No abstract

Malign mezotelyoma asbest temasından 20-40 yıl sonra ortaya çıkan, prognozu kötü bir tümördür. Burada 27 yaşında, bilinen asbest teması olmayan bir malign plevral mezotelyoma olgusu sunulmuştur. Sigara öyküsü olmayan bayan hasta nefes darlığı ve yan ağrısı ile başvurdu. Fizik muayenede, sağ alt zonda matite tespit edilen hastanın akciğer grafisinde sağda orta zona kadar uzanan serbest plevral efüz-yon ve her iki akciğerde multipl, nodüler dansiteler saptandı. Plevral sıvı incelemesinde hemorajik karakterde eksüdatif efüzyon izlendi. Plevral sıvın ADA seviyesi, ARB ve mikobakteri kültürü negatif bulundu. Sıvı sitolojisi malignite açısından kuşkulu bulunan hastada primer odak araştırması yapıldı. Hastaya torakoskopi planlandı, pariyetal plevrada lokal tümör odakları, visseral plevrada kalınlaşma gözlendi. Biyopsi örneklerinin patoloji ve immün boyama sonuç-ları bifazik malign mezotelyoma olarak raporlandı. Sonuç olarak, malign plevral efüzyon varlığında mezotelyoma olasılığı göz ardı edilmemeli ve tanıyı kesinleştirmek için ayrıntılı immünohistokimyasal inceleme yapılmalıdır. Anahtar Sözcükler: Genç yaş, plevral efüzyon, immün boyama, podoplanin, mezotelyoma.Malignant mesothelioma is a tumor with poor prognosis that usually occurs 20-40 years after asbestos exposure. Here, we present a 27-year-old patient with malignant pleural mesothelioma who did not report any asbestos exposure. A female non-smoker was admitted with complaints of chest pain and dyspnea. The physical examination revealed dullness with percussion in the right lower lobe. There was a free-flowing pleural effusion through the right middle zone and bilaterally nodular opacities in chest x-ray. The ADA level, ARB and mycobacterium culture of the pleural fluid were negative. There were some malignant cells in the cytological examination of the fluid, and therefore the primary site of malignancy was evaluated. Thoracoscopy revealed nodular tumoral infiltration in the parietal pleura and thickness of the visceral layer. The pathological examination and immunostaining of the biopsy specimens were reported as biphasic malignant mesothelioma. In conclusion, mesothelioma should be considered in the case of malignant pleural effusion and detailed immunostaining should be performed in order to ascertain the diagnosis.

Background Systemic mastocytosis is a clonal disease of mast cell progenitors in bone marrow which causes abnormal growth and accumulation of mast cells in skin and other organs. Bone involvement occurs in 70% of cases; sclerotic, lytic lesions, and osteoporosis have been described, but they are rare as a form of disease presentation. Objectives To describe the main epidemiological and clinical features of systemic mastocytosis, focusing especially on bone involvement. Methods We make a review of systemic mastocytosis patients diagnosed in our hospital (by Rheumatology, Allergy and Hematology departments) and describe the main epidemiological and clinical characteristics. Results We found 5 cases of systemic mastocytosis diagnosed in a 22 year period. The study includes 3 women and 2 men, ages ranged 27 to 59 years. The delay of diagnosis from the onset of symptoms varies between 3 and 13 years. It presented with respiratory involvement in 2 cases, digestive in 1 case, skin in 1 case, and bone in only 1 case. 3 patients had allergic reactions in some stage of evolution, 3 skin symptoms, 3 patients had episodes of hypotension, dizziness, palpitations and syncope. Regarding to the musculoeskeletal system, 4 patients had bone involvement, 1 patient previously diagnosed with chronic osteomyelitis and 3 patients with osteoporosis and multiple vertebral collapses, one patient also had multiple rib fractures. The skeletal survey study showed 3 patients with lytic bone lesions and 2 patients with sclerotic lesions. Tryptase levels were elevated in 4 patients, whereas urine histamine levels were elevated in all 5 patients. Every patient had a bone marrow biopsy with diagnosis of systemic mastocytosis. Conclusions Systemic mastocytosis is a rare disease in our environment, the diagnosis uses to be late and requires high index of suspicion. Osteoporosis secondary to mastocytosis is usually severe, and it is very rare as the first manifestation of the disease. Two of the cases reviewed in this series had been admitted to our department for vertebral compression fractures, and we made the diagnosis in one of them, a middle-aged man with no other cause of osteoporosis, with initial bone marrow aspirate apparently normal. It has been reported that up to 9% of cases of “idiopathic” male osteoporosis may be caused by mastocytosis. We believe that tryptase and histamine levels should be included in the lab exams in patients with osteoporosis and fractures after ruling out other common causes. References References: Barete S, Assous N, de Gennes C, Grandpeix C, Feger F, Palmerini F, Dubreuil P, Arock M, Roux C, Launay JM, Fraitag S, Canioni D, Billemont B, Suarez F, Lanternier F, Lortholary O, Hermine O, Francès C. Systemic mastocytosis and bone involvement in a cohort of 75 patients. Ann Rheum Dis. 2010 Oct;69(10):1838-41. doi: 10.1136/ard.2009.124511. Epub 2010 Jun 22. Salles M. Holgado S. Navarro J.-T. Ribera J.-M. Riera E. Milla F. Tena X. Osteoporosis as a first manifestation of systemic mastocytosis. Study of 6 cases. M...

ÖZ Amaç: Malignite ön tanısı ile fiberoptik bronkoskopi yapılan ve endobronşiyal lezyon izlenen hastalarda bronş lavajının tanıya katkısının değerlendirilmesi amaçlandı. Gereç ve