Trichostasis Spinulosa of the Heel: Unique Presentation with Characteristic Morphology

Trichostasis Spinulosa is a peculiar lesion of the hair follicle that typically presents on the face. We present a case of a 59 years old Middle Eastern male who presented with a dark lesion on his heel. Examination revealed a 1.7 cm well-circumscribed black necrotic plaque with surrounding overhanging border with a differential diagnosis of melanoma. Histologically, the lesion was formed by inflamed clusters of numerous small hair shafts, consistent with Trichostasis Spinulosa of the heel. We report this case because of its unusual location and unique presentation

Correlation of PD-L1 expression, clinicopathologic and molecular characteristics in an array of solid tumors: A large-scale real world study

Background: Programmed death ligand-1 (PD-L1) is a predictive marker of anti-programmed death protein 1 (PD- 1)/PD-L1 therapies for solid tumors. Limited literature exists correlating PD-L1 expression, clinicopathological & molecular profiles. We aimed to 1) correlate PD-L1 immunohistochemistry (IHC) results with these profile across multiple solid tumors & 2) assess clinical outcomes (overall survival (OS) & disease-free survival (DFS)) of PD-L1 status with / without anti-PD-L1 immunotherapy (IT). Design: All cases tested for PD-L1 IHC over 2 years (Aug 2019-Sep 2020) were retrieved for this study. Clinicopathological variables recorded included age, race, tumor type, type of PD-L1 clone, PD-L1 status (Tumor Proportion Score (TPS): negative:50%), Combined Positive Score (CPS): negative10), clinical stage, anti-PD-L1 IT. Microsatellite instability (MSI) status using IHC & Ploymerase chain reaction (PCR) assays was recorded. High PD-L1 was defined as PD-L1 expression of TPS \u3e50%/CPS\u3e10. Outcome studies included OS and DFS after generating Kaplan-Meier curves & compared using log rank test. Univariate analysis using Cox regression models were also used. Results: There were 205 cases tested for PD-L1 by IHC. Cohort included non-small cell lung cancers (127), head & neck carcinomas (37), gastric or gastroesophageal carcinoma (20), kidney or urothelial carcinoma (16), cervical carcinomas (5). Median age was 70 years (range 28-90). Most were high stage cancers [stage 1: 5/205, stage 2: 5/205, stage 3: 30/205, stage 4 165/205]. PD-L1 IHC clones included: 22C3 (152/205), 28-8 (21/205) & both (32/205). High PD-L1 expression was observed in 52/205 (25.3%), out of which [37/127 (29.1%) were adenocarcinoma, 13/54 (24%) were squamous cell carcinoma, 2/24 (4.1%) others]. Anti PD-L1 IT was given in 65/205 (31.7%) patients. Anti PD-L1 IT was significantly associated with longer median survival OS (p=0.015) & DFS (p=0.004) (Figure 1). PD-L1 status was significantly associated with OS (p=0. 034) but not DFS (p=0. 076) (Figure 1). High PD-L1 had shorter median survival and higher hazards of death in OS (HR=5.4, CI-1.3-23.1) irrespective of IT. Association between three groups of PD-L1 status when compared with IT was statistically significant (p=0.048, Figure 2). PD-L1 & MSI testing was available in 29 patients & did not show any statistical correlation in this small cohort. No significant difference in survival for those received IT (4/29) vs no IT (25/29) & tested for both PD-L1 & MSI (OS: p= 0.277, DFS: p= 0.107). Conclusions: This study supports the rational approach for PD-L1 therapy. High PD-L1 expression is more commonly seen in adenocarcinoma. Expression of high PD-L1 is associated with worse OS but not DFS. PD-L1 IT is significantly associated with longer median survival, OS & DFS. Larger, prospective studies are needed to support our findings

Salivary gland hyalinizing clear cell carcinoma with cutaneous metastasis: a rare and deceptive tumor

