Adult T-cell leukemia predominantly involving exocrine glands.

OBJECTIVES: We describe a rare case of adult T-cell leukemia (ATL) presenting with dry mouth and swelling of bilateral parotid and submandibular glands. The unusual involvement of these exocrine glands by malignant cells prompted us to conduct a detail characterization of these infiltrating and circulating leukemic T cells, which may provide insight to the pathogenesis of exocrine involvement in ATL. METHODS: Immunophenotyping of peripheral ATL cells and microscopic examinations of various organs prepared by autopsy were performed. Analysis of the repertoire of T-cell receptor (TCR) of parotid gland-infiltrating ATL cells using molecular and immunohistochemical examinations were also performed. RESULTS: Microscopic examinations of various organs prepared by autopsy revealed the predominant and specific exocrine gland infiltration of ATL cells. Reverse transcription-polymerase chain reaction (RT-PCR) followed by both TCR spectratyping and complementary determining region (CDR)-3 sequencing analysis of TCR Vbeta of parotid gland-infiltrating T cells revealed a relatively restricted but not single usage of TCR Vbeta. Immunohistochemical analyses of parotid gland specimens detected only a small number of TCR Valphabeta-positive cells in parotid gland-infiltrating ATL cells. CONCLUSIONS: The predominant infiltration of ATL cells in exocrine glands implied that these T cells recognized exocrine gland-specific antigen. However, the absence of both TCR Vbeta mRNA transcripts and TCR Valphabeta protein expression in most ATL cells suggested that antigen recognition via TCR may not have played a major role in adhesion and subsequent infiltration into the exocrine glands in this patient. These results provide important background information to further elucidate the pathogenesis of exocrine gland-specific inflammation.

L-asparaginase-Based induction therapy for advanced extranodal NK/T-cell lymphoma.

We describe treatment of a patient with advanced extranodal NK/T-cell lymphoma, nasal type, with multiple subcutaneous lesions and hemophagocytic syndrome. Considering the projected poor outcome of conventional treatments, we designed an L-asparaginase-based induction therapy. L-asparaginase (4000 units/day, day 1 to day 7) combined with vincristine (1 mg, day 1) and prednisolone (100 mg/day, day 1 to day 5) was administered by intravenous infusion every 3 weeks. Within a week after treatment was started, excellent response was observed. Because of an allergic reaction to L-asparaginase, 6 courses of CHOP (adriamycin, cyclophosphamide, vincristine and prednisolone) therapy were administered as consolidation after 4 courses of L-asparaginase. The lymphoma was controlled with complete remission lasting longer than 2 years without additional treatment. These results and related reports may contribute to greater therapeutic efficacy against at least some cases of extranodal NK/T-cell lymphoma and other related diseases. Further evaluations based on clinical study are expected to clarify these results.

A clinical study of adult human parvovirus B19 infection.

OBJECTIVE: We investigated the clinical aspects of adult human parvovirus (HPV) B19 infection. PATIENTS AND METHODS: We retrospectively reviewed the charts of 21 consecutive patients [4 males, aged 32 to 43 years (average 38.0 years), and 17 females, aged 15 to 43 (average 34.2)] with adult HPV B19 infection who visited our outpatient department between July 1997 and June 1998. All diagnoses of adult HPV B19 infection were based on positive anti-HPV B19 IgM antibody in serum and/or positive HPV B19 DNA in peripheral blood. RESULTS: The predominant signs and symptoms of the patients were: fever (81.0%), arthralgia/myalgia (61.9%), skin rash (47.6%), general fatigue (42.9%), lymph node swelling (38.1%) and edema (38.1%). Six patients had the following underlying diseases or complications: pregnancy, myoma uteri, cervical cancer of the uterus, lupus diathesis/ endometriosis, hereditary spherocytosis, and multiple sclerosis. The following abnormal laboratory findings (more or less than normal limits) were observed: anemia (81.0%), leukopenia (33.3%), elevated transaminases (28.6%), and elevated lactate dehydrogenase (LDH) (57.1%). Six patients were considered to have persistent infection. CONCLUSION: HPV B19 can infect healthy adults and causes more predominant signs and symptoms (arthralgia, myalgia and fever) than in children, and adult HPV B19 infection can be suspected from the familial history and clinical findings. Accordingly, more attention must be paid to adult HPV B19 infection, particularly when erythema infectiosum is prevalent in children.

Adult T-cell leukemia (ATL) cells which express neural cell adhesion molecule (NCAM) and infiltrate into the central nervous system.

We encountered a patient with adult T-cell leukemia/lymphoma (ATL) which expressed neural cell adhesion molecule (NCAM). The tumor cells markedly infiltrated the central nervous system (CNS) during the course of the ATL. The patient died 20 months after disease onset, which was considered to be early in the course. During the invasion of the CNS, the surface phenotype of the peripheral blood ATL cells by flow cytometric analysis was CD2+, CD3+, CD4+, CD7-, CD8-, CD16-, NCAM (CD56)+, HLA-DR-. We speculate that the infiltration of ATL cells into the CNS was closely related to the expression of the NCAM in this patient.