Kiyoshige Niina

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.Int J Hematol. 2003;78:248-250.

Hair mercury value and fish-eating habit.

Mercury levels in the hair segment close to the scalp were well correlated with fish‐eating habits for residents on a small island in the south of Japanese mainlands. Mercury levels changed in segmented hair samples along with the distance from the scalp. The time of sprouting of hair segment in which the mercury level showed the peak coincided well with the peak of catch of fish on the island.

Type IIa hyperlipoproteinemia masquerading as cerebrotendinous xanthomatosis

We describe an adult patient with type IIa hyperlipoproteinemia, presenting with Achilles tendon xanthomas, cataracts, dementia, ataxia, pyramidal tract signs, and peripheral neuropathy, which are commonly seen in cerebrotendinous xanthomatosis (CTX). However, the diagnosis of CTX was excluded on the basis of the cholestanol level and the normal cholestanol/cholesterol ratio in his serum and tendon. The pathomechanism for some of the clinical manifestations in type IIa hyperlipoproteinemia and CTX might be caused by a common biochemical disturbance.

Adult T-cell leukemia predominantly involving exocrine glands.

Abstract:  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 Vβ of parotid gland‐infiltrating T cells revealed a relatively restricted but not single usage of TCR Vβ. Immunohistochemical analyses of parotid gland specimens detected only a small number of TCR Vαβ‐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 Vβ mRNA transcripts and TCR Vαβ 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.