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An 80 year-old woman was referred for a one-eyed palpebral edema. She had ptosis and oblique diplopia resistant to corticotherapy. An orbit magnetic resonance imaging showed a mass infiltrating the optic nerve, with an enhancement of the whole orbital musculature. Biopsies of the lacrimal gland were positive for immunoglobulin light chain amyloidosis. Debulking surgery is the main treatment modality for symptomatic patients with localized orbital amyloidosis. Diffuse orbital involvement may make excision difficult. We managed to treat her through radiation therapy to allow a surgical procedure, delivering 20Gy in 10 fractions. The main aim of radiation therapy was to eradicate the B cells that produced the amyloid light chain deposition. After radiation therapy, the orbital inflammatory caused by the lymphoid infiltration diminished and corticotherapy could be stopped. The surgery could be performed with excellent clinical results. At 2-year follow-up, neither tumor regrowth nor late complications were detected., (Copyright © 2022 Société française de radiothérapie oncologique (SFRO). Published by Elsevier Masson SAS. All rights reserved.)

Purpose: Localized amyloidosis is a condition characterized by deposits of fibrillary proteins confined to a single organ. The most common subtype is amyloid light chain amyloidosis, which is caused by secretion of amyloidogenic light chain by a monoclonal population of plasma cells. We present a review and discussion of the literature in the context of a case presentation of localized amyloid light chain amyloidosis of the nasopharynx treated with radiation alone., Methods and Materials: We reviewed literature relevant to this topic from 1970 to the present. Relevant studies, reports, and articles were summarized in table form., Results: Surgical resection has historically been the primary therapeutic modality for these patients, with radiation being reserved for recurrent lesions or for those unfit for surgery. Although the data are limited to small retrospective series, radiation has been shown to provide good control with mild toxicity that is as good as or better than surgery. Doses range from 20 to 45 Gy, conventionally fractionated. There is no known risk of progression to systemic disease without local therapy., Conclusions: We recommend local therapy for symptomatic patients after systemic disease has been excluded. We generally recommend radiation in the setting of recurrent lesions, unacceptable toxicity with surgery, poor surgical candidates, and as the initial modality in select patients (elderly individuals with bothersome but nonobstructive lesions)., (Copyright © 2022. Published by Elsevier Inc.)

Amyloidosis is known to be caused by the deposition of amyloid fibrils into various biological tissues; effective treatments for the disease are little established today. An infrared free-electron laser (IR-FEL) is an accelerator-based picosecond-pulse laser having tunable infrared wavelengths. In the current study, the irradiation effect of an IR-FEL was tested on an 11-residue peptide (NFLNCYVSGFH) fibril from β2-microglobulin (β2M) with the aim of applying IR-FELs to amyloidosis therapy. Infrared microspectroscopy (IRM) and scanning electron microscopy showed that a fibril of β2M peptide was clearly dissociated by IR-FEL at 6.1 µm (amide I) accompanied by a decrease of the β-sheet and an increase of the α-helix. No dissociative process was recognized at 6.5 µm (amide II) as well as at 5.0 µm (non-specific wavelength). Equilibrium molecular dynamics simulations indicated that the α-helix can exist stably and the probability of forming interchain hydrogen bonds associated with the internal asparagine residue (N4) is notably reduced compared with other amino acids after the β-sheet is dissociated by amide I specific irradiation. This result implies that N4 plays a key role for recombination of hydrogen bonds in the dissociation of the β2M fibril. In addition, the β-sheet was disrupted at temperatures higher than 340 K while the α-helix did not appear even though the fibril was heated up to 363 K as revealed by IRM. The current study gives solid evidence for the laser-mediated conversion from β-sheet to α-helix in amyloid fibrils at the molecular level., (open access.)

