RESUMEN
Erdheim-Chester disease is a rare non-langerhans cell histiocytic disorder of an unknown cause that may associate with multisystem, rapidly fatal, infiltrative disease. We report here on a case of Erdheim-Chester disease in a 45-year-old man who presented with chronic renal failure. The patient visited a local clinic due to renal failure that was accidentally diagnosed on a routine health examination. At that time, abdominal computed tomography revealed retroperitoneal fibrosis and bilateral hydronephrosis. However, he did not visit hospital for further evaluation. After 1 year, he visited our clinic due to exertional dyspnea that had developed several months previously. The chest and abdominal CT showed interstitial pneumonitis and pleural effusion and there was progressed hydronephrosis and perirenal fibrosis. He was diagnosed with Erdheim-Chester disease based on the characteristic findings of pleural biopsy (interstitial fibrosis, infiltration of macrophge, CD68-positive but S-100 negative). The patient was treated with insertion of a double J catheter and immunesuppression with prednisolone and azathioprine. Erdheim-Chester disease is a rare disease, but it should be considered in those patients displaying chronic renal failure with retroperitoneal fibrosis.
Asunto(s)
Humanos , Persona de Mediana Edad , Azatioprina , Biopsia , Catéteres , Disnea , Enfermedad de Erdheim-Chester , Fibrosis , Hidronefrosis , Fallo Renal Crónico , Enfermedades Pulmonares Intersticiales , Derrame Pleural , Prednisolona , Enfermedades Raras , Insuficiencia Renal , Fibrosis Retroperitoneal , Tórax , Tomografía Computarizada por Rayos XRESUMEN
The occurrence of granulocytic sarcoma as a pattern of relapse after allogenic bone marrow transplantation (allo-BMT) is rare. We report two AML1/ETO positive patients with granulocytic sarcoma as a pattern of relapse after allo-BMT. The first case is 39-year-old woman who had lower back pain without any abnormal peripheral blood exam. MRI showed a paraspinal mass, and the pathologic report was granulocytic sarcoma. The second case is 34-year-old woman who had breast humps without any abnormal peripheral blood exam. CT showed multiple breast masses, and the pathologic report was granulocytic sarcoma. We believe that this is the first cases report of extramedullary relapse after allo-BMT in AML1/ETO positive AML in Korea. These cases suggest that we should be aware of the possibility of an extramedullary relapse in bone marrow transplant recipients with AML1/ETO positive AML.