Challenges in utilizing ALK expression to distinguish primary cutaneous from systemic anaplastic large cell lymphoma.

Anaplastic large cell lymphoma (ALCL) is a CD30+ peripheral T-cell lymphoma with a clinical spectrum including cutaneous and systemic presentations. While primary cutaneous ALCL (pcALCL) has a favorable prognosis, systemic ALCL (sALCL) has poorer survival outcomes. Expression of anaplastic lymphoma kinase (ALK) by malignant cells has been suggested to distinguish sALCL from pcALCL. However, there have been documented cases of ALK-positive ALCL confined to the skin. The present study reviewed characteristics of published cutaneous ALK-positive ALCL cases to distinguish between these two entities. In 23 identified adults with ALK-positive pcALCL, 26% developed systemic involvement and 74% had skin-limited disease. In 14 pediatric patients, 36% had both cutaneous and systemic involvement and 64% had cutaneous disease only. This analysis revealed that pcALCL and sALCL could not reliably be distinguished by ALK expression or nuclear vs. cytoplasmic localization. Localized treatment with frequent monitoring may be sufficient in ALK-positive pcALCL until there is evidence of progression. Physicians should be aware of the overall spectrum of ALCL, including cutaneous limited disease, systemic disease, disease with NPM-ALK translocation, disease with ALK positivity and disease with skin recurrence.

A Practical Approach to the Diagnosis of Lymphedema: A Narrative Review.

INTRODUCTION: Lymphedema often presents as progressive, unremitting swelling and skin changes that are extremely distressing to patients. Hereditary lymphedema (HL) constitutes a type of primary lymphedema that is passed down through generations. OBJECTIVES: The primary aims of this narrative review are to illustrate a framework to distinguish lymphedema from other causes of swelling and to differentiate the hereditary lymphedemas from each other. RESULTS: A literature search was undertaken using relevant search terms. The articles were evaluated to generate a diagnostic algorithm to approach the swelling of an extremity using clinical and laboratory data. First, the stemmer sign should be evaluated. If it is negative, other causes should be considered. History and additional physical exam findings suggest either a primary or secondary cause of lymph-edema. CONCLUSIONS: The hereditary lymphedemas have been classified by age of onset and then stratified by clinical criteria and genetic testing.

Incidence of cutaneous melanoma and Merkel cell carcinoma in patients with primary cutaneous B-cell lymphomas: A population study of the SEER registry.

Introduction: The increased incidence of cutaneous melanoma (CM) and Merkel cell carcinoma (MCC) in patients with hematologic malignancies (HM) is well established. While the risk of CM has been assessed in some subtypes of HM including cutaneous T-cell lymphoma, the incidence in patients with primary cutaneous B-cell lymphoma (PCBCL) has not been interrogated. Methods: Here we evaluated the standardized incidence ratio (SIR) of CM and MCC in 5,179 PCBCL patients compared to approximately 1.5 billion individuals in the general population using the Surveillance, Epidemiology and End Results (SEER) database. Among patients with PCBCL, we identified subgroups that were at increased risk for CM or MCC as a second primary cancer. Results: We found 36 cases of CM in the PCBCL cohort (SIR, 1.35; 95% CI, 0.94-1.86), among which SIR was significantly elevated for non-Hispanic White patients compared to the general population (SIR, 1.48; 95% CI, 1.03-2.06). Males had a significantly increased risk of developing CM after a diagnosis of PCBCL (SIR, 1.60; 95% CI, 1.10-2.26). We found that males in the age group of 50-59 were at increased risk for CM development (SIR, 3.02; 95% CI, 1.11-6.58). Males were at increased risk of CM 1-5 years after PCBCL diagnosis (SIR, 2.06; 95% CI, 1.18-3.34). Patients were at greater risk of developing MCC within 1 year of diagnosis of PCBCL (SIR, 23.60; 95% CI, 2.86-85.27), particularly in patients who were over the age of 80 (SIR, 46.50; 95% CI, 5.63-167.96). Males aged 60-69 with PCBCL, subtype marginal zone, were also at increased risk for MCC (SIR, 42.71; 95% CI, 1.08-237.99). Conclusion: There is an increased incidence of CM in White, middle-aged males within 5 years of diagnosis of PCBCL and an increased risk of MCC in elderly patients within 1 year of PCBCL diagnosis. These data suggest that certain subgroups of patients with PCBCL may require more rigid surveillance for CM and MCC.

