A case of myasthenia gravis in a patient with diffuse systemic sclerosis developed during D-penicillamine treatment / 대한류마티스학회지

D-penicillamine has been used to reduce skin thickening and prevent the development of significant organ involvement in the treatment of scleroderma. This drug has a number of serious adverse reactions including glomerulonephritis with nephrotic syndrome, aplastic anemia, thrombocytopenia, and myasthenia gravis. A 44-year-old woman was admitted for weakness of the extremity muscle during repeated use. Eight months before admission, she visited dermatology department of our hospital. She was diagnosed as having scleroderma. D-penicillamine was started for the treatment of skin lesions. Based on the fluctuation of proximal muscle weakness, high titer of acetylcholine receptor antibody and definite decremental response of Jolly test, she was diagnosed as myasthenia gravis. D-penicillamine was discontinued because of the suspicion of D-penicillamine induced myasthenia gravis. Muscle weakness improved after D-penicillamine was withdrawn. The development of reversible myasthenia gravis may be regarded as a part of general predisposition for autoimmune disease related to the D-penicillamine therapy.

Retroperitoneal Fibrosis with Duodenal Stenosis

Retroperitoneal fibrosis is a rare disease characterized by the formation of dense plaque of fibrous tissue covering the retroperitoneal structures. This disease is commonly presented as ureteral obstruction, but the involvement of duodenum is rare. We report a case of retroperitoneal fibrosis which was complicated with duodenal stenosis and was successfully treated with corticosteroids. A 58-yr-old man, who had history of aorto-iliac bypass graft due to arteriosclerosis obliterans with infrarenal aortic occlusion was admitted to the hospital with abdominal pain and a mass. Abdominal CT scan revealed the periaortic soft tissue mass encircling grafted aorta and stenosis of duodenal third portion. Retroperitoneal fibrosis with duodenal stenosis was diagnosed and prednisolone therapy was initiated. Follow-up CT scan showed that the patient responded to prednisolone therapy with eased pain, shrinking periaortic mass, and reduced duodenal stenosis.

A Case of Systemic Lupus Erythematosus Presented with Sensorineural Hearing Loss / 대한류마티스학회지

A link between sensorineural hearing loss and autoimmune disease is postulated.The pathogenesis of sudden sensorineural hearing loss (SNHL)in patients with systemic lupus erythematosus (SLE)is not clear.However,several reports have suggested vasculitis secondary to SLE as underlying mechanism or association of antiphospholipid antibody (aPL).Although there were some cases of sensorineural hearing loss associated with systemic lupus erythematosus or lupuslike syndrome treated with plasmapheresis or immunosuppressive agents,there has been no case of SNHL in patients with SLE reported in Korea.We describe an 18-year-old female with systemic lupus erythematosus who presented with sudden sensorineural hearing loss.She was treated with high-dose corticosteroids with favorable clinical outcome.

A Case of a Young Woman with Hepatosplenic gamadelta T-cell Lymphoma / Journal of the Korean Cancer Association, 대한암학회지

Most T-cell lymphomas arise from mature alpabeta T-cells and commonly involve the nodes. Lymphomas bearing the gamadelta T-cell receptor (TCR) are very rare, and involve the lymph nodes minimally, if at all. Hepatosplenic gamadelta T-cell lymphoma is a recently identified, rare entity in which lymphoma cells bearing the gamadelta TCR infiltrate the sinusoids of the liver, splenic red pulp, and bone marrow. Its leukemic transformation is even more rare. Recently, we experienced a case of hepatosplenic gamadelta T-cell lymphoma in a 19-year-old woman who presented with epigastric pain, fever, massive splenomegaly, andpancytopenia. The splenectomy specimen and excisional biopsy of the liver revealed the infiltration of atypical T lymphocytes with the immunophenotypic markers of CD3 (+), CD45RO (pan-T antigen) (+), TIA-1(+), CD4(-),CD8 (-), CD56 (-), and S100 (-) in the sinusoids of the liver and splenic red pulp. Polymerase chain reaction (PCR) showed that these cells had the expression of the TCR gama gene rearrangements. Though the pancytopenia had improved after the splenectomy, the response of chemotherapy was transient. Her disease progressed rapidly and she expired in the leukemic phase. We report a case of hepatosplenic gamadelta T-cell lymphoma that developed in a young woman, along with a brief review of the literature.