Increased Number of Perivascular CD20-Positive B Lymphocytes in the Neuropathology of CLIPPERS: Findings of 6 Patients from Mainland China

2021 ◽  
pp. 1-9
Author(s):  
Baifeng Yang ◽  
Fan Liu ◽  
Jing Li ◽  
Yanbin Wen
Keyword(s):  
Histopathological Examination ◽  
Mainland China ◽  
Clinical Manifestations ◽  
Lymphocytic Infiltration ◽  
Final Diagnosis ◽  
Chronic Inflammatory Disease ◽  
The Other ◽  
Cell Infiltration ◽  
T Cell Infiltration ◽  
Pepper Powder

<b><i>Introduction:</i></b> Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome is a recently described chronic inflammatory disease of the central nervous system. There are few reports of CLIPPERS in the Chinese population to date. We summarized the clinical characteristics of 6 CLIPPERS patients to deepen the understanding of this disease. <b><i>Methods:</i></b> The clinical manifestations and treatment of 6 CLIPPERS patients confirmed by pathology or clinical diagnosis in our hospital were retrospectively analyzed. <b><i>Results:</i></b> The common clinical manifestations included ataxia, dysarthria, diplopia, dysphagia, dizziness, cognitive impairment, facial paresthesia, and paralysis. Most of the lesions showed typical symmetric “pepper powder”-like dot and nodular enhancement centered in the pontine and cerebellum except 1 patient with unilateral nodular enhancement. The brain histopathological examination of the 5 biopsied patients indicated that, with the exception of patient 4 with no lymphocyte infiltration, a large amount of perivascular lymphocytic infiltration was found in the other 4 patients, among whom only 1 patient was dominated by CD3+ T cell infiltration and the other 3 patients were dominated by CD20+ B cell infiltration. After treatment with intravenous methylprednisolone, all patients had significant clinical recovery associated with complete or significant MRI recovery, but they were prone to relapse after withdrawal or reduction of the corticosteroid. <b><i>Conclusion:</i></b> Our reports highlight the importance of neuropathological examinations when encountering atypical imaging manifestations, such as unilateral and large nodular Gd+ lesions, in order to establish a final diagnosis of CLIPPERS. In addition, the lymphocytic infiltration in the lesions of CLIPPERS may be dominated by CD20+ B cells instead of CD3+ T cells.

scholarly journals The Frequency and Causes of Subepidermal Gaps or Blisters and the Compliance Rate Between Clinical Diagnosis and Pathology Reports from 2015 to 2019

2021 ◽  
Vol In Press (In Press) ◽  
Author(s):  
Abbas Darjani ◽  
Hojat Eftekhari ◽  
Seyedeh Rojin Amini Rad ◽  
Narges Alizadeh ◽  
Rana Rafiee ◽  
...  
Keyword(s):  
Clinical Diagnosis ◽  
Skin Diseases ◽  
Histopathological Examination ◽  
Compliance Rate ◽  
Clinical Manifestations ◽  
Final Diagnosis ◽  
Direct Immunofluorescence ◽  
Diagnostic Challenge ◽  
Cross Sectional ◽  
Pathology Reports

Background: Skin diseases are the fourth most common cause of human illness, and blisters with different clinical manifestations make a diagnostic challenge. Objectives: This study aimed to evaluate the frequency and causes of subepidermal gaps or blisters, as well as the compliance rate between the initial and final clinical diagnoses based on pathology reports. Methods: In this cross-sectional study, pathology reports of subepidermal blisters or gaps were evaluated in the patients referred to the Razi Laboratory of Rasht from 2015 to 2019. The samples were examined by a pathologist after hematoxylin and eosin staining. The reports included demographic information, clinical differential diagnoses, final diagnosis, direct immunofluorescence findings, and salt split results. Finally, the compliance rate of clinical diagnosis with pathology reports was determined. Results: A total of 183 pathology reports were evaluated, 170 of which contained the final diagnosis. Females were more frequently affected by the disease, and pemphigoid bolus and lichen planus were the most prevalent final diagnoses. The compliance rate between the initial and final diagnoses was 94%. About 37.2% of the reports lacked direct immunofluorescence (DIF) and salt split, and only 42.6% of the samples had undergone DIF examination, while 20.2% had both DIF and salt split. There was no significant association between the compliance rate of the final diagnosis with age, sex, and undergoing diagnostic tests. Conclusions: A high incidence of subepidermal gaps or blisters was seen in middle-aged individuals and females. The compliance rate of the initial clinical diagnosis with the final diagnosis based on pathological reports was high. Our findings emphasize the importance of histopathological examination and the complementary role of direct immunofluorescence and salt split in diagnosis.

scholarly journals Targeting pulmonary tumor microenvironment with CXCR4-inhibiting nanocomplex to enhance anti–PD-L1 immunotherapy

