic mastocytosis is a rare myeloproliferative disorder characterized by abnormal accumulation of mast cells in a variety of organs. When affecting the gastrointestinal tracts, it may manifest with steatorrhea, malabsorption, hepatomegaly, splenomegaly, portal hypertension, and ascites, among others. To our knowledge, only one case of systemic mastocytosis has been reported affecting the appendix. We present another case of a 47-year-old woman who was admitted for right-sided acute abdominal pain and found to have systemic mastocytosis in her appendectomy specimen as the first and only manifestation of her disease.
RESUMO
Three histologic patterns of gastric siderosis (GS) are described: pattern A (predominantly in lamina propria stromal cells-gastric lamina propria siderosis [GLPS]), pattern B (mostly extracellular crystalline iron) and pattern C (predominantly in glandular epithelium-gastric glandular siderosis [GGS]). This study aimed to analyze the association of GGS with clinicopathologic features using 3 cohorts. Cohort #1 consisted of 76 gastric siderosis cases. Upon classifying the cases into 3 groups by percentage of glandular involvement (negative, 1% to 5%, ≥5% GGS), the degree of GGS was positively associated with serum ferritin levels ( P =0.002), transferrin saturation ( P =0.003), and history of blood transfusion ( P =0.009). After excluding cases with coarse extracellular crystalline iron, cohort #1 was reclassified into 3 groups by degree of GLPS (no, rare [discernible at ×20 or ×40], overt [readily visible at low power]). The degree of GLPS was positively correlated with oral iron pill use ( P =0.01), but not serum ferritin levels or transferrin saturation. Cohort #2 contained 31 gastric samples from patients with hereditary hemochromatosis, most received phlebotomy treatment. GGS was identified in 2 (6.4%) patients; both had high ferritin levels. Cohort #3 included 38 gastric samples from patients with cirrhosis. Three (8%) cases showed GGS; serum ferritin level was available for 1 case and was elevated. These results indicate that GGS is associated with systemic iron overload, while GLPS is correlated with oral iron pill use. The identification of GGS, especially when it's ≥5%, should trigger further workup for potential systemic iron overload and underlying etiologies.
Assuntos
Sobrecarga de Ferro , Siderose , Humanos , Siderose/complicações , Siderose/patologia , Ferro/metabolismo , Sobrecarga de Ferro/etiologia , Ferritinas , TransferrinasRESUMO
Recently, the use of immunotherapy has increased substantially for the treatment of several malignancies. It is associated with several gastrointestinal adverse events; however, severe complications such as intestinal perforation are rare. We present a 75-year-old man with metastatic melanoma, presented with profuse diarrhea and abdominal pain, after ipilimumab and nivolumab administration. Shortly after, he developed fulminant colitis and intestinal perforation and was found to have concurrent Rosai-Dorfman disease of pericolonic lymph nodes. With the increasing use of immunotherapy, reporting of serious adverse events and their mimics is essential. In addition, further studies are required to investigate whether an association exists between Rosai-Dorfman disease and immunotherapy.
RESUMO
Workers in a print shop are exposed to photocopier toner dust and paper dust over a prolonged period of time. However, there are only rare case reports of toner and paper dust induced lung damage in humans. We reviewed our consultation files for a period of 30 years from 1987 to 2018 to look for cases with a diagnosis of giant cell interstitial pneumonia (GIP), printer toner exposure and paper dust exposure resulting in lung disease. There were two cases which met our inclusion criteria. Slides, clinical histories and imaging were reviewed. Both the patients had worked in print shops, and had no history of exposure to hard metals. Patient 1 presented with shortness of breath and cough over several months, while patient 2 was asymptomatic at presentation. Both the patients underwent surgical lung biopsies. Histopathologic examination from both the cases showed a spectrum of pathology, including features of GIP, desquamative interstitial pneumonia, chronic bronchiolitis with lymphoid hyperplasia, and particulate matter consistent with toner. Energy dispersive spectroscopy was performed on one case, and it revealed no cobalt or tungsten particles. The unusual combination of findings is very suggestive that toner particles with or without paper dust exposure were responsible for the pathologic changes in the lungs of these patients. This possibility should be explored further with additional patients who work in print shops where they are exposed to paper dust and paper toner and have signs or symptoms of diffuse lung disease.
Assuntos
Poeira , Tinta , Exposição Ocupacional/efeitos adversos , Papel , Pneumoconiose/etiologia , Impressão , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Cutaneous myxomas are rare benign neoplasms which are frequently associated with Carney complex (CNC). Although more than 500 cases of CNC are reported, there is no literature on cytologic diagnosis of Cutaneous myxomas. An 18-year-old male, with no significant family history, presented with multiple cutaneous swellings, largest measuring 15 cm on the right cheek. He also had spotty skin pigmentations, raised adrenocorticotropic hormone levels and recurrent cardiac myxomas. Fine-needle aspiration cytology from the right cheek and suprapubic swellings revealed paucicellular smears with abundant myxoid material in the background, admixed with fragments of spindle and stellate cells with bland nuclear morphology, and vascular proliferation in few fragments. There was no mitosis, necrosis, or any epithelial element. Hence, diagnosis of cutaneous myxomas in CNC was made which was confirmed on histopathology. This is the first report of cytologic diagnosis of multiple cutaneous myxomas in CNC and the largest cutaneous myxoma reported in literature.