Clear cell carcinoma (CCC) is an uncommon malignant tumor of minor salivary glands. It characteristically has a low-grade morphology and a favorable outcome by most reports. An EWSR1-ATF1 fusion can be detected in the majority of cases. We present a rare case of CCC which had an aggressive course with the development of cutaneous metastases. Practicing dermatopathologists should be aware of this tumor given its low-grade appearance and histologic resemblance to other primary cutaneous adnexal and metastatic neoplasms

P-TS-39: Red Blood Cell Alloimmunization in Hyper-Inflammatory States: A Study on Hemophagocytic Lymphohistiocytosis

Background/Case Studies: Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of pathologic immune activation resulting in hyper-inflammatory response. We have previously reported on the high transfusion needs in HLH. Recipient inflammatory state at time of transfusion has been shown to regulate alloimmunization as in sickle cell disease. With HLH being a hyper-inflammatory disease with increased blood transfusion needs, we wanted to assess if HLH patients are at increased risk of alloimmunization to RBC antigens. Study Design/Methods: We identified all adult patients (age ≥18 years) with a diagnosis of HLH managed at our institution between April 2010 and February 2020. The Institutional Review Boards approved this retrospective chart review study. HLH diagnosis was established based on the 2004 Histiocyte Society Criteria (HLH-2004). The following exclusion criteria were applied: 1- Patients clinically diagnosed with HLH, but did not meet the HLH-2004 criteria, 2- Patients who received packed RBCs in an outside institution before their admission to our institution, 3- Patients not transfused with packed RBCs at our institution, and 4- Patients without a follow up antibody screen. Serologic testing was performed using standard immunohematology techniques following our blood bank procedures. All our blood components were pre-storage leukoreduced. Results/Findings: Forty-four adult patients with HLH fulfilled the inclusion criteria. The median age of patients was 57.1 years (range 18.2 - 77.6 years); 27 (61%) were male. Patients received a median of 8 RBC units (range 2 - 38 units) during admission when HLH diagnosis was established. The median serological follow up after the first transfused RBC unit was 22.5 days (range 3 days - 108.1 months). After a median follow up of 43 days (range 8 days - 122 months), 14 (31.8%) were alive. We only had one patient who developed a new anti-E antibody that was identified 13 days after transfusion of an E-positive RBC unit. The patient was a 27-year-old Caucasian male with no history of previous transfusions. At the time of alloimmunization, his DAT was negative and hemolysis workup was unremarkable. Conclusions: The rate of alloimmunization (2.3%) in our cohort was not increased compared to that of the general population (3-5%). The patient who had alloimmunization seemed to have had a primary immune response based on negative transfusion history and absence of delayed hemolytic transfusion reaction. However, his alloimmunization was detected after only 13 days from transfusion of the involved RBC unit which was earlier than the usual window period for primary immune responses following transfusion. This early primary response might be due to the hyper-inflammatory state of HLH. RBC alloimmunization in HLH warrants further investigation

Trichostasis Spinulosa of the Heel: Unique Presentation with Characteristic Morphology

Trichostasis Spinulosa is a peculiar lesion of the hair follicle that typically presents on the face. We present a case of a 59 years old Middle Eastern male who presented with a dark lesion on his heel. Examination revealed a 1.7 cm well-circumscribed black necrotic plaque with surrounding overhanging border with a differential diagnosis of melanoma. Histologically, the lesion was formed by inflamed clusters of numerous small hair shafts, consistent with Trichostasis Spinulosa of the heel. We report this case because of its unusual location and unique presentation

Pretibial Myxedema, Nodular Variant: Unique Presentation and Clinical Course

Literature has shown morphological diversity in the presentation and occurrence of pretibial myxedema following I-131 treatment. We present a case of a 47 y.o African American male with post I-131 hypothyroidism for Graves disease that presented with enlarging bilateral lower extremity and ankle lesions. Examination revealed firm, tender, hemispherical, large, hyperpigmented nodules. Ultrasound demonstrated nonspecific hypovascular nodular thickening of the skin. Excisional biopsy showed reactive dermal and subcutaneous fibrosis with dermal mucinosis. Alcian blue stain confirmed the diagnosis. Graves’ dermopathy is generally rare, with the nodular variant accounting for 10% of cases. Our case reflects a variable appearance of post-I-131 treatment dermopathy partially resistant to the multimodal treatment

