Safety of a new, single, integrated, closed photopheresis system in patients with cutaneous T-cell lymphoma.

BACKGROUND: Extracorporeal photopheresis (ECP) is an approved palliative treatment for cutaneous T-cell lymphoma (CTCL). The THERAKOS CELLEX (continuous flow separation) system (Therakos, Exton, PA, U.S.A.) has been developed from the current THERAKOS UVAR XTS system. It is designed to reduce treatment times and extracorporeal volumes, and allows the use of either a single- or a dual-needle configuration. OBJECTIVES: To assess the safety of the THERAKOS CELLEX system to provide ECP for patients with CTCL. METHODS: Patients received ECP with the THERAKOS CELLEX system for up to 6 months. The treatment schedule was defined by their current treatment and response to ECP. At least 150 treatments were required to assess safety of the new system. Safety was assessed using reports of adverse device effects (ADEs) and unanticipated ADEs (UADEs), device malfunctions and defects, vital signs, laboratory parameters and physical examinations. RESULTS: Thirteen patients were enrolled and 12 completed the study; 155 ECP treatments were initiated and 153 completed. There were no ADEs or UADEs reported during the study. The mean treatment time was shorter for patients who received dual- compared with single-needle treatments (74.4 vs. 103.0 min, P < 0.0001) and the extracorporeal volume was lower (216 vs. 266 mL). CONCLUSIONS: This new ECP system provides lower extracorporeal volumes, faster treatment times, and flexibility to use either single- or dual-needle access, while not being associated in this study with any ADEs, and therefore having a positive benefit-risk ratio for patients with CTCL.

The surgical correction of protuberant ears.

While prominent ears are considered a sign of good fortune in the Far East, Western society looks upon prominent ears in a far less positive manner. Children with prominent ears are often the subjects of verbal and at times physical abuse by their peers, resulting in adverse psychological effects. Advances in otoplasty have made it possible not only to pin back the ears, but also to reshape them, reduce their size, or make them more symmetrical. For a dermatologic surgeon, an otoplasty may be an unfamiliar surgical procedure, however, the surgery itself does not significantly differ from ear wedges or cartilage removal procedures for skin cancer, procedures with which the dermatologic surgeon is quite familiar.

Treating refractory chronic graft-versus-host disease with extracorporeal photochemotherapy.

Extracorporeal photochemotherapy (ECP; photopheresis), an immunomodulatory therapy, has previously demonstrated promising results in treating chronic graft-versus-host disease (cGvHD). We treated six patients (ages 33-54 years) with long-standing refractory extensive-stage cGvHD. ECP was performed thrice weekly initially in all patients. Concomitant therapies included prednisone (n=6), tacrolimus (n=5), cyclosporin A (n=2), hydroxychloroquine (n=2), mycophenolate mofetil (n=1), and psoralen plus ultraviolet A radiation (n=1). After an average of 7.2 months (range, 2-13 months) of ECP, all patients experienced either improvement or stabilization in sclerodermatous skin changes, as well as partial improvements in liver enzyme levels. Skin softening occurred in four patients and was noted as early as 3-8 weeks into treatment. Two patients were able to taper steroid therapy, and two patients were able to taper ECP to twice weekly. ECP was well tolerated. Our results support those of previous studies, suggesting that ECP may be beneficial in patients with refractory cGvHD.

Dermatologic surgery into the next millennium, Part III.

Face-lift procedures and blepharoplasties are performed as in-office surgeries. Either local sedation or conscious sedation is used, and the patient's vital signs are carefully monitored. To avoid complications, the patient is followed closely postoperatively. It is highly suggested that the patient receives a medical clearance prior to undergoing either of these procedures.

Dermatologic surgery into the next millennium, part II.

This is the second article in a 4-part series on dermatologic surgery. This section provides detailed information about filling agents and botulinus toxin A. The filling agents discussed here are frequently used in our office. It is emphasized that meticulous technique and patient selection predict a good cosmetic result. To select the right agent, patient safety must be a priority.

Feasibility of photopheresis to reduce the occurrence of restenosis after percutaneous transluminal coronary angioplasty: a clinical pilot study.

BACKGROUND: Photopheresis was evaluated as a means of preventing restenosis on the basis of immune modulation. METHODS: This was a prospective, randomized, controlled clinical trial analyzing clinical restenosis at 6 months after percutaneous transluminal coronary angioplasty (PTCA). Seventy-eight patients with single-vessel angioplasty were randomly assigned to a control group of 41 patients and a treatment group of 37 patients. At 6 months, there were 72 evaluable patients: 39 control patients and 33 treated. Twenty-nine control patients received balloon PTCA only and 10 patients received stents. Twenty treated patients received PTCA only and 13 patients received stents. Baseline clinical characteristics of both groups were similar. The treatment group received photopheresis for a total of 5 treatments. Primary end points were death from any cause, myocardial infarction, ischemia, and repeat revascularization procedures. RESULTS: By intention-to-treat analysis, clinical restenosis occurred in 27% of control patients versus 8% of treated patients (P =.040, relative risk = 0.30). CONCLUSIONS: Photopheresis therapy in patients undergoing balloon PTCA with and without stent deployment has been shown to be effective in reducing restenosis. The use of photopheresis in such patients merits further investigation.