Combination cryotherapy and intralesional corticosteroid versus steroid monotherapy in the treatment of keloids.

BACKGROUND: Keloids are common and have significant negative effects on quality of life. There is a need for more effective treatment approaches for keloids. AIMS: We investigated treatment outcomes of intralesional triamcinolone acetonide (IL TAC) compared with combination IL TAC and cryotherapy, including changes in pruritus, pain, and keloid size. PATIENTS/METHODS: We performed a prospective study of patients referred to one provider who treated patients with combination therapy and compared them to a historic control cohort treated with IL TAC alone. All patients were seen at Thomas Jefferson University between 2019 and 2021. Patient demographics, location of keloids, and inciting events were recorded. Pruritus and pain scores were self-reported by patients using a 10-point Likert scale administered as standard of care. Changes in keloid size were denoted as "No change," "up to 50% decrease," "more than 50% decrease," and "completely flattened." RESULTS: While both treatments produced a significant reduction in mean pruritus and pain scores, there was no difference between the two treatment groups (p = 0.3933 and p = 0.2123, respectively). A greater percentage of keloids in the combination therapy group had a post-treatment size difference greater than 50% compared with those in the IL TAC only treatment group (p = 0.0021). In the subgroup of pubic keloids, all lesions treated with combination IL TAC and cryotherapy responded remarkably well to treatment. CONCLUSIONS: While both IL TAC and IL TAC with cryotherapy were effective at reducing pruritus and pain, combination therapy was more effective in reducing keloid size, specifically for pubic keloids.

Utility of T-cell immunosequencing in distinguishing mycosis fungoides progression from treatment related cutaneous adverse events.

Cutaneous adverse events of both topical and systemic drugs in patients with mycosis fungoides (MF) present a diagnostic challenge as it is often difficult to distinguish drug associated rash from disease progression in the skin. Mogamulizumab and mechlorethamine gel are approved treatments for MF, both of which can cause treatment related cutaneous adverse events. It can often be challenging to distinguish mogamulizumab associated rash (MAR) and mechlorethamine gel associated hypersensitivity dermatitis from MF progression both clinically and histologically. High-throughput sequencing (HTS) of the T-cell receptor (TCR), also known as immunosequencing, can be used to assess T-cell clonality to support a diagnosis of MF. After identification of the malignant TCR clone at baseline, immunosequencing can track the established malignant TCR sequence and its frequency over time with high sensitivity. As a result, immunosequencing clone tracking can aid in distinguishing disease progression from treatment side effects. Here, we present a case series to demonstrate how monitoring of the malignant T-cell frequency by immunosequencing can aid in diagnosis of mogamulizumab and mechlorethamine gel cutaneous adverse events.

Recalcitrant Subcutaneous Panniculitis-Like T-Cell Lymphoma Responsive to Histone Deacetylase Inhibitor.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of cutaneous T-cell lymphoma that usually presents with tender subcutaneous nodules on the trunk and extremities. Immunosuppressive therapy is considered first-line treatment for SPTCL, while multiagent chemotherapy is used for SPTCL complicated by hemophagocytic lymphohistiocytosis (HLH). Here, we report a 42-year-old Hispanic man that presented with a 5-year history of recurrent painful subcutaneous lesions in the absence of constitutional symptoms, lymphadenopathy, and hepatosplenomegaly. A punch biopsy revealed an atypical lymphoid infiltrate in between subcutaneous adipose lobules. Lymphocytes expressed CD3, CD8, and Beta F-1 and did not express CD4 and CD56. Based on clinical and histologic findings, the patient was diagnosed with SPTCL. In addition, laboratory findings did not demonstrate any evidence of HLH. He was initially started on both prednisone and hydroxychloroquine with no improvement. A trial of cyclosporine and methotrexate yielded no clinical improvement. As the lesions failed to resolve after treatment with multiple immunosuppressive agents, romidepsin, an intravenous histone deacetylase (HDAC) inhibitor, was initiated. After two cycles of romidepsin, the patient achieved complete clinical response. He continues to be in remission 12 months later with monthly maintenance therapy. This case illustrates that romidepsin can be useful as monotherapy for refractory SPTCL without HLH.

