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Complete remissions in psoralen and UV-A (PUVA)-refractory mycosis fungoides-type cutaneous T-cell lymphoma with combined interferon alfa and PUVA.

UNLABELLED: BACKGROUND AND DESIGN--The optimal therapy for the mycosis fungoides type of cutaneous T-cell lymphoma has yet to be determined. Based on recent reports on the efficacy of high-dose interferon alfa in cutaneous T-cell lymphoma, we chose to test the hypothesis that systemic adjunctive therapy with low-dose interferon alfa along with psoralen and long-wave UV-A radiation (PUVA) could decrease the amount of PUVA necessary to achieve the best response, as well as improve the therapeutic effect of PUVA. Five patients with cutaneous T-cell lymphoma (mycosis fungoides type) were initially treated unsuccessfully with PUVA alone (three times a week for at least 3 months); PUVA was stopped, patients' diseases were re-staged, then interferon alfa (3 x 10(6) U/d for 1 week, then 6 x 10(6) U/d for 1 week) was initiated 2 weeks prior to restarting PUVA. Responses to treatment were graded as complete remission (skin clear, or negative biopsy specimens of remaining dermatitic lesions), partial remission (> 50% improvement), progression (> 25% worsening), or no change (failure to qualify for other categories) at 4, 8, 12, and 16 weeks. OBSERVATIONS--After 3 to 5 months of treatment with PUVA thrice weekly, none of the patients treated with PUVA alone had achieved complete remission. By contrast, systemic adjunctive therapy with low-dose interferon alfa along with PUVA resulted in complete remissions in all five (100%) patients in an average of 3.2 months (in three patients, lesions were cleared by 3 months). Average of 107 additional joules (68% more) and 1.6 additional months of PUVA administered thrice weekly was required to achieve the best response attainable with PUVA alone compared with complete remissions achieved with combined interferon alfa and PUVA therapy. CONCLUSION: --These data indicate that patients with disease refractory to PUVA alone can achieve a complete remission if they restart treatment with PUVA in combination with well-tolerated low doses of interferon alfa. In addition, the duration of PUVA exposure can be reduced with the addition of low-dose interferon alfa to the conventional PUVA treatment of cutaneous T-cell lymphoma of the mycosis fungoides type in such patients.

Adult↗

[A case of mycosis fungoides with pulmonary involvement: effect of etoposide and prednisolone].

Persistent coughing and bloody sputum developed in a 75-year-old man with mycosis fungoides. Chest X-ray films on admission showed combined patterns of alveolar and interstitial shadows in both lung fields. Histological examination of the transbronchial lung biopsy specimen revealed patchy infiltration of mycosis fungoides cells in alveolar septa. VEPA chemotherapy was ineffective against skin lesions and caused bacterial pneumonia, but combined treatment with etoposide (200 mg, once a week) and prednisolone (30 mg, 3 days per week) resulted in remission. The patient has been taking oral etoposide and prednisolone regularly, and has been free of skin and pulmonary lesions for two years. Combined therapy with low-dose etoposide and prednisolone may be worth trying in cases of advanced mycosis fungoides with pulmonary involvement.

Aged↗

Pilotropic cutaneous T-cell lymphoma without mucinosis. A variant of mycosis fungoides? French Study Group of Cutaneous Lymphomas.

BACKGROUND: In the course of mycosis fungoides, pilofollicular manifestations without mucinosis (papules, keratoses, comedones, or epidermal cysts) are rare (15 cases reported). Therefore, histological and clinical diagnoses may be difficult. The clinical course and histopathological and immunohistochemical findings in 9 patients are described. OBSERVATIONS: Pilofollicular lesions were present at the onset (n = 3), before (n = 3), or during a relapse of mycosis fungoides (n = 3). Comedones and epidermal cysts were most frequent (n = 5). They disappeared with lymphoma therapy (n = 4), therapy with isotretinoin (n = 3), or spontaneously (n = 1), or they persisted (n = 1). Clues to the histopathological diagnosis consisted of pilotropism of the infiltrate with minor alteration of the hair follicle walls. The infiltrate was monomorphous and composed of sezariform CD4+ lymphocytes. Pilotropic or peripilofollicular infiltrates, or the absence of infiltrate, were detected in consecutive biopsy specimens obtained from the same patient. The keratinocyte expression of intercellular adhesion molecule type 1 was observed in the hair follicle bulb in front of the pilotropic infiltrate but not in the epidermis. No staining was observed in biopsy specimens of 6 of 7 patients with follicular mucinosis, of folliculitis lesions, or of normal hair follicles. CONCLUSIONS: Our findings indicate the role of adhesion molecules in pilotropism leading to mechanical obstruction of the follicle by tumoral cells followed by hyperkeratosis and cyst formation. It remains to be determined if the expression of intercellular adhesion molecule type 1 is the cause or the consequence of pilotropism. By becoming more aware of it, this variant of mycosis fungoides is probably not so rare.