Background: Low-dose radiation therapy (LD-RT) has been shown to decrease amyloidosis or inflammation in systemic diseases and has recently been proposed as possible treatment of Alzheimer's disease (AD). A positive effect of LD-RT on tauopathy, the other marker of AD, has also been suggested. These effects have been shown in preclinical studies, but their mechanisms are still not well understood., Objective: This study aimed to evaluate if anti-amyloid and anti-inflammatory effects of LD-RT can be observed at an early stage of the disease. Its impact on tauopathy and behavioral alterations was also investigated., Methods: The whole brain of 12-month-old 3xTg-AD mice was irradiated with 10 Gy in 5 daily fractions of 2 Gy. Mice underwent behavioral tests before and 8 weeks post treatment. Amyloid load, tauopathy, and neuroinflammation were measured using histology and/or ELISA., Results: Compared with wild-type animals, 3xTg-AD mice showed a moderate amyloid and tau pathology restricted to the hippocampus, a glial reactivity restricted to the proximity of amyloid plaques. LD-RT significantly reduced Aβ42 aggregated forms (-71%) in the hippocampus and tended to reduce other forms in the hippocampus and frontal cortex but did not affect tauopathy or cognitive performance. A trend for neuroinflammation markers reduction was also observed., Conclusion: When applied at an early stage, LD-RT reduced amyloid load and possibly neuroinflammation markers, with no impact on tauopathy. The long-term persistence of these beneficial effects of LD-RT should be evaluated in future studies.

Objectives: Laryngeal amyloidosis (LA) is a rare disease characterized by extracellular protein deposition within the larynx. Treatment is difficult due to the frequently submucosal and multifocal nature of disease. The mainstay of treatment is surgical resection; however, recurrence rates are high. Recently, use of radiotherapy (RT), either alone or postoperatively, for LA has been adapted from the management of extramedullary plasmacytoma and has been shown to provide local disease control. Here, we describe the experience with adjuvant RT for LA at our center., Study Design: Retrospective case series., Methods: Retrospective study of patients with amyloidosis of the larynx, with or without other disease sites, seen at a tertiary academic center between 2011 and 2019. Outcomes included disease characteristics, recurrence rates, treatment modalities, and pre- and posttreatment voice handicap index (VHI)-10., Results: Ten patients met eligibility criteria. Mean follow-up time for all patients was 62.0 ± 41.0 months; mean follow-up time after last treatment was 51 ± 55 months. All but one patient underwent surgical resection of disease. Seven patients underwent subsequent RT. Of these seven, six underwent RT at our institution; five received a dose of 45 Gray (Gy); and one received a dose of 20 Gy. All seven completed RT without toxicity-related interruption. Patients undergoing RT underwent 2.1 ± 1.3 surgical procedures prior to RT; no patients required surgery after RT. Mean pretreatment VHI-10 was 22.9 ± 8.1; mean posttreatment VHI-10 was 12.9 ± 13.3., Conclusion: RT after surgery for LA can provide good local control without unacceptable toxicity and may decrease the need for further surgery., Level of Evidence: 4 Laryngoscope, 131:1810-1815, 2021., (© 2020 American Laryngological, Rhinological and Otological Society Inc, "The Triological Society" and American Laryngological Association (ALA).)

Background: No topical and systemic treatment is proven to be effective on Cutaneous Macular Amyloidosis (CMA). The physical removal of the deposited protein by lasers may be a better choice., Objective: To compare the efficacy of different lasers including Pulsed dye laser (PDL), 1064 mode of Q-Switched Nd-YAG, CO 2 , and combined CO 2 and Q-Switched Nd:YAG lasers for the treatment of CMA., Patients and Method: 17 adult female patients with clinical CMA and histopathological confirmation were included in this study. Four close 1 by 1 cm 2 areas were treated simultaneously with PDL, Q-Switched Nd-YAG, CO 2 and combined CO 2 and Nd-YAG Lasers. The energy used for 585 nm PDL were 9 J/cm 2 with the spot size of 5 mm. The energy used for 1064 nm mode of Nd-YAG laser was 5 Joules/cm 2 , with the spot size of 4 mm, and the frequency of 10 hertz. The energies used for CO 2 laser were 4 and 3.5 Micro Joules for the first and second Passes., Results: No desirable result obtained after PDL therapy. The Q-Switched Nd-YAG was effective in only 3 patients. All areas treated with CO 2 laser were cleared., Conclusion: The CO 2 laser is seems to be the most effective laser treatment of CMA.