Does Undergoing Outpatient Hand Surgery Lead to Prolonged Opioid Use? A Comparison of Surgical and Nonsurgical Patients.

BACKGROUND: Orthopedic surgical patients in general have been found to be at higher risk for developing opioid dependence in the postoperative period. However, there is conflicting evidence in the literature whether opioid exposure after hand surgery leads to prolonged use. In the absence of a nonoperative control group, it is not clear whether prolonged opioid use in hand surgical patients is related to undergoing a surgical intervention. The purpose of our study to compare opioid prescription fulfillment patterns in surgical and nonoperative patients in a hand surgery practice. METHODS: We retrospectively compared 320 patients that underwent elbow, wrist, and hand surgery procedures with 741 nonoperative patients treated by 2 hand surgeons. The Pennsylvania Drug Monitoring Program (PDMP), a mandatory statewide database, was used to evaluate the primary outcomes of filling more than one opioid prescription and filling opioid prescriptions beyond 6 months of the index surgery or clinic visit. Bivariate and multivariable logistic regression analysis was performed using the following variables: surgery, prior benzodiazepine use, and prior opioid use. RESULTS: There was no difference in prior opioid use (15.2% vs 16.9%, P = .51) or prior benzodiazepine (10.4% vs 8.4%, P = .33) use between the nonoperative and operative groups. Patients that underwent surgery had a higher incidence of filling more than one opioid prescription (20.9% vs 8.8%, P < .001). However, continued opioid use was not statistically different between nonoperative and operative patients (2.8% vs 5%, P = .08). Bivariate analysis demonstrated that prior opioids (odds ratio [OR] = 12.94, P < .001) and prior benzodiazepines (OR = 1.95, P < .001) were significant independent risk factors for prolonged opioid use. Multivariable analysis demonstrated prior opioid use to be the only independent risk factor for prolonged opioid use (OR = 12.58, P < .001). CONCLUSION: Undergoing outpatient hand surgery do not appear to be an independent risk factor for filling opioid prescriptions beyond 6 months. Significant risk factors for prolonged opioid use include prior use of controlled substances, particularly prior opioid use.

Wide-Awake Surgery With Local Anesthesia and Epinephrine Is Safe.

Hand and upper extremity surgery performed with the patient wide awake involves the use of a local anesthetic and epinephrine. Controversy persists as to whether epinephrine is safe for use in the hand. The goal of this study was to evaluate the safety of epinephrine in hand and upper extremity surgery. The hypothesis was that epinephrine is safe and can be used for a wide breadth of surgical procedures of the hand and upper extremity. A 4-year retrospective chart review was conducted of consecutive patients undergoing wide-awake surgery performed by 2 surgeons at a single institution. All procedures were performed with local anesthesia and epinephrine. Data collected included patient demographics, procedure volume, procedure type, surgical setting, and complications related to epinephrine use. During the study period, 4054 consecutive patients underwent 4287 wide-awake procedures with local anesthesia and epinephrine. Average patient age was 59 years, and 64% of patients were female. No complications occurred as a result of the use of epinephrine, and no tissue necrosis, phentolamine reversal, anaphylaxis, or readmissions occurred. No patients required conversion to general anesthesia or monitored anesthesia care. This analysis of more than 4000 consecutive patients undergoing wide-awake hand and upper extremity surgery with epinephrine confirmed that epinephrine use is safe, with no reported cases of tissue necrosis, reversal, readmission, anaphylaxis, or anesthetic conversion. Epinephrine is safe for use in the hand and upper extremity for patients undergoing wide-awake hand surgery with a local anesthetic. [Orthopedics. 2020;43(6):e529-e532.].

The Reliability of Determining the Presence of Surgical Site Infection Based on Retrospective Chart Review.