Adult↗

[Hypopigmented mycosis fungoides].

INTRODUCTION: Isolated areas of hypopigmented skin can be a manifestation of mycosis fungoides. This is a rare clinical form with a particular clinical course and histology which has been described only in black subjects. CASE REPORT: Over a 10-year period, a 28-year-old woman from Mali developed non-infiltrated hypopigmented and discretely squamous macules involving the entire body. The diagnosis of mycosis fungoides was made on pathology evidence of epidermotropic lymphocyte infiltration with a few small nests. PUVA led to rapid regression of the lesions. DISCUSSION: Hypopigmented mycosis fungoides has been described in young black subjects (mean age 19 years). The diagnosis is usually made late because benign skin affection is usually the first diagnosis (pityriasis alba, pityriasis versicolor). The pathogenesis of these hypopigmented lesions is not known. PUVA appears to be effective in most cases.

Adult↗

[Macrophage activation syndrome disclosing leukemic transformation of mycosis fungoides].

BACKGROUND: Mycosis fungoides can mimic pigmented purpuric dermatitis. We report such a case which progressed to peripheral T-cell lymphoma; progression was revealed by reactive hemophagocytic syndrome (RHS). CASE REPORT: A 65-year old male patient was hospitalized for a pigmented and purpuric eruption. The skin lesions appeared 2 years earlier and at that time biopsy had shown pigmented and purpuric dermatitis. One month before hospitalization, general signs appeared. On admission, he had papular and purpuric rash, mainly on the trunk, hepatosplenomegaly, enlarged axillar and inguinal lymph nodes, and fever at 38.2 degrees. A skin biopsy showed histologic changes typical of mycosis fungoides. He also had bicytopenia, hepatitis, and increased triglyceride and ferritin levels suggesting RHS which was proved by means of bone marrow biopsy. These tests also evidenced peripheral T-cell lymphoma. The patient was treated with two courses of chemotherapy (CHOP) but the disease progressed and he deceased. DISCUSSION: Mycosis fungoides can occasionally begin with an eruption very closely resembling pigmented purpuric dermatitis. Therefore, repeated biopsies should be done in case of widespread permanent pigmented purpuric dermatitis of no apparent origin. RHS is a life-threatening disease. The diagnosis should be suspected in any cytopenic patient with fever, increased triglyceride levels and abnormal liver tests. A search for an etiology must then be undertaken a prompt treatment is needed.

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The changing survival of patients with mycosis fungoides: a population-based assessment of trends in the United States.

BACKGROUND: The authors sought to evaluate trends in survival among a population-based group of patients with mycosis fungoides, in response to informal evidence of improved prognosis and concerns about the influence of detection bias on incidence data. METHODS: Data used in this study were drawn from the Surveillance, Epidemiology, and End Results Program of the National Cancer Institute, which includes 9 population-based cancer registries. Together, these registries cover approximately 10% of the U.S. population and have tracked the survival of all mycosis fungoides patients who were registered during the years 1973-1992. Data included follow-up through 1994. RESULTS: There was a total of over 10,000 person-years of follow-up for the 1633 patients studied. Relative survival changed little after 11 years, at which point it was 66%. Advanced age and black race were associated with poorer survival. The prognoses of Asian and Hispanic patients were slightly but not significantly worse than those of whites, and there were no significant geographic differences related to prognosis. The survival of married women was superior to that of other gender and marital-status groups. Prognosis did improve substantially over the 20-year period of study. CONCLUSIONS: Mycosis fungoides is usually not a fatal disease, although the mortality risk is substantial. The authors quantified various prognostic factors and documented an improved prognosis over the 20-year period of study. The data from this study raise concern about possible detection bias in incidence data. These data are also consistent with the concept that beyond 11 years after diagnosis, this disorder has relatively little impact on the risk of death.

Aged↗

A pathological study of bovine alimentary mycosis.

