Extramedullary relapse in RARA rearrangement-negative acute promyelocytic leukemia successfully treated in combination with chemotherapy, local radiotherapy, and cord blood transplantation.

RARA rearrangement-negative acute promyelocytic leukemia (APL) is uncommon, and its extramedullary relapse is extremely rare. We report a 5-year-old girl with RARA rearrangement-negative APL, which recurred solely at the external auditory canal and mastoid air cells. She was successfully treated with chemotherapy, local radiotherapy, and unrelated cord blood transplantation. She has maintained complete remission for 24 months after transplantation. The clinical features and our therapeutic strategy in this patient will provide valuable information for extramedullary relapse of RARA rearrangement-negative APL.

[A middle to long-term clinical study on pars flaccida and pars tensa cholesteatoma using survival analysis].

The current clinical study was performed on 311 cases of pars flaccida and 89 cases of pars tensa cholesteatoma which were treated with canal wall reconstructed tympanoplasty between 1991 and 2012. The average follow-up time of these patients was 5.3 years. Since follow-up periods were different in each case and some censored patients were involved, we used survival analysis on this study to discuss the cumulative rates of disease-free successful cases and the rates of recurrent cholesteatoma throughout the postoperative course. The disease-free successful cases were defined as those cases in which patients were both out of re-operation with recurrent and residual cholesteatoma and out of revision operation with another problem, furthermore, maintained good hearing outcome. Based on the criteria set by the Japan Otological Society (2010), the cases that satisfied the following were evaluated as good hearing results; (a) a successful case in which preoperative bone conduction was used, and (b) a case in which the postoperative air-bone gap was within 20dB after tympanoplasty for chronic otitis media. The analysis results were shown for each of (a) and (b). 1. In pars flaccida cholesteatoma, the 5-year survival rate of successful case was (a) 76.1% and (b) 83.9%, the 10-year survival rate was (a) 58.9% and (b) 73.0%. In pars tensa cholesteatoma, the 5-year survival rate of successful cases was (a) 57.7% and (b) 63.5%, the 10-year rate was (a) 42.1% and (b) 56.9%. A significant difference was seen between pars flaccida and pars tensa cholesteatoma (p < 0.001). 2. In pars flaccida cholesteatoma, the 5-year recurrence rate was 7.6% and the 10-year rate was 15.3%, and the recurrence rate increased gradually throughout the follow-up period. On the other hand, in pars tensa cholesteatoma, the increase in the recurrence rate reached a peak 15.8% at 5.5 years after the surgery. A long-term follow-up is necessary when evaluating the clinical results after tympanoplasty.

High frequency of Alloiococcus otitidis in the nasopharynx and in the middle ear cavity of otitis-prone children.

OBJECTIVE: Colonization of middle ear pathogens is facilitated in the nasopharynx of otitis-prone children, and is associated with the development of otitis media. Recently, a new species of bacterium, Alloiococcus otitidis, is considered as one of the major middle ear pathogens. However, as far as we know, no study has been reported concerning the prevalence of A. otitidis in the nasopharynx of otitis-prone children. And, no study has been conducted on the association of A. otitidis in the nasopharynx with otitis media. METHODS: The frequency of A. otitidis in 83 middle ear effusions (MEE) and 56 nasopharyngeal swab (NPS) specimens from 56 children with otitis media was investigated by culture and polymerase chain reaction (PCR). RESULTS: A. otitidis was detected in 24 (28.9%) of MEE and in 6 (10.7%) of NPS specimens. When the frequency was investigated in relation to proneness to otitis media, A. otitidis was detected in 16 (64%) of 25 MEE and in 5 (29.4%) of 17 NPS specimens from otitis-prone children, whereas it was detected in 8 (13.8%) of 58 MEE and in 1 (2.6%) of 39 NPS specimens from non-otitis-prone children. The frequency of A. otitidis in both NPS and MEE specimens was significantly higher in otitis-prone than in non-otitis-prone children. CONCLUSIONS: Our results suggest that colonization of A. otitidis is facilitated in the nasopharynx of otitis-prone children. And, nasopharyngeal colonization of A. otitidis may be associated with the frequency of otitis media.

Colonization and turnover of Streptococcus pneumoniae, Haemophilus influenzae, and Moraxella catarrhalis in otitis-prone children.

Recurrent otitis media are frequently intractable during childhood. It is unclear whether recurrent otitis media is caused by etiological bacteria colonization or by new infections. Streptococcus pneumoniae, Haemophilus influenzae and Moraxella catarrhalis were isolated from the nasopharynx of 7 otitisprone and 2 non-prone children with recurrent otitis media. Plural bacterial species and strains were found in all children while affected by otitis media. The same strain was repeatedly isolated from all otitisprone children even after administration of antibiotics but was not from the non-prone children. Antibiotic susceptibility did not differ significantly among the same repeatedly isolated strains. This pilot study suggests that the etiological bacteria tend to colonize and is hard to eliminate in otitis-prone children.