The Webster-type face and neck lift: an extensive cervico-facial rhytidectomy employing a minimally invasive technique.

BACKGROUND: Facelifting has incorporated a wide range of new techniques and developments over the past few decades. Many techniques employ a more aggressive surgical approach that may be advocated for seeming advantage, yet upon closer review may be unwarranted for the average patient seeking facial rejuvenation. The Webster-type face and neck lift utilizes a minimally invasive approach to thoroughly resupport the face and neck substructure and skin. This approach affords the patient excellent results while limiting untoward complications. OBJECTIVE: To evaluate the efficacy of the Webster-type face and neck lift. METHODS: The Webster-type face and neck lift was utilized in 200 facelift patients. We reviewed these cases for aesthetic results and associated complications. RESULTS: The great majority of patients have been uniformly pleased with the aesthetic restoration via the Webster-type lift. Postoperative courses have been routinely uneventful and major complications have been avoided. CONCLUSION: The evolution of more invasive approaches to cervicofacial rhytidectomy has not been shown to produce consistently better or longer-lasting results. Many of these aggressive techniques have extended the operating time, heightened the potential morbidity of the operation, and prolonged the duration of convalescence. The Webster-type lift is a proven, modern approach that provides comparable and lasting results while limiting risk and avoiding serious complications.

Extracorporeal photochemotherapy alone or with adjuvant therapy in the treatment of cutaneous T-cell lymphoma: a 9-year retrospective study at a single institution.

BACKGROUND: Extracorporeal photochemotherapy (ECP; photopheresis) is a treatment option for cutaneous T-cell lymphoma (CTCL). OBJECTIVE: This study describes the outcomes obtained with ECP alone or with adjuvant therapy in treating CTCL. METHODS: A 9-year retrospective study was performed at a single institution. RESULTS: Among 69 patients, 37 were treated with 6 months or more of ECP alone over an average of 36.9 months. Of these patients, 68% (25/37) had stage T2, 5% (2/37) had stage T3, and 27% (10/37) had stage T4 CTCL. Complete response (no skin or systemic disease for 1 month or more) and partial response (50%-99% skin improvement for 1 month or more) were achieved by 14% (5/37) and 41% (15/37) of patients, respectively, giving an overall response rate of 54% (20/37). In recalcitrant patients, adjuvant therapy significantly increased the response rate from 31% (4/13) to 69% (9/13) (P = 0.004). ECP was well tolerated in the entire patient population. CONCLUSION: Response rates in this study compared favorably with those in previous studies, underscoring the potential value of ECP in treating CTCL. To our knowledge, this investigation included the largest group of CTCL patients ever treated with ECP at a single institution.

Scleromyxedema with dermato-neuro syndrome.

Scleromyxedema is a rare connective tissue disease of unknown cause characterized by a generalized papular eruption, dermal fibroblast proliferation, and monoclonal paraproteinemia. A paroxysmal triad consisting of high fever, seizures, and coma with a flu-like prodrome can rarely occur in patients with scleromyxedema and is termed "dermato-neuro syndrome." We describe a 41-year-old patient with scleromyxedema in whom the dermato-neuro syndrome developed.

The use of conscious sedation for outpatient dermatologic surgical procedures.

BACKGROUND: Dermatologic surgery has undergone increasing levels of sophistication over the past few decades. Commensurate with this demand, an established anesthesia technique called conscious sedation has been employed. OBJECTIVES: Methods for performing office-based conscious sedation are described. Recommendations are made regarding prerequisites for conscious sedation in an office setting, patient selection, complications management, and postoperative discharge requirements. CONCLUSION: The goals of anesthesia are to provide for patient safety and comfort, to increase patient acceptance of the procedure, and to enhance the surgeon's efficiency and satisfaction.

Dermatologic surgery into the next millennium: Part I.

Dermatologic cosmetic surgery is a subspecialty of dermatology that has recently been developed. In this four-part series, we describe many of the procedures performed in our ambulatory surgery setting. We also highlight the significant changes in the techniques and treatment environments for these procedures over the past three decades. In part one, we focus on liposuction and face lift operations. Patients increasingly expect less invasive procedures, decreased "down time," and superior results. These expectations create a greater demand for the dermatologic surgeon. The trends and prospects for dermatologic cosmetic surgery in the next millennium are illustrated.