A 10-year study showed that 45 of 692 cattle aged > 6 months had systemic mycosis and, of these, 38 had alimentary mycosis. The percentages of these 38 cattle with lesions in various sites were: rumen (73.7), omasum (71.1), abomasum (34.2), reticulum (21.1), intestine (15.8), omasoabomasal orifice (7.9) and tongue (2.6). Infections in both the forestomachs and abomasum occurred in 31.6 per cent; mucormycosis was found in 94.7 per cent and aspergillosis in 31.6 per cent; 26.3 per cent (10 cattle) had complicated infections with both Mucorales and Aspergillus and eight of these animals had mixed lesions of both mucormycosis and aspergillosis. Macroscopically, focal haemorrhagic necrosis was seen in all 38 cattle. Histologically, thrombosis, coagulative necrosis and hyphae typical of the order Mucorales or of Aspergillus spp. were found. Many of the cattle were also affected by ruminal acidosis-inducing factors (ruminal atony 81.6 per cent, mastitis 63.2 per cent, "downer cow syndrome" 57.9 per cent and parturition 50.0 per cent), which may have predisposed to alimentary mycosis.

Animals↗

Effect of exposure method to Beauveria bassiana and conidia concentration on mortality, mycosis, and sporulation in cadavers of Chilo partellus (Lepidoptera: Pyralidae).

The effects of exposure methods, conidial concentrations, and temperature on mortality, mycosis and sporulation in second instar Chilo partellus cadavers infected by Beauveria bassiana was investigated in laboratory studies. Larvae directly sprayed with conidia, exposed to conidia-treated leaves, and dipped into conidial suspension resulted in high mortality (98-100%). The longest LT50 (3.5 days) and days to mortality (2.6 days) were observed in the treated-leaves exposure method. The shortest LT50 (1 day) and days to mortality (1 day) were recorded for the dipping method. With increasing conidial concentrations, there were decreasing LT50 and days to mortality. Larvae exposed to treated leaves and larvae directly sprayed with conidial suspensions produced high mycoses in cadavers. Exposure of larvae to treated-leaves resulted in high sporulation. At lower concentrations of conidia, both mycosis and sporulation in cadavers were high. The optimum temperature for mycosis was 20 and 15 degrees C for sporulation.

Animals↗

[Prevalence and clinical aspects of superficial mycosis in hospitalized diabetic patients in Tunisia].

OBJECTIVES: The prevalence of mycotic infection seems to be higher among diabetic patients than in the non-diabetic population. The aims of this study were to determine the frequency of mycosis and to compare clinical and fungal results. PATIENTS AND METHODS: This transversal study included 307 type 1 and 2 diabetic patients admitted between January 1998 and January 2000. A dermatologist examined all patients. The mean age was 44 +/- 17 years and the duration of diabetes 8 +/- 6 years. Patients with suspected lesions underwent mycological examination. RESULTS: Clinical signs of presumed fungal infection were found in 61% of patients, but mycosis was confirmed only in 30%. Fungal foot infection accounted 38% of the patients, mostly due to dermatophytes (94%). The commonest localizations of dermatophytes were interdigital (60%) followed by onychomycosis (30%). The main fungal agent was Trichophyton rubrum. The main risk factors for fungal infections were the age of patients (P = 0.0003) and duration of diabetes (P < 0.05). Interdigital foot localization of dermatophytes was correlated to age (P < 0.0001) and to the male gender (P < 0.01). The frequency of dermatophytes in nails was higher in type 2 diabetic patients (P < 0.01). Vulvovaginal candidosis and interdigital dermatophytes were more frequent in obese than in non-obese patients. The accuracy and specificity of direct examination were respectively 85% and 79%. CONCLUSION: The high frequency of mycosis in diabetic patients at hospital is demonstrated. The main risk factors were age, male gender and obesity.

Adult↗

Endoscopic aspects of mycosis in the upper digestive tract.

In an evaluation of 2537 endoscopic investigations of the upper digestive tract, yeast-like fungi were found in cultures from 53 purposive brushings (2.1%) obtained from visible mycotic areas. In 46 cases the esophagus only was affected. In the stomach and surrounding the anastomosis after resection, we found 6 positive results. Once, findings were seen in the esophagus as well as in the stomach. Determination of the yeast-like fungi revealed Candida albicans in 43 cases. Other fungi were cultured 18 times, among them Torulopsis glabrata 3 times. In every mycosis culture it is useful to determine the species of yeast-like fungi and the drug sensitivity. Every mycosis should be assessed endoscopically with regard to its degree of severity. Endoscopy is a very important method for the diagnosis of gastrointestinal mycosis. The investigation of more easily obtainable specimens such as throat swabs or feces, may often be of no use.

Candida↗

Systemic mycosis due to Aspergillus deflectus in a dog.