Background: Emerging evidence suggests that the gut microbiota plays an important role in the pathological progression of Alzheimer's disease (AD). Photobiomodulation (PBM) therapy is believed to have a positive regulatory effect on the imbalance of certain body functions, including inflammation, immunity, wound healing, nerve repair, and pain. Previous studies have found that the intestinal flora of patients with AD is in an unbalanced state. Therefore, we have proposed the use of gut flora-targeted PBM (gf-targeted PBM) as a method to improve AD in an Aß-induced AD mouse model., Methods: PBM was performed on the abdomen of the mice at the wavelengths of 630 nm, 730 nm, and 850 nm at 100 J/cm 2 for 8 weeks. Morris water maze test, immunofluorescence and proteomic of hippocampus, and intestinal flora detection of fecal were used to evaluate the treatment effects of gf-targeted PBM on AD rats., Results: PBM at all three wavelengths (especially 630 nm and 730 nm) significantly improved learning retention as measured by the Morris water maze. In addition, we found reduced amyloidosis and tau phosphorylation in the hippocampus by immunofluorescence in AD mice. By using a quantitative proteomic analysis of the hippocampus, we found that gf-targeted PBM significantly altered the expression levels of 509 proteins (the same differentially expressed proteins in all three wavelengths of PBM), which involved the pathways of hormone synthesis, phagocytosis, and metabolism. The 16 s rRNA gene sequencing of fecal contents showed that PBM significantly altered the diversity and abundance of intestinal flora. Specifically, PBM treatment reversed the typical increase of Helicobacter and uncultured Bacteroidales and the decrease of Rikenella seen in AD mice., Conclusions: Our data indicate that gf-targeted PBM regulates the diversity of intestinal flora, which may improve damage caused by AD. Gf-targeted PBM has the potential to be a noninvasive microflora regulation method for AD patients., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Solitary extramedullary plasmacytoma (SEP) of the larynx is a rare haematological malignancy and an infrequent cause of persisting dysphonia. We present the case of a 54-year-old woman with a long-standing history of dysphonia. While clinical examination showed a rather inconspicuous prominent right vestibular fold, an MRI revealed a laryngeal mass with erosion of the thyroid cartilage. A biopsy taken during rigid endoscopy demonstrated plasma cell infiltration with light chain restriction amidst amyloid deposits. After exclusion of systemic involvement, the diagnosis of an SEP of the larynx with secondary amyloidosis was made. The patient received primary radiation therapy. Another biopsy taken 3 months after the end of therapy did not show any signs of ongoing neoplastic plasma cell disease. The patient was therefore considered to be in remission. She is currently receiving regular follow-up and has not shown signs of persistent or progressive disease for the past 18 months., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose : To evaluate the effectiveness of low dose external beam radiation therapy to halt progression of localized periocular light chain (AL) amyloidosis, a clonal plasma cell disorder. Methods : This is a retrospective review of patients referred to a tertiary care center for external beam radiation treatment of biopsy proven localized periocular light chain amyloidosis. The primary outcome measure was clinical disease stability at one year following radiation therapy as evidenced by slit lamp exam and external photography. Pre and post radiation MRI imaging of the affected area were also used as a means to monitor disease progression. Results : Four symptomatic patients with localized periocular AL amyloidosis received external beam radiation therapy ranging from 20-30 Gy fractioned over 10-20 fractions. Three of the four patients had prior surgical debulking with or without ptosis repair. Amyloid deposition did not progress in any patient at one year. Further follow-up of two patients revealed amyloid progression at two years post radiation. Conclusions : External beam radiation therapy for localized periocular AL amyloidosis demonstrated efficacy at halting disease progression at one year; however, the long-term efficacy is unknown. Monitoring of periocular amyloid is best achieved with slit lamp exam and external photography as opposed to MRI.

