Clinical course of human T-lymphotropic virus type I carriers with molecularly detectable monoclonal proliferation of T lymphocytes: defining a low- and high-risk population

To characterize the prodromal phase of adult T-cell leukemia (ATL), a prospective follow-up study was conducted on 50 carriers in a putative pre-ATL state. This state was defined by the presence of molecularly- detectable monoclonal proliferation of human T-lymphotropic virus type I (HTLV-I)-infected T lymphocytes, and the absence of clinical symptoms of leukemia. The median observation time was 50 months. The pre-ATL subjects were divided into two groups according to initial white blood cell (WBC) counts: group A, those with a normal WBC count (9,000/microL) (n = 30), and group B, those with an increased WBC count (9,000 to 15,000) (n = 20). Comparisons were made between the two groups and with a group of 25 patients with chronic ATL (group C) who had WBC counts of more than 15,000. Significant differences in survival rate were found between groups A and B (10-year survival 65.7%) and group C (32.8%) (P < .01), and between group A (10-year survival 90.0%) and group B (52.1%) (P < .05). The incidence of transformation to overt ATL was 10% (3 of 30) in group A and 50% (10 of 20) in group B (P < .01). In six transformed cases (one in A and five in B) we found exactly the same integration sites in pre-ATL and overt ATL phases, confirming the multistep leukemogenesis hypothesized for this disease. However, the pre-ATL subjects could be divided into two distinct prognostic groups based on the initial WBC count; those with good and those with poor prognosis. Although the 10% transformation rate (2.5% annually) in group A seemed to be extremely high compared with that in the general population of HTLV-I carriers (around 0.06% to 0.4% annually), the majority of group A subjects and some in group B showed stable clinical courses without transformation. Further, development of ATL was not observed in four group A subjects with HTLV-I-associated myelopathy (HAM), which is rarely associated with ATL. We propose to call this group of rather benign HTLV-I carriers "HTLV-I carriers with monoclonal proliferation of T lymphocytes (HCMPT)." Thus far we have been unable to identify reliable parameters other than WBC counts that prospectively distinguish HCMPT from the true pre-ATL state, in which there is a high probability of developing ATL. Further clinical and biologic approaches should elucidate the natural history of the HTLV-I carrier state and early events in ATL leukemogenesis.     

Ligation of eustachian tube for intractable patulous eustachian tube: a preliminary report

Pharyngeal orifice of the eustachian tube was ligated on ten patients, 15 ears with intractable patulous eustachian tube. While the eustachian tube orifice was observed by an endoscope inserted through the contralateral nostril, the orifice was ligated transnasally and/or transorally using instruments usually used in the endoscopic nasal surgery. Now 13–27 months after the surgery, the outcome was excellent (both symptoms and sonotubometry were normalized) in two ears, good (either symptoms or sonotubometry was improved) in seven ears, and unchanged in the remaining six ears. In one of the ears with an outcome of unchanged, the ligation was found to be spontaneously released soon after surgery, but the symptom was improved after the second operation 2.5 months after the first operation. Temporary otitis media with effusion was seen in one ear, mild inflammation around the ligated site also in one ear, but no other serious complication has been observed. Although further improvement in the surgical procedure and further discussion about its long-term outcome should be required, this procedure appeared to be one of the therapeutic options for intractable patulous eustachian tube.     

The influence of Er. YAG laser application in fenestration to the inner ear

OBJECTIVE: Erbium (Er.) YAG laser may be usable for middle ear surgery because of its ability to ablate bony tissue. We investigated the inner ear damage caused by the fenestration to the inner ear with Er. YAG laser. DESIGN: We investigated the influence of Er. YAG laser on the inner ear using electrophysiological technique. RESULTS: Several cases had a decrease in endocochlear potential (EP) and cochlear microphonics (CM) after the fenestration to the inner ear. CONCLUSIONS: Er. YAG laser is safe if it is used for the small and superficial fenestration to the stapes footplate. However, a few extra pulses after fenestration are dangerous.     

Types and Characteristics of Short-Term Course of Depression after Delivery: Using Zung's Self-Rating Depression Scale

Abstract: In order to clarify the prevalence and clinical features of so-called "maternity blues" and the relationship with depression after delivery, we conducted a prospective study on 220 mothers using the Zung's Self-Rating Depression Scale (ZSDS) 4–7 days after childbirth and again one month after childbirth. Their physical condition during their pregnancy had been evaluated earlier. As in a previous epidemiological study conducted in Japan, we classified subjects with a ZSDS score of 60 and over as a high scoring group. Eighteen mothers (8.18%) fell into this group ("maternity blues" group) in the one week post-partum evaluation. Comparing the ZSDS scores one month after childbirth with the earlier scores, in 10 women (4.5%), their scores decreased from 60 and over to below 60 (a "maternity bines" core group), and in 8 women (3.6%), their scores were still over 60 (a prolonged "maternity blues" group). Eleven (5.0%) out of the 202 women whose scores at one week after childbirth were below 60 showed a high score (60 and over) one month after childbirth (a potential depression group). All in, 191 women scored below 60 in both evaluations. Although we could divide the subjects into 4 groups as described above, we could not find any clinical symptoms to distinguish between the 3 groups with "maternity blues" or depression in the evaluation one week after childbirth. However, one finding suggests that these three groups may have different features.     

Comparative genomic hybridization analysis of Japanese B-cell chronic lymphocytic leukemia: correlation with clinical course

B-cell chronic lymphocytic leukemia (B-CLL) is the most common leukemia in Westerners. By contrast, B-CLL is rare in Asians, including Japanese. We applied comparative genomic hybridization (CGH) to screen 26 newly diagnosed Japanese B-CLL patients for genomic aberrations. Chromosomal imbalances were detected in 12 of the 26 cases (46%). The most frequent changes observed were gains of chromosomes 3q in five cases (19%) and 17q in three cases (12%). Other recurrent imbalances included gains of chromosomes 8q, 18q and losses of chromosomes 13q and 17p. Samples obtained at different sites disclosed identical CGH findings in all of the three cases examined. Genomic imbalances as detected by CGH were associated with disease progression and shorter survival. Two patients, with chromosomal imbalances, including gains of both 3q and 18q, developed large cell transformation of the disease within 4 years. In conclusion, CGH abnormality was associated with poor prognosis in Japanese B-CLL, and features of Japanese B-CLL, compared to chromosomal abnormalities of Western B-CLL in the literature, include a lower incidence of any abnormality in particular regarding gain of 12q, with the exception of a higher incidence of gains at 3q.     

Mutations in the retinoblastoma-related gene RB2/p130 in adult T-cell leukaemia/lymphoma

The retinoblastoma (Rb) family consists of the tumor suppressor Rb/p105 and related proteins p107 and Rb2/p130. Although the involvement of the RB / p105 gene in Adult T-cell leukaemia/lymphoma (ATL) has been studied, no mutational data is reported regarding the RB2 / p130 gene in ATL. We screened for mutations of the RB2 / p130 gene. Mutation was detected in 1 of 41 primary ATL sample. This is the first report describing mutation of the RB2 / p130 gene in ATL, suggesting that RB2/p130 may be involved in the development of ATL, and may behave as a tumor suppressor gene in T lymphocytes.