A 4-year-old, entire female, German Shepherd Dog was referred with a 3-month history of right foreleg lameness that partially responded to nonsteroidal anti-inflammatory and antimicrobial therapy. The bitch lost weight, was polydipsic and had reduced exercise tolerance. On referral, the animal was in poor condition, pyrexic and exhibited moderate pain on full extension of the right shoulder. Blood, urine and joint fluid were obtained and radiographs were taken of the right shoulder and chest. The bitch was lymphopaenic, hyperfibrinogenaemic, hyperglobulinaemic, mildly azotaemic, mildly proteinuric and isosthenuric. Branching fungal hyphae were present in the urine. On radiography, the thorax contained a large ventral mediastinal mass and the humeral head had extensive areas of radiolucency. An aspirate from the right humeroscapular joint exhibited branched fungal hyphae and numerous neutrophils and macrophages. A diagnosis of disseminated mycosis was made and euthanasia was performed. At necropsy, numerous caseating granulomas were present, especially in the kidneys, adrenal glands, heart and lymph nodes. Extensive osteomyelitis involved the head of the right humerus, the sternebrae and the fifth intervertebral disc. Fungal hyphae were detected in sections of granulomas in all affected organs and a diagnosis of disseminated fungal granulomatosis was made. Aspergillus deflectus was readily isolated from affected lymph nodes, but confirming its identity as A deflectus using standard procedures proved difficult. The identity of the fungus was finally confirmed by sequencing part of the 185 rRNA of the isolate. This is the first report in Australia of a disseminated mycosis caused by A deflectus. Previously, the involvement of A deflectus as a cause of disseminated mycosis was limited to 5 cases from the West Coast of the USA, four of which occurred in German Shepherd Dogs.

Animals↗

Outcome of treatment in 35 cases of guttural pouch mycosis.

This paper describes the outcome of treatment of 30 cases of guttural pouch mycosis by ligation of the internal carotid artery on the cardiac side of the lesion and lavage of the affected pouch with natamycin. Twenty-three horses recovered fully following this treatment while laryngeal hemiplegia persisted in one case and slight dysphagia caused by pharyngeal hemiplegia in another. The remaining five horses died or were destroyed. Five horses with guttural pouch mycosis, which had shown no epistaxis but had pharyngeal hemiplegia, were treated by topical natamycin alone. Only two of these survived, of which one remained slightly dysphagic. Ligation of the internal carotid artery of the cardiac side of the lesion is an effective means of reducing the chance of fatal epistaxis in cases of guttural pouch mycosis. Some cases of pharyngeal hemiplegia can make a complete recovery although it may take 12 to 18 months.

Administration, Topical↗

Non-aspergillus allergic bronchopulmonary mycosis in a pediatric patient with cystic fibrosis.

This article describes a child with cystic fibrosis (CF) and allergic bronchopulmonary mycosis caused by Tricosporon beigelii. An 11-year-old boy with CF failed to respond to conventional treatment for a pulmonary exacerbation. Bronchial washings contained copious budding yeast forms, subsequently identified as T beigelii. Total serum immunoglobulin E was elevated and precipitating antibodies to T beigelii were positive. Together these findings support the diagnosis of allergic bronchopulmonary mycosis. The patient improved with antifungal therapy and systemic glucocorticoid therapy. The pathologic potential of yeast in the airways of patients with CF is unclear. The diagnosis of non-Aspergillus allergic bronchopulmonary mycosis requires a high degree of suspicion and has potentially important implications for the management of patients with CF.

Bronchial Diseases↗

Progression of mycosis of the auditory tube diverticulum (guttural pouch) after arterial occlusion in a horse with contralateral temporohyoid osteoarthropathy.

CASE DESCRIPTION: A 6-year-old Appaloosa mare was examined because of inappetance, difficulty eating, and swelling and mucopurulent discharge in the right eye. CLINICAL FINDINGS: Results of a CBC and serum bio-chemical analysis revealed no important findings. Ophthalmologic examination revealed scarring and ulceration of the superficial layers of the cornea. Endoscopic examination of the upper portion of the respiratory tract and auditory tube diverticula (guttural pouches) revealed abnormal thickness of the right stylohyoid bone and a plaque suggestive of mycotic growth on the left internal carotid artery. Radiographic examination revealed right-sided otitis media. Temporohyoid osteoarthropathy in the right guttural pouch and mycosis in the left guttural pouch were diagnosed. TREATMENT AND OUTCOME: Ceratohyoidectomy of the right stylohyoid bone was performed, and the left internal carotid artery was occluded via placement of stainless steel spring embolization coils. The mare regained the ability to eat without difficulty and improved clinically for approximately 4 weeks. However, the mare returned to the medical center 53 days after surgery with left-sided Horner syndrome, atrophy of the right side of the tongue, and a 3-week history of dysphagia and weight loss. Endoscopic evaluation revealed progression of mycotic growth in the left guttural pouch. The mare was euthanatized. CLINICAL RELEVANCE: Although the mycotic lesion in the left guttural pouch was an incidental finding at the time of initial examination, the lesion progressed to cause dysphagia and Horner syndrome after occlusion of the left internal carotid artery, a treatment that is typically associated with resolution of guttural pouch mycosis. Arterial occlusion is not necessarily a reliable method of resolving guttural pouch mycosis.