PURPOSE: Surgical site infection (SSI) can be a challenging complication after hand surgery. Retrospective studies often rely on chart review to determine presence of an SSI. The purpose of this study was to assess reliability of Centers for Disease Control and Prevention (CDC) criteria for determining an SSI as applied to a chart review. We hypothesized that interobserver and intraobserver reliability for determining an SSI using these criteria while reviewing medical record documentation would be none to minimal (κ < 0.39) based on an interpretation of Cohen's κ statistics. METHODS: We created and used a database of 782 patients, 48 of whom received antibiotics within 3 months of a surgical procedure of the hand. Three fellowship-trained orthopedic hand surgeons then evaluated the charts of those 48 patients, in which each reviewer determined whether an SSI was present or absent based on CDC criteria provided to the reviewers. Patients' charts were then reassessed 1 month later by the same reviewers. Kappa statistics were calculated for each round of assessment and averaged to determine intraobserver and interobserver reliability. RESULTS: Overall κ values were 0.22 (standard error, 0.13), indicating fair reliability. Average κ value between reviewers was 0.26 (standard error, 0.13. On average, intrarater reliability was 68.7%. CONCLUSIONS: We found poor interobserver and intraobserver reliability when using CDC criteria to determine whether a patient had an SSI, based on chart review. CLINICAL RELEVANCE: Better criteria or documentation may be needed in patients with an infection after hand surgery. Retrospective chart reviews to assess infection may be unreliable.

Treatment Outcomes in Patients Undergoing Surgical Treatment for Arthritis of the Distal Radioulnar Joint.

Objective Surgical treatment options for distal radioulnar joint (DRUJ) arthritis include distal ulnar resection (DUR), DRUJ arthrodesis, and ulnar head replacement. Ulnar convergence leading to persistent pain and clicking is a relatively common complication of complete DUR and DRUJ arthrodesis with distal ulnar segment resection (DRUJA). This led to the development of the distal ulna hemiresection (DUHR) and distal ulnar stump stabilization techniques to reduce the risk of this complication. Patients may experience incomplete relief of pain and limited range of motion (ROM) with these procedures. We hypothesized that there would be no differences in outcomes between the treatment groups, but patients undergoing DUHR, tendon interposition, or distal ulnar stump stabilization would be at lower risk of complications. Methods Records were retrospectively reviewed for 121 patients undergoing DRUJ procedures between 2000 and 2018 at a single institution to collect patient demographics, surgical details, preoperative diagnosis, and outcomes including complications, revision procedures, ROM, pain, and swelling. Patients were grouped for analysis by procedure type: DUR (Darrach procedure), DUHR (Bowers procedure), and DRUJA (Sauve-Kapandji procedure). Continuous variables were compared using an analysis of variance test and categorical variables using the Freeman-Halton extension of the Fisher's exact test. A multivariate logistic regression analysis was performed to identify significant predictors of outcomes. Results Seventy-three patients underwent a DUR procedure, while 33 patients underwent a DUHR procedure and 11 underwent a DRUJA procedure. Mean follow-up was 70.6 months. Patients undergoing DRUJA were significantly younger than those undergoing DUR or DUHR procedure (42.4 vs. 60.0 vs. 62.1, p < 0.001). No significant differences between groups were demonstrated in measured outcomes. Posttraumatic arthritis was the most common preoperative diagnosis (43.4%). Persistent pain was the most common negative outcome (25.6%) followed by limited ROM (19.7%). Five patients (4.3%) suffered postoperative complications, most common being rupture of extensor tendons. Five patients (4.3%) underwent revision procedures. Body mass index (BMI) was a significant predictor of persistent pain (odds ratio = 1.09, p = 0.031). Conclusion The results of our study suggest that outcomes are equivalent between the three distinct treatment groups. Despite the potential benefits, hemiresection, tendon interposition, and distal stump stabilization had no significant effect on outcomes in this study. More than a quarter (25.6%) of patients undergoing DRUJ procedures experience persistent pain postoperatively, while one-fifth (19.7%) experienced limited ROM. Patients with higher BMI are at a significantly greater risk of experiencing persistent postoperative pain. Level of Evidence This is a Level III, retrospective comparative study.