2020 ◽  
Vol 6 (20) ◽  
pp. eaaz9240 ◽  
Author(s):  
Zhaoting Li ◽  
Yixin Wang ◽  
Yuexin Shen ◽  
Chenggen Qian ◽  
David Oupicky ◽  
...  
Keyword(s):  
T Cell ◽  
Tumor Microenvironment ◽  
Objective Response ◽  
Suppressor Cells ◽  
Pulmonary Delivery ◽  
Lymphocytic Infiltration ◽  
Cell Infiltration ◽  
T Cell Infiltration ◽  
Cell Accumulation ◽  
Immunosuppressive Cell

Anti–programmed cell death 1 ligand 1 (PD-L1) therapy is extraordinarily effective in select patients with cancer. However, insufficient lymphocytic infiltration, weak T cell–induced inflammation, and immunosuppressive cell accumulation in the tumor microenvironment (TME) may greatly diminish the efficacy. Here, we report development of the FX@HP nanocomplex composed of fluorinated polymerized CXCR4 antagonism (FX) and paclitaxel-loaded human serum albumin (HP) for pulmonary delivery of anti–PD-L1 small interfering RNA (siPD-L1) to treat orthotopic lung tumors. FX@HP induced T cell infiltration, increased expression of calreticulin on tumor cells, and reduced the myeloid-derived suppressor cells/regulatory T cells in the TME, thereby acting synergistically with siPD-L1 for effective immunotherapy. Our work suggests that the CXCR4-inhibiting nanocomplex decreases tumor fibrosis, facilitates T cell infiltration and relieves immunosuppression to modulate the immune process to improve the objective response rate of anti–PD-L1 immunotherapy.

Congenital primary cerebral angiosarcoma

2000 ◽  
Vol 92 (3) ◽  
pp. 466-468 ◽  
Author(s):  
Yasuhiro Suzuki ◽  
Yasuko K. Yoshida ◽  
Reizo Shirane ◽  
Takashi Yoshimoto ◽  
Mika Watanabe ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Histopathological Examination ◽  
Good Condition ◽  
Clinical Manifestations ◽  
Final Diagnosis ◽  
Abdominal Ultrasonography ◽  
Content Type ◽  
The Central Nervous System ◽  
Specific Antigens

✓ Reports of angiosarcoma arising in the central nervous system are rare. The authors present the case of a 30-day-old infant with clinical manifestations of projectile vomiting and tense anterior fontanelle resulting from a left frontotemporal tumor. Total excision of this highly vascular, well-circumscribed tumor was performed without incident, and histopathological examination revealed a malignant angiosarcoma. Immunohistochemical reaction of the neoplastic cells was diffusely positive for endothelium-specific antigens including factor VIII-related antigen, CD31, and CD34. The final diagnosis of congenital primary cerebral angiosarcoma was thus confirmed. The patient's postoperative course was uneventful, and he was discharged 2 weeks after the operation. He was in good condition with no sign of recurrence after 11 months; follow-up computerized tomography, magnetic resonance (MR) imaging, and abdominal ultrasonography studies demonstrated no tumor regrowth. The characteristic findings for this tumor on MR imaging, the immunohistochemical findings, and surgical outcome are discussed.

scholarly journals Deceptively complex diagnosis of early onset primary chronic osteomyelitis: a case report

2021 ◽  
Vol 45 (1) ◽  
Author(s):  
T. Kavya Priya ◽  
Amith Hadhimane ◽  
Kirthi Kumar Rai ◽  
H. R. Shiva Kumar ◽  
Amarnath P. Upasi ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Early Onset ◽  
Chronic Osteomyelitis ◽  
Malignant Tumors ◽  
Histopathological Examination ◽  
Final Diagnosis ◽  
Chronic Inflammatory Disease ◽  
Fistula Formation ◽  
Unknown Etiology ◽  
Disease Entity

Abstract Background Primary chronic osteomyelitis is a rare occurrence in the pediatric mandible though it exclusively affects the mandible with no age preference. The absence of pus, fistula and sequestration are characteristic with insidious onset lacking an acute state. It is a chronic inflammatory disease of unknown etiology. It has two peaks of incidence: initial peak at adolescence and the second peak after the age of 50 years. Clinical and radiological presentation does not suffice the diagnosis of Primary chronic osteomyelitis necessitating biopsy followed by histopathological examination. Use of several terms in the literature to describe this disease entity has led to further confusion. The Zurich classification system satisfactorily describes the early onset Primary Chronic Osteomyelitis based on etiology and pathogenesis. Case presentation A case of Early Onset Primary Chronic Osteomyelitis in a 10 year old boy is reported comprehensively from clinical presentation to diagnosis and treatment. A