The combined use of propofol and fentanyl for outpatient intravenous conscious sedation.

BACKGROUND: Intravenous conscious sedation is currently being widely utilized for outpatient surgery including dermatologic surgery. Even though this type of anesthesia is typically administered by a trained, licensed anesthetist, it is important for dermatologists who either intend to or are currently utilizing this type of anesthesia to be familiar with some of the methods and agents that are commonly employed. OBJECTIVE: Propofol and fentanyl are two anesthetic agents that are in prevalent use for skin and soft tissue surgery of brief or limited duration. With the goal of familiarizing dermatologic surgeons with this form of anesthesia, a study was conducted to evaluate the safety and effectiveness of the combination of propofol and fentanyl when used for conscious sedation in an outpatient dermatology center. METHODS: Twenty patients, ages 25-65 years, who required conscious sedation were enrolled. Each patient received a standard dosage of fentanyl and propofol, as determined on a kilogram basis. Sedation time, total procedure time, recovery time, and total propofol dose, along with side effects, were determined. RESULTS: The mean onset to sedation was 52.5 seconds, the mean procedure time was 40 minutes 37 seconds, and the mean interval to recovery was 3 minutes 43 seconds, with a mean total dose of propofol of 5.83 mg/kg. Minimal side effects occurred. CONCLUSION: Propofol when used in conjunction with fentanyl appears to be a safe, quick, and effective method of providing conscious sedation.

Surgical intervention for the aging face. Combination of mini-face-lifting and superficial carbon dioxide laser resurfacing.

BACKGROUND: Patients seeking facial rejuvenation often would benefit from a combination of face-lifting and laser resurfacing. Fear of skin slough has prevented the study of this issue. OBJECTIVE: We report our experience utilizing a combination of simultaneous cervicofacial rhytidectomy and carbon dioxide (CO2) laser resurfacing, and summarize the results of a survey completed by each patient. METHODS: Forty patients underwent the combination treatment. Patients were followed and asked to respond to a questionnaire 12 months postprocedure regarding their surgical experience. RESULTS: Our survey revealed an overall degree of satisfaction. Reported complications included transient swelling, redness, pigmentary alteration, and numbness. One patient experienced minimal slough in a nonresurfaced site. CONCLUSIONS: The control of depth of thermal damage with the CO2 UltraPulse laser allows for precision in its combined application along with face-lifting. In the area of the flap, one pass at low power settings is safe in our experience and results in an improved cosmetic result.

A lymphocyte cell surface heat shock protein homologous to the endoplasmic reticulum chaperone, immunoglobulin heavy chain binding protein BIP.

BE2 is a cell surface monomeric 78-kDa protein (BE2-78) expressed on the malignant lymphocytes of cutaneous T-cell lymphoma and adult T-cell leukemia, on some lymphocytes from patients with acquired immunodeficiency syndrome and on Epstein-Barr virus-transformed B cells. BE2-78 positivity of cutaneous T-cell lymphoma tumor cells is a useful diagnostic and prognostic determinant in evaluating patients with that disorder. The BE2-78 protein was isolated from Epstein Barr virus-transformed B cells, purified by 1- and 2-dimensional electrophoresis and then sequenced. The sequence of 4 isolated peptide fragments was highly homologous with the 78-kDa heat shock protein, BiP, an endoplasmic reticulum chaperone. The similarity between BiP and BE2-78 was supported by the demonstration that BE2-78, like BiP, avidly binds to ATP. However, polyclonal and monoclonal reagents that recognize cytoplasmic 70- and 78-kDa heat shock proteins do not detect the BE2-78 antigen on the cell surface of cutaneous T-cell lymphoma or Epstein Barr virus-transformed lymphocytes, and peptide mapping demonstrates sequence divergence, suggesting that either they are distinct or conformationally different molecules. Our results indicate that BE2-78 is a cell surface heat shock protein. The possibility that malignant or transformed lymphocytes may express cell surface molecules with the capacity to bind a spectrum of exogenous or endogenous peptides has potential implications for tumor immunology.

Extracorporeal photochemotherapy in progressive systemic sclerosis: a follow-up study.

BACKGROUND: Extracorporeal photochemotherapy (photopheresis), an immune-modulating therapy, has been demonstrated to elicit a therapeutic response in the treatment of several autoimmune disorders. We evaluated the use of photopheresis in the treatment of patients with progressive systemic sclerosis (PSS; scleroderma). METHODS: Five patients with early-onset, diffuse PSS were treated with photopheresis on 2 successive days monthly for an average of 59 months (range 54-89 months). We initially reported the response this group of patients had to photopheresis treatment at an average of 11 months (range 6-21 months). RESULTS: An improvement or stabilization was noted in most patients in skin thickening, joint mobility, pulmonary function studies, oral aperture, functional index, as well as symptoms including Raynaud's phenomenon, dyspnea, fatigue, dysphagia, arthralgias, and cutaneous ulcers. Renal function tests remained within normal range. A total of 296 monthly treatments were administered without significant toxicity. CONCLUSIONS: This study suggests that extended use of extracorporeal photochemotherapy in the management of early-onset, diffuse PSS is well tolerated and may provide an increasingly beneficial clinical outcome.

