Aggressive natural killer-cell leukemia (ANKL) is a rare form of large granular lymphocyte leukemia, which is characterized by a systemic proliferation of NK cells. The clinical features of 22 ANKL cases were analyzed. Hepatomegaly (64%), splenomegaly (55%) and lymphadenopathy (41%) were also frequently observed. Leukemic cells were identified as CD1 À

Patients with Graves' disease (n = 61) treated with propylthiouracil (PTU) or thiamazole (MMI) were studied retrospectively to investigate differences in the prevalence of anti-myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA) in relation to treatment with anti-thyroid drugs. The patients were divided into two groups: PTU-treated group (n = 32) and MMI-treated group (n = 29). There were no significant differences between the two groups in terms of age, gender distribution, or duration of treatment. In the PTU group, 8/32 (25%) patients were positive for MPO-ANCA, whereas in the MMI group, 1/29 (3.4%) patients were positive. There were no significant differences in age, duration, or dosage between the MPO-ANCA positive and negative patients. Most of the MPO-ANCA positive patients were asymptomatic, except for two patients in whom rheumatic arthritis or membranous glomerulonephritis developed. None of the MPO-ANCA positive patients were diagnosed as having classical ANCA-associated vasculitis. Thus, there is a high frequency of MPO-ANCA in patients with Graves' disease treated with PTU, compared with patients treated with MMI, although classical ANCA-associated vasculitis develops in only a few MPO-ANCA positive patients.

Summary:spleen, serosal effusions, mucosal ulceration, elevated liver enzymes and hemorrhage. The clinical course is often fatal, 4-6 and liver failure, hemorrhagic diathesis, or infec-A 20-year-old Japanese man developed generalized, subcutaneous, painless nodules, fever, abnormal liver tion are the main causes of death, 2,7,8 but a number of patients with CHP with a benign clinical course have also function, serosal effusions, hepatosplenomegaly, lymphadenopathy and anemia. Skin biopsies revealed lobubeen described. 9 Thus, CHP can have a broad spectrum from mild to severe. In CHP, the following clinical and lar panniculitis with a morphologically benign histiocytic infiltration and prominent phagocytosis. Atypical laboratory features are associated with a poor prognosis: bleeding, mucosal ulcers, lymphadenopathy, fever, hepato-T lymphocytes were also present in the skin and liver. The diagnosis given was aggressive cytophagic histiosplenomegaly, serosal effusions, anemia, leukopenia, elevated liver enzyme levels, coagulopathy and hypercalcemia. cytic panniculitis (CHP) or aggressive subcutaneous panniculitic T cell lymphoma (SPTCL). He receivedDespite the absence of malignant changes within the histiocytic infiltrate, some patients die with hemorrhage and pancyclophosphamide, doxorubicin, and vincristine on day 1, prednisolone on days 1-5, and etoposide on days 1, cytopenia after a variable course. Other workers have suggested that cutaneous hemophagocytosis is observed as a 3 and 5 (CHOP-E), with the support of granulocyte colony-stimulating factor. This regimen was repeated reaction to subcutaneous T cell lymphoma, 10-12 and this lymphoma has been renamed subcutaneous panniculitic T every 2 weeks and complete clinical remission (CCR) was attained after three cycles of CHOP-E. As the clinicell lymphoma (SPTCL) in the recently proposed revised European-American classification of lymphoid neoplasms cal course of aggressive CHP is recurrent and often fatal, he was given high-dose chemotherapy followed by (REAL classification). 13 An effective therapeutic strategy for aggressive CHP or aggressive SPTCL has not been autologous peripheral blood stem cell transplantation (APBSCT), after five cycles of CHOP-E. He has established. We describe here the clinical features in a young man with aggressive CHP and our successful treatremained in CCR for 12 months after APBSCT. Highdose chemotherapy followed by APBSCT is considered ment which included APBSCT. to be one of the most beneficial therapies for patients with aggressive CHP and aggressive phase SPTCL. Keywords: cytophagic histiocytic panniculitis (CHP);Case report CHOP-E; APBSCT In 1990, a 15-year-old Japanese man developed small nontender subcutaneous nodules on his left cheek which disappeared spontaneously within a few weeks, leaving only a Cytophagic histiocytic panniculitis (CHP), a disorder few depressions. Skin biopsy of the lesions demonstrated described by Winkelmann et al 1 and Crotty et al, 2 is characatypical lymphocytes infiltrating to subcutaneous f...

Sirs: Idiopathic intracranial hypertension (pseudotumour cerebri) is uncommon in Japan, but no accurate data have been reported on its incidence. To determine the incidence of idiopathic intracranial hypertension (IIH) in Hokkaido, the northernmost island of Japan, we surveyed patients by post or telephone who were diagnosed with the condition in 1993. Hokkaido is the second largest island in Japan, at a latitude of 42°-46°N. It is served by 230 hospitals with 70 neurology departments, 196 neurosurgery departments and 64 ophthalmology departments. The population of Hokkaido was 5,780,000 in 1993. Inquiries were sent to all hospitals asking for information on cases of IIH diagnosed according to the modified Dandy criteria [1]. Of the 230 hospitals 221 responded.Only two cases of IIH were diagnosed during the year 1993.Case 1 was a 25-year-old woman presented with a 3-month history of severe headache and nausea. On examination she was thin and had bilateral papilledema. Magnetic resonance imaging of the brain and angiography ruled out mass lesion and sinus occlusion. Laboratory studies were normal. She was taking no medications. CSF pressure was 260 mmH 2 O. Administration of diuretics improved her symptoms.Case 2 was a 20-year-old man with non-Hodgkin lymphoma presented with a 2-month history of headache, nausea, and diplopia. He was not obese. Neurological examination showed bilateral papilledema and abducens nerve paresis. At this time he was not receiving chemotherapy or other drugs. Mass lesion and sinus occlusion were ruled out by magnetic resonance imaging of the brain and angiography. Laboratory studies were normal. CSF pressure was 290 mmH 2 O. Glycerol (hyperosmolar agent with concentrated glycerin fructose to reduce raised intracranial pressure) improved his symptoms. After 1 year he died of pneumonia. Autopsy showed no abnormalities in the central nervous system including the meninges, which supported the clinical diagnosis of IIH by excluding a meningitic infiltrative process.During this 1-year period only two patients, one man and one woman, were diagnosed as IIH. The crude incidence of IIH in Hokkaido in 1993 was thus 0.03 per 100,000. Both patients were atypical in the sense that neither was obese or was taking any medication. The papilledema and documented CSF pressures over 250 mmH 2 O were compatible with IIH according to the modified Dandy criteria.

We report on a 23-year-old Japanese female with a 13-year history of systemic lupus erythematosus (SLE), and two episodes of deterioration followed by treatment with high dose prednisolone. Although she had been recently treated with prednisolone (12.5 mg daily), her liver function became worse in July 1998. Results of a liver biopsy revealed multi-focal hepatic cell death in a severe fatty liver, without any inflammatory cell invasion. The biopsy also showed a positive TUNEL (Tdt-catalysed DNA nick end labelling) reaction indicating apoptosis. Her liver function recovered rapidly following steroid pulse therapy. Serum soluble Fas ligand (sFasL) was found to be elevated to a concentration of 0.395 ng/ml at the time of liver damage, but was less than 0.03 ng/ml before liver damage and after prednisolone treatment. The liver damage in this case appeared to be involved with apoptosis induced by sFasL. Although hepatitis associated with SLE is rare, apoptosis directly related to elevated sFasL levels might cause this complication.