Purpose: Airway amyloidosis is an uncommon condition and may have variable clinical course. There is no proven systemic therapy, and the management mainly relies on local invasive treatments. Several reports have found external beam radiotherapy to produce durable responses. The optimal dose and fractionation has not been determined. Endobronchial brachytherapy (EBB) has never been reported to affect this disease., Methods: The study includes a retrospective chart review of all patients with airway amyloidosis treated with EBB at the Davidoff Cancer Center. Data include symptoms, radiotherapy method and dose, treatment outcomes, and toxicities., Results: Three patients were included. Patients were either symptomatic with extensive airway involvement or have undergone multiple local procedures with rapid recurrence of the amyloid deposits. Two patients had upper and 1 patient had lower airway involvement. Two were treated with external beam radiotherapy and EBB and 1 was treated with EBB only. All patients achieved rapid improvement of symptoms with visible improvement in followup bronchoscopies. EBB doses ranged from 7.5 to 10 Gy. Of the seven lesions treated with EBB, only one lesion recurred 6 months from treatment. No major toxicities were reported., Conclusions: EBB is effective and safe for the management of airway amyloidosis and can offer long-term control. This is the first report of EBB for this indication. EBB should be further explored as means of minimizing the irradiated lung volume., (Copyright © 2018 American Brachytherapy Society. Published by Elsevier Inc. All rights reserved.)

Background: Primary localized amyloidosis is characterized by the deposition of amyloid proteins restricted to one organ, without systemic involvement. Primary nasopharyngeal amyloidosis is an exceedingly rare condition, for which the standard treatment remains unknown. Because of its challenging anatomical position, surgery alone hardly results in complete resection of the localized amyloidosis. Therefore, an interdisciplinary planning board to design optimal treatment is of particular importance., Patient and Methods: A 39-year-old man presented with a several-week history of nasal obstruction and epistaxis. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed the presence of a retro-odontoid nonenhancing soft tissue mass., Results: The endoscopic biopsy demonstrated that the mass was amyloid in nature. An extensive systemic workup revealed an absence of inflammatory process, systemic amyloidosis, or plasma cell dyscrasia. The patient was treated with a combination of surgery and radiotherapy, showing no evidence of recurrence or progression at his 1‑year follow-up., Conclusion: Primary solitary amyloidosis is a rare form of amyloidosis. To the best of our knowledge, this is the first report of a nasopharyngeal amyloidosis case treated with excision and radiation leading to complete remission. Because of the difficulty for surgeons to achieve radical resection with such lesions, radiotherapy proved to be an excellent adjuvant treatment in this case.

Macular amyloidosis typically presents as small, dusky-brown or greyish pigmented macules, the result of altered keratin deposition. Treatment of these hyperpigmented regions with topical and systemic therapies remains challenging, however Q-Switched neodymi- um-doped yttrium aluminum garnet (ND:YAG) laser has proven to be an effective treatment modality to reduce hyperpigmentation. In this case report we investigated the ef cacy of Q-Switched Nd:YAG laser treatment on a 34-year old woman with recalcitrant macular amyloidosis who failed to respond to over-the-counter bleaching creams. The patient was treated with 7 treatment sessions of Q- Switched Nd:YAG laser at one month intervals. According to our photographic analysis and patient self-assessment, the patient ap- peared to improve with each treatment session. Post-recurrence of the lesion after reaction to Triluma ( uocoinolone actetonide 0.01%, hydroquinone 4%, tretinoin 0.05%), the patient has been continuing to respond well to a second round of treatment with Q-switched Nd:YAG laser at 1064 nm. J Drugs Dermatol. 2016;15(11):1456-1458..