Serum ferritin trends are associated with survival in adult hemophagocytic lymphohistiocytosis

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of excessive and ineffective activation of the immune system resulting in hyper-inflammatory response. Common presenting features include fever, cytopenias, elevated serum ferritin, and splenomegaly. HLH diagnosis is challenging as the available diagnostic criteria lack specificity. In addition, patients have a dismal outcome, and markers predicting survival are lacking. Ferritin concentration is a surrogate marker for inflammation, and repeated measurements might be associated with disease severity and outcome. We explored the prognostic significance of serum ferritin in adult HLH. Methods: We reviewed diagnoses of adult HLH treated at Barnes-Jewish Hospital (St. Louis, Missouri) and Henry Ford Hospital (Detroit, Michigan). We included 98 patients who met the 2004 Histiocyte Society criteria for HLH and had at least 2 serial ferritin values during the admission when diagnosis was established. Admission ferritin was defined as the initial value, and peak ferritin was defined as the highest value during admission. We examined ferritin levels change during admission which could be increasing (peak ferritin being the last ferritin value) or decreasing (ferritin values decreasing after peak ferritin). Predictive factors for survival during hospital admission were evaluated by univariate analysis followed by logistic regression. Analyses were performed using SPSS version 22, and results were considered significant at p \u3c0.05.Results: The median age of patients was 48 years (range, 18-79 years); 64 (65%) were male. The most common clinical and laboratory abnormalities during hospitalization were: hyperferritinemia (100%; median 27,306 µg/L; range 1,823 - 684,000 µg/L), fever (96%), cytopenias (91%), hemophagocytosis (76.7%), elevated triglycerides (72.4%), splenomegaly (67.3%), and hypofibrinogenemia (42.7%). Median duration of hospital stay was 20 days (range, 1 - 89 days); 38 (39%) patients died during admission. Death was attributed to sepsis in 23 patients, lymphoma in 7, bleeding in 4, GVHD in 1, liver failure in 1, metastatic solid tumor in 1, and unknown in 1.Table 1 shows the parameters analyzed as predictors of mortality during hospital stay. In the univariate analysis, we found that age ≥50 years, increasing ferritin levels during admission, and malignancy were associated with higher mortality. At the multivariate regression level, age ≥50 years (OR = 3.31; 95% CI: 1.2 - 9.15; p = 0.021) and increasing ferritin levels (OR = 8.96; 95% CI: 2.97 - 27.02; p \u3c0.0001) correlated with mortality. In addition, patients with declining ferritin levels by more than 50% from the peak were more likely to survive (OR = 2.21; 95% CI: 1.13 - 4.3; p = 0.026).Conclusion: Our results showed that ferritin trends rather than absolute concentration are associated with risks of mortality when HLH is diagnosed during a hospitalization. A declining ferritin trend is associated with lower risk of death and an increasing ferritin trend is associated with an increased risk of death. Prospective investigations of ferritin trends in HLH are warranted. Disclosures No relevant conflicts of interest to declare

Hemophagocytosis on ascitic fluid cytology: Diagnosis of HLH

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of pathologic immune response characterized by excessive activation of macrophages. Hemophagocytosis is one of the diagnostic criteria for HLH, and it usually involves the bone marrow, spleen, lymph nodes, or any part of the reticuloendothelial system. Hemophagocytosis in the ascitic fluid has rarely been reported in HLH. Here, we report the case of a patient who presented with fever and abdominal distention and ascites. Ascitic fluid cytology showed hemophagocytosis which was the clue for HLH diagnosis. We also review the literature for this rare cytological occurrence