Morphometric Assessment of the Residual Width of the Distal Hamate Articular Surface after Graft Harvest for Hemi-hamate Arthroplasty.

BACKGROUND: The hemi-hamate arthroplasty (HHA) can restore joint congruity and stability in chronic fracture-dislocations of the proximal interphalangeal joint (PIPJ). Purpose of this study was to compare the width of the distal hamate articular surface (DHAS) to the width of the base of the middle phalanges (P2) of the fingers. We hypothesized the dimensions of the width of the DHAS would be similar to those of P2, leaving a small amount of residual DHAS width after autograft harvest. METHODS: Fifty-nine CT scans of the hand without any bony pathology were evaluated. Three observers measured the following parameters and compared: (a) Width of the DHAS in the axial and coronal planes; (b) Width of the P2 articular bases of all four fingers; (c) Maximum capitate length (MaxCap) in the coronal plane. RESULTS: The residual DHAS on the coronal plane after graft harvest (bone remaining on the radial and ulnar aspects each, not accounting for saw blade or osteotomy width thickness) among all patients was 1.3, 0.9, 1.4, and 2.4 mm for the index, long, ring and small fingers respectively. There was a strong correlation between DHAS and MaxCap r=0.76. CONCLUSION: There is likely to be a very small amount of residual hamate articular surface width left after the graft is harvested if the entire base of P2 is reconstructed.

Perioperative Risks Are Similar for Normal versus Selected High-Body Mass Index Patients Undergoing Outpatient Hand and Elbow Surgery.

BACKGROUND: Many ambulatory surgery centers use body mass index as a screening tool to make admissions decisions because of complication risks associated with high-body mass index patients. The objective of this investigation was to evaluate perioperative complications in a cohort of high-body mass index patients undergoing hand and elbow surgery at an ambulatory surgery center. The authors' hypothesis was that anesthesia-related complications for this cohort would be similar to those of a normal-body mass index group. METHODS: The authors retrospectively reviewed data from all hand and elbow procedures performed on patients with a high body mass index (>40 kg/m). One hundred eighty-nine high-body mass index patients and 189 normal-body mass index patients were included in the analysis. RESULTS: The average weight-based dosage of propofol was similar in both groups but was lower in the high-body mass index group for midazolam and fentanyl. Two high-body mass index patients had oxygen desaturations in the postanesthesia care unit. No patients developed complications related to anesthesia. In the high-body mass index group, one patient developed hypotension in the postanesthesia care unit, was admitted to the emergency room for monitoring, but was discharged the following morning. CONCLUSIONS: Outpatient hand surgical care of high-body mass index patients can be performed safely. Body mass index alone should not be considered as an absolute contraindication for surgery. Careful patient selection, evaluation of comorbidities, and close involvement of the anesthesia and medical teams are critical. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

The Effect of Intraoperative Corticosteroid Injections on the Risk of Surgical Site Infections for Hand Procedures.

PURPOSE: The aim of the study was to assess the risk of surgical site infection (SSI) in patients who received an intraoperative injection (IOI) with a corticosteroid at the same time as hand surgery for a different condition. METHODS: This was a retrospective chart review of all patients who underwent hand surgery and corticosteroid injections concurrently over an 8-year period. Comparison of the rates of SSI was made of patients who had received an IOI and a matched control cohort of patients with no intraoperative injection (nIOI). There were 391 patients in each group. RESULTS: There were 8 SSIs in the IOI group compared with 2 in the nIOI group. One patient in the IOI group had a deep infection whereas all other infections were superficial. In the IOI group, 206 patients had injections on the side ipsilateral to their surgical procedure. Six of these patients had SSIs, a significant difference compared with the control group. There were 185 patients who had contralateral injections. Two of these patients had SSIs. Compared with the control group, this difference was not significant. CONCLUSIONS: Concomitant injection of steroid into the same side as the surgical site increases the risk of postoperative infection. We do not recommend administering a corticosteroid injection at the time of hand surgery. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic III.