Introduction: Amyloidosis is characterized by the deposition of amorphous fibrillar protein (amyloid) in the intercellular or intracellular space. Localized amyloidosis is rare in the head and neck area. In Polish literature of the last decade it has been the subject of case studies., Aim: The goal of the study was to carry out a retrospective analysis of patients treated for amyloidosis in the Department of Otolaryngology and Head and Neck Surgery of the Poznań University of Medical Sciences in the period from 2000 to 2009. Based on the collected clinical material, the authors made an attempt to determine the most frequent anatomical location of amyloid deposits and the most frequently affected organs, the type and duration of symptoms, the therapeutic approach, further diagnostic measures taken and the final outcome of the treatment. The next goal was to assess the number of microlaryngoscopies performed in patients with amyloidosis affecting the larynx as compared to the overall number of such procedures., Material: The detailed analysis included 16 patients with laryngeal amyloidosis., Results: Amyloidosis of the larynx was found in only 0.52% of patients undergoing microlaryngoscopies in the period from 2000 to 2009. In the vast majority of patients (11 of 16), there was no suspicion of amyloidosis in the initial diagnosis. Only the histopathological assessment definitely confirmed the nature of pathological changes in the larynx. Glottis was the most common location of amyloid deposits in the study group. In all cases, additional studies and long-term monitoring of the postoperative course excluded generalized amyloidosis., Conclusions: Laryngeal amyloidosis is very rare, however it should be considered in the differential diagnosis in patients with laryngeal dysfunction. Surgery remains the treatment of choice in most patients, although the attempts of radiotherapy are undertaken. Preserving the normal function of the organ remains the priority.

The aim of the prospective study was to explore the safety and effectiveness of external beam radiation therapy (EBRT) in three patients with biopsy proven primary pulmonary amyloidosis, including two tracheobronchial amyloidosis patients and one primary parenchymal amyloidosis patient. All three patients were treated to 24 Gy in 12 fractions utilizing CT simulation and 3-D planning. All three patients had significant improvement in clinical symptoms, radiological imaging as well as pulmonary function tests. The improvement in the clinical symptoms was evident in 2 days. Toxicities related to EBRT were not observed during the follow-up range from 42 to 54 months. EBRT to 24 Gy was safe and effective in the three patients with primary pulmonary amyloidosis, and resulted in rapid relief of pulmonary symptoms., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Purpose: To evaluate the efficacy of conformal external beam radiotherapy (RT) for local control of progressive airway amyloidosis., Methods and Materials: We conducted a retrospective review of patients with biopsy-proven progressive airway amyloidosis treated with conformal RT between 2000 and 2006 at Boston Medical Center. The patients were evaluated for performance status and pulmonary function, with computed tomography and endoscopy after RT compared with the pretreatment studies. Local control was defined as the lack of progression of airway wall thickening on computed tomography imaging and stable endobronchial deposits by endoscopy., Results: A total of 10 symptomatic airway amyloidosis patients (3 laryngeal and 7 tracheobronchial) received RT to a median total dose of 20 Gy in 10 fractions within 2 weeks. At a median follow-up of 6.7 years (range, 1.5-10.3), 8 of the 10 patients had local control. The remaining 2 patients underwent repeat RT 6 and 8.4 months after initial RT, 1 for persistent bronchial obstruction and 1 for progression of subglottic amyloid disease with subsequent disease control. The Eastern Cooperative Oncology Group performance status improved at a median of 18 months after RT compared with the baseline values, from a median score of 2 to a median of 1 (p = .035). Airflow (forced expiratory volume in 1 second) measurements increased compared with the baseline values at each follow-up evaluation, reaching a 10.7% increase (p = .087) at the last testing (median duration, 64.8 months). Acute toxicity was limited to Grade 1-2 esophagitis, occurring in 40% of patients. No late toxicity was observed., Conclusions: RT prevented progressive amyloid deposition in 8 of 10 patients, resulting in a marginally increased forced expiratory volume in 1 second, and improved functional capacity, without late morbidity., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: Localized amyloidosis of the larynx is a rare entity of unclear etiology. Surgical debulking is the primary treatment modality but often is not curative., Methods and Results: A 41-year-old woman presenting with increasing hoarseness, dysphagia, dyspnea, and weight loss was found to have a submucosal mass in the left false vocal fold. Biopsy of the specimen revealed amyloid. After negative work-up for systemic disease, the patient underwent surgical debulking. Specimens revealed a population of clonal plasma cells demonstrating lambda restriction. The patient was treated with adjuvant external beam radiation to a dose of 45 Gy. At 11 months, the patient's voice, breathing, and swallowing have all improved substantially., Conclusions: Recent pathologic studies suggest that localized amyloidosis of the larynx is caused by a localized, nonmalignant plasma cell disorder. Because full resection is difficult, we recommend a combination of surgery and radiation therapy to cure this disease., (Copyright © 2010 Wiley Periodicals, Inc.)