Animals↗

Transarterial coil embolisation in 31 horses (1999-2002) with guttural pouch mycosis: a 2-year follow-up.

REASONS FOR PERFORMING STUDY: There have been no previously published large case series describing short- and long-term outcome of transarterial coil embolisation (TCE) in horses with guttural pouch mycosis (GPM). OBJECTIVES: To describe the clinical and surgical features of horses with GPM presented at the Veterinary School of Lyon during a 28 month period; and to evaluate immediate to long-term results of TCE as a treatment. METHODS: Medical records of all horses with GPM treated with TCE between February 1999 and July 2002 were analysed. To be included in the study, no other surgical or medical treatment for the mycosis was administered. Subject details, case history and results of initial clinical examination and endoscopy were reviewed. For all individuals, evaluation of long-term complications and case evolution was based on owner or trainer interviews between 24 and 41 months after surgery. RESULTS: Thirty-one horses were identified with unilateral (n = 25) or bilateral (n = 6) GPM affecting only the medial (n = 28), only the lateral (n = 2) or both compartments simultaneously (n = 7). Of the 23 individuals presented with epistaxis, 20 showed complete resolution of the problem. Of the 19 horses presented with neurological signs, 2 were subjected to euthanasia for persistence of severe dysphagia. Prognosis for survival was excellent (84%) and prognosis for return at the level expected by the owner or trainer was good (71%). CONCLUSIONS AND POTENTIAL RELEVANCE: TCE of horses affected with GPM is an effective method of preventing haemorrhage and resolving the majority of mycotic lesions without further specific treatment. Treated horses did not present relapse of mycosis during the follow-up period.

Angiography↗

[Severe mycosis-factors with fungus and host].

The deep-seated mycosis occurred in immunocompromized patients. Normally, the deep-seated mycosis became sever infection because of the defects of host defense or the adverse effects of antifungal agents. The major factors of the reason on the severity of the deep-seated mycosis depends on the pathogenesity and the drug resistance for antifungal agents in infected fungi. The clinical factors related with hosts defenses are important to the other reason on the severity. We investigated that the multiple drug resistant (MDR) mechanism may be one of the major roles plays in the azole resistant Candida albicans strains isolated form the patients with oropharyngeal candidiasis infected HIV. We analyzed which clinical factors are related with the prognosis of the patients with pulmonary cryptococcosis and aspergilloma. The titer of cryptococcal capsular antigen was earlier improve in the patient without underline disease than in the patients with underline diseases diagnosed pulmonary cryptococcosis. CRP was higher in the death cases in the patients with pulmonary aspergilloma, compared with alive cases.

AIDS-Related Opportunistic Infections↗

[Treatment of mycosis sphenoiditis with endoscopic sinus surgery].

OBJECTIVE: To evaluate the treatment effect of endoscopic sinus surgery for mycosis sphenoiditis. METHOD: From 1995 to 1998, 9 patients of mycosis sphenoiditis were treated with endoscopic sinus surgery. RESULT: In the follow-up of 6-36 months, all the 9 patients were cured. CONCLUSION: Treatment with endoscopic sinus surgery is a safe and effective method for mycosis sphenoiditis.

Adult↗

[Saprophytic mycosis appearing as a necrotic bronchial tumor and masking lung cancer].

We describe four cases of saprophytic mycosis superficially covering lung cancer to form a bronchial necrotic tumor. Although the first biopsy in each case disclosed mycosis with necrosis, repeated transbronchial biopsy revealed malignant cells. In two of the four cases, we started treatments for lung cancer based on the clinical diagnosis preceding histological diagnosis. Although no treatment to eliminate fungi was performed, all cases showed an uneventful clinical course. It is important not to misdiagnose cancer as a fungal disease on the basis only of a transbronchial biopsy, and to bear in mind this type of saprophytic mycosis.

Aged↗