Beneficial effects of interferon-alpha in a case with Behçet's disease.

At the age of 20 years, a Japanese man with recurrent oral aphthae, genital ulcers, folliculitis, erythema nodosum, episodic arthritis and epididymitis was diagnosed as having Behcet's disease (BD) in 1966. He has had active ocular manifestations of BD since 1990. These symptoms recurred and never abated for a long period of time. A right renal cell carcinoma developed and he underwent right nephrectomy in April 1996. Treatment with interferon-alpha was started from June 1996 as supplemental chemotherapy. No active phase developed during administration of IFN-alpha. We suggest that IFN-alpha may play a role as an immunomodulatory agent in BD.

[Moderate aplastic anemia associated with Crohn's disease during antithymocyte globulin treatment].

A 53-year-old woman with moderate aplastic anemia (AA) was treated with antithymocyte globulin (ATG). However, on the 4th day of treatment, ATG was discontinued because of bloody vomiting and melena. The patient improved with conservative treatment but complained of abdominal pain when the prednisolone (PSL) dose was decreased. Crohn's disease was finally diagnosed on the basis of upper and lower gastrointestinal X-ray studies. The patient responded well to ATG with hematologic improvement, and maintained remission with low-dose PSL and nutritional support. Drug-induced AA may occur during treatment for Crohn's diseases. The association of AA and Crohn's disease is rare, and to our knowledge, has not yet been reported in the literature. We discussed the pathogenesis of Crohn's disease during immunotherapy for AA.

[Intracranial hypertension in a patient with acute promyelocytic leukemia treated with all-trans retinoic acid].

A 21-year-old Japanese woman was referred to our hospital because of severe anemia and thrombocytopenia. Bone marrow aspiration showed a hypercellular marrow with 91.5% promyelocytes. Cytochemical study and surface marker a diagnosis of acute promyelocytic leukemia. Because leukocyte count elevated, she was treated with all-trans retinoic acid (ATRA) after conventional chemotherapy. After 11 days of ATRA therapy, the patient started to develop severe headache, nausea and diplopia. Ophthalmologic examination revealed bilateral papilledema. Computed tomography and magnetic resonance imaging of the head showed no intracranial lesion. ATRA was discontinued because it was suspected to cause intracranial hypertension. Her symptoms were relieved and patilledema improved gradually. ATRA is safe and well-tolerated, if the retinoic acid syndrome can be prevented or managed. As the tolerable dose of ATRA in adults is higher than that in children, the side effects tend to occur in children. In Japan, only two childhood cases of intracranial hypertension during ATRA therapy have been reported. We must remember the possibility of intracranial hypertension during ATRA therapy, even in adults.

Aggressive non-Hodgkin's lymphoma with massive involvement of the right ventricle.

A 69-year-old women with malignant lymphoma (diffuse large:Working formulation) had associated with intracavital involvement of the right ventricle. The patient was referred to us for further examination with a diagnosis of heart failure. A tumor mass was detected by magnetic resonance imaging in the right ventricular outflow tract. While in the hospital the patient experienced cardiogenic shock due to neoplastic pulmonary embolism. The tumor was surgically resected and the patient was then treated with systemic chemotherapy. She went into complete remission and has continued in this state for six months. This case demonstrates that early diagnosis and intensive chemotherapy contribute to a good prognosis of patients with malignant lymphoma associated with massive involvement of a cardiac chamber. A review of the Japanese literature is presented.

A patient with Tn syndrome associated with myelodysplastic syndrome showed abnormal glycophorin B.

A Japanese male patient with myelodysplastic syndrome (MDS) was shown to have associated Tn syndrome; the first report of Tn syndrome with MDS. The Tn expression was demonstrated on erythrocytes, granulocytes, monocytes, platelets, and lymphocytes by flow cytometric analysis using a lectin and an antibody. Electrophoresis of erythrocyte membrane proteins revealed slower mobility of glycophorin B from the patient than that from normal individuals, suggesting a glycophorin B molecular abnormality.

Point mutation in the band 4.2 gene associated with autosomal recessively inherited erythrocyte band 4.2 deficiency.

A patient who represented acute hemolytic crisis was studied. Analysis of the erythrocyte membrane proteins by SDS-PAGE revealed a deficiency of band 4.2. In the family, the sister of the patient who had been clinically normal was also shown to be deficient in band 4.2. Binding studies showed that the propositus' membranes were able to bind normal band 4.2 protein as much as control. It was suggested that the binding sites for the protein were prepared on the membrane. We analyzed the band 4.2 cDNA of the propositus and detected a mutation that changes a codon for alanine to one for threonine at residue 142. Band 4.2 exon III of genomic DNA which included the mutation site was amplified and sequenced directly in the family members, and it was revealed that only the homozygotes of the mutation allele manifested band 4.2 deficiency and the parents, who were heterozygotes, showed normal amounts of band 4.2. Recently, the same mutation was reported as Protein 4.2NIPPON in another 4 cases (Bouhassira et al. Blood 1992: 79: 1846-1854). This study supports the hypothesis that this mutation is the pathogenetic cause of band 4.2 deficiency and not a polymorphism.

Malignant fibrous histiocytoma of the liver: a case report and review of the literature.

Primary malignant fibrous histiocytoma (MFH) of the liver is reported in a 79-year-old man. The tumor, measuring 8.0 x 8.0 x 6.0 cm, was located in the left lobe of the liver and consisted of spindle cells in a storiform pattern intermingled with bizarre giant cells. Immunohistochemically, most of the tumor cells expressed vimentin. Cytoplasmic immunoreactivity for alpha 1-antichymotrypsin was documented in the giant cells. However, epithelial expression could not be demonstrated.