Tracheobronchial amyloidosis (TBA) is a rare disease. No general consensus exists with regard to its optimal treatment, resulting in a variety of modalities used to manage this condition. In this article, we present a case of TBA treated with external beam radiation therapy with encouraging results. A brief literature review of this rare ailment is also included.

Objective: To describe two patients with localized orbital amyloidosis and the response of their condition to surgical debulking followed by external beam radiotherapy., Design: Retrospective noncomparative interventional case series., Outcome Measures: Stabilization or regression of orbital signs after treatment., Methods: Patients with biopsy-proven diagnosis of localized progressive orbital amyloidosis received 34 and 30 Gy fractionated external beam radiotherapy. The clinical case notes and histopathology for the two patients were reviewed., Results: A 69-year-old man with orbital amyloid deposition in association with localized MALT lymphoma had a marked improvement in orbital signs following surgical debulking and radiotherapy, with no recurrence over two years. A 59-year-old woman with localized orbital amyloidosis showed regression of disease after surgical debulking and radiotherapy, with no evidence of recurrence after six years of follow-up., Conclusion: External beam radiotherapy following surgical debulking appears to halt the progression of localized orbital amyloidosis. Radiotherapy may be used in conjunction with surgical debulking of disease.

AL amyloidosis was diagnosed in 2 patients, women aged 61 and 43 respectively. The first patient, who had a nephrotic syndrome, died soon after diagnosis as the disease appeared to be already widespread. The second patient was still alive at the last follow-up, 17 years after diagnosis, because of effective elimination of her light chains by high-dose chemotherapy. AL amyloidosis is a rare, but severe, systemic disease with high mortality. Its aetiology lies in deregulated plasma cells producing excessive numbers of free immunoglobulin light chains. These light chains are the precursor proteins of amyloid fibrils. Amyloid fibrils are deposited extracellularly in tissue leading to organ dysfunction. Symptomatology is diverse, often non-specific, and generally not very well-known. Therefore, the diagnosis is often delayed for a long time. This is unfortunate, as high-dose chemotherapy targeted at elimination of the precursor protein considerably improves prognosis. However, this type of therapy can only be given in patients with limited and moderately progressive disease.

Background: Tracheobronchial amyloidosis is a rare form of primary amyloidosis. There have been no regimens for treatment of this disease that have proven to be effective. There have been case reports of external beam radiation therapy (EBRT) providing marked improvement in symptoms and the appearance of lesions. We report a series of seven consecutive patients who were treated with EBRT., Methods: All patients with tracheobronchial amyloidosis were identified who had received EBRT at Mayo Clinic, Rochester, MN. A retrospective chart review was performed. Data were collected including symptoms, method of diagnosis, treatments, result of treatments, and side effects., Results: All patients received 20 Gy of radiation in 10 fractions. All patients had a favorable response to treatment ranging from symptom relief to a decrease in the frequency of pulmonary infections and objective improvement in pulmonary function. The time to subjective improvement ranged from 1 month to 1 year from the completion of EBRT. Grade 1 esophagitis developed in four patients, and grade 2 esophagitis developed in one patient. Grade 2 pneumonitis that resolved after 10 days of treatment with antibiotics and corticosteroids developed in one patient. FEV(1) was the most consistently used method of objective follow-up, and three of seven patients showed improvement. Follow-up ranged from 10 to 69 months (median, 40 months). The recurrence of asymptomatic endobronchial narrowing requiring no additional treatment was noted on bronchoscopy in one patient after 17 months., Conclusions: Tracheobronchial amyloidosis has been difficult to treat due to the limitations of treatment, recurrence, and complications. EBRT appears to be safe and can provide symptomatic as well as objective improvement.