HTLV-associated diseases: human retroviral infection and cutaneous T-cell lymphomas.

An array of neurologic, oncologic, and autoimmune disorders are associated with infection with the human pathogenic retroviruses human T-cell leukemia virus types I and II (HTLV-I, II), as well as the human immunodeficiency viruses (HIV). The cutaneous T-cell lymphomas, mycosis fungoides (MF) and its hematogenous variant Sezary Syndrome (SS), share similar clinical and pathological features to HTLV-I-associated adult T-cell leukemia (ATL) and speculation of a retroviral link to MF and SS, especially in areas non-endemic for ATL, has lead to an intensified search for HTLV- and HIV-like agents in these diseases. To further explore a potential role for human retroviruses in MF and SS, skin biopsy-derived or peripheral blood mononuclear cell-derived DNA from 17 patients (MF, n = 7; erythrodermic MF (EMF), n = 5; SS, n = 5) from the North Eastern United States were screened using gene amplification by PCR and a liquid hybridization detection assay. Previously published primers and probes for HTLV-I (LTR, gag, pol, env, and pX), and our own primers and probes for HTLV-I (gag, pol, and env), HTLV-II (pol and env) and HIV-I (gag and pol) were employed. Serum antibodies to HTLV-I were negative in all but one EMF patient. The single HTLV-I seropositive patient carrying a diagnosis of EMF generated positive amplified signals for all of the eight HTLV-I regions tested. Ultimately, this individual evolved to exhibit clinical manifestations indistinguishable from ATL. The other 16 patients were negative for all 12 HTLV and HIV retroviral regions. Our findings suggest that none of the known prototypic human retroviruses are associated with seronegative MF and SS. The uniformly positive results for HTLV-I in the seropositive patient suggests that this patient initially presented with a smoldering form of ATL and illustrates the difficulty that sometimes may be encountered in the differential diagnosis of MF, SS, and ATL based solely on clinical and histopathological criteria.

Cutaneous T-cell lymphoma, tropical spastic paraparesis, cerebral vasculitis, and protein S deficiency in a patient with HTLV-I.

The clinical spectrum of retroviruses is expanding rapidly. Human T-cell lymphotropic virus type I (HTLV-I) was the first retrovirus to be described, and its role had been established in adult T-cell leukemia/lymphoma and tropical spastic paraparesis. We report the case of a 35-year-old woman with HTLV-I and the unusual combination of cutaneous T-cell lymphoma, tropical spastic paraparesis, cerebral vasculitis, and protein S deficiency. We discuss the relationship of all her diseases to HTLV-I.

Cytolytic response to HIV in patients with HIV disease treated with extracorporeal photochemotherapy: preliminary study.

Extracorporeal photochemotherapy (photopheresis), an immunomodulatory therapy that targets circulating T helper lymphocytes, has been applied to the management of human immunodeficiency virus (HIV) disease. Any therapy that exerts its actions on CD4+ T cells has the potential of exacerbating HIV infection. Therefore, it was necessary to observe immune function during treatment. Because cytotoxic T lymphocytes (CTL) and natural-killer cells are thought to play an important role in the response against HIV infection, we examined the effect of photopheresis on HIV cytolytic activity. The study group consisted of seven patients with late-stage HIV disease who had not received any previous treatment for HIV infection. Patients were treated exclusively with photopheresis on two consecutive days each month for 14-32 months (average, 25 months). Peripheral lymphocytes, collected at various points during treatment, were used as effectors in a 51Cr release assay. Epstein-Barr virus (EBV)-transformed autologous B cell lines transfected with recombinant vaccinia vectors that expressed the HIV env (gp120, gp41) and gag (p24) proteins were used as target cells. All seven patients demonstrated relatively constant levels of cytolysis (>10% above controls) during treatment in the context of stable CD4+ T cell counts and a stable clinical status. These results suggest that extracorporeal photochemotherapy did not impair the cytolytic response to HIV infection and may have enhanced it in some patients.

Case report: post-transfusion purpura.

Post-transfusion purpura (PTP) is a rare disorder characterized by the abrupt onset of severe thrombocytopenia following a blood transfusion. We describe a case of PTP in a patient treated with plasmapheresis, noting that this is an uncommon but treatable disease entity.