Tracheobronchial amyloidosis (TBA) refers to the deposition of localized amyloid deposits within the upper airways. Treatments have historically focused on bronchoscopic techniques including debridement, laser ablation, balloon dilation, and stent placement. Local excisions often prove temporarily effective, with multiple local recurrences and progressive compromise pulmonary function occurring frequently. We present a case of TBA managed with definitive external beam radiation therapy. Eighteen months after moderate-dose radiation, the patient demonstrated improvements in functional status, pulmonary function, bronchoscopic visualization, and CT-based luminal diameters. The literature involving the role of radiation therapy in the treatment of TBA is reviewed.

Objective: To describe otologic manifestations of amyloidosis., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: Case report of a patient with bilateral external auditory meatal stenosis secondary to amyloidosis., Results: The patient is a 60-year-old man who presented with several months' history of bilateral ear blockage and drainage. Physical examination revealed markedly thickened skin in each ear in the area of concha and meatus that was associated with fissuring. The meatus were markedly stenosed. The medial aspect of the external auditory canal and the tympanic membrane were normal. He had partial improvement on medical therapy with topical steroid-containing eardrops and ointment. Past medical history was positive for multiple myeloma. Meatal biopsy was performed. Grossly, the tissue was hypovascular and markedly thickened. Histopathologic examination was consistent with amyloidosis. The patient was continued on topical steroid ointments, frequent ear cleaning, and close follow-up. He was subsequently diagnosed with systemic amyloidosis. A review of the literature was done. Head and neck involvement with amyloidosis is reviewed, with emphasis on otologic manifestations., Conclusion: Otologic involvement with amyloidosis is extremely rare. The pathophysiology may be related to the local factors in the external auditory canal.

Tracheobronchial amyloidosis is characterized by deposits of amyloid in airway walls. No effective treatment is known. We describe a 59-year-old woman who presented with increasing symptoms of airway obstruction due to diffuse deposition of amyloid throughout her tracheobronchial tree. She was treated with external-beam radiation therapy (20 Gy) with marked improvement in her symptoms, effort tolerance, bronchoscopic appearance, and forced expiratory volume in 1 second (1.39 L to 1.97 L [42%]). This improvement was maintained during 21 months of follow-up.

Autologous stem cell transplantation is a reasonable approach for patients younger than 70 years and should be discussed with each patient before instituting alkylating agent therapy. Ideally, it should be done in a clinical trial. Although most patients relapse, it does provide a modest prolongation of survival. The major needs are an improved preparative regimen before transplantation and the removal of myeloma cells and, more importantly, their precursors from the peripheral blood. Conventional allogeneic transplantation is associated with too high a mortality rate at present and cannot be recommended. Efforts must be directed toward reducing transplant-related mortality by T-cell depletion or other means. The preparative regimen must be improved, because most patients relapse after transplantation. The use of dendritic cells and vaccines is an important area of research. The role of autologous transplantation in primary amyloidosis (AL) is indeterminate at present. Longer follow-up evaluation of patients who have undergone transplantation is needed. Patient selection is a critical aspect. Stem-cell transplantation should be performed in a clinical trial for primary AL.

A 67-year-old man presented with localized tracheobronchial amyloidosis involving the distal trachea and the right-sided airways. The disease caused right middle lobe collapse and threatened the right upper and lower lobes. A variety of bronchoscopic methods, including Nd:YAG laser resection, dilation, and stenting, were used as temporizing methods. External beam radiation therapy, considered because of disease progression, caused a measurable local response. Radiation therapy should be considered as a treatment option for localized tracheobronchial amyloidosis causing airway obstruction.

We retrospectively investigated the feasibility and the toxicity of autologous stem cell transplantation (ASCT) in 21 cases of systemic amyloidosis (AL). The conditioning regimens consisted of high-dose melphalan (HDM) alone (n = 18) or in combination with 12 Gy total body irradiation (n = 3). Toxic death rate was high: 9/21 patients (43%) died within the first month following ASCT, and 10/12 surviving patients achieved a response. With a median follow-up of 14 months, the OS and the EFS rates at 4 years were 57.1% (+/-10.8) and 29.9% (+/-14.5) respectively for the whole group. The major prognostic factor for both response and survival was the number of clinical manifestations at the time of ASCT, of the following five criteria, i.e. creatinine clearance < 30 ml/min, nephrotic syndrome with urinary protein excretion > 3000 mg/24 h, congestive heart failure, neuropathy, or hepatomegaly associated with alkaline phosphatase level > 200 IU/l. For patients presenting with two or more clinical manifestations the 4-year OS and EFS were both 11.1% compared with 91.7% and 46.3% respectively in patients with fewer than two clinical manifestations at the time of ACST. We conclude that ASCT is feasible in AL in a subset of patients with fewer than two clinical manifestations at the time of ASCT. Given the severe extra-haematological toxicity, ASCT should not be considered in other cases.

Many questions about amyloidosis are still unanswered. Etiology and pathogenesis remain unclear. Diagnosis is difficult to prove without a biopsy. Several approaches have been tried, but no effective cure has been documented. Russian authors report the use of cobalt therapy for a patient with local laryngeal amyloidosis. This paper presents a case report of a patient with local amyloidosis of the conjunctiva. Superficial radiation therapy was used, and resulted in some shrinkage of the lesions. Surgical excision was later utilized to remove the remaining amyloid deposits.

An example of localized amyloid deposition in the breast of an elderly woman is reported. Fine needle aspiration biopsy yielded clumps of amorphous material, which stained violet with the May-Grünwald-Giemsa technique. The amyloid appeared to be of the AA type on histochemical analysis. The lesion was clinically associated, probably coincidentally, with a stage II carcinoma of the cervix. The nature and classification of amyloid is briefly reviewed, and the role of fine needle aspiration cytology in its diagnosis is discussed.

Macular amyloidosis, a variant of lichen amyloidosus, is an uncommon cutaneous eruption that presents with moderate to severe pruritus associated with areas of hyperpigmentation. Effective modalities for the treatment of this condition have been lacking. A case of macular amyloidosis is reported in which successful management of pruritus was obtained using ultraviolet B (UVB) therapy. Mechanisms of UVB action in this condition are discussed.

Tracheobronchial amyloid is uncommon and has a high morbidity and mortality. In the past, progressive airways obstruction has been treated with bronchoscopic resection but benefit has been limited. We report the successful use of a laser to treat this condition and the responses of amyloid tissue to laser photoirradiation.

Primary amyloidosis is a rare disease, and even more so when it appears as a tumorlike formation in the lung or tracheobronchial tree. On rare occasions it has been treated by pulmonary resection but never before by tracheal or bronchial sleeve resection. A case of tracheal sleeve resection for tumorlike amyloidosis is reported. If the lesion is well circumscribed to a relatively short tracheal or bronchial segment, sleeve resection appears to be a satisfactory and economical solution. However, the problem of a mucosa undermined by amyloid deposits above and below the tumorlike lesion entails the risk of anastomotic stenosis. For this reason in future cases, particularly those with extensive involvement, piecemeal endoscopic removal should be considered as the method offering the best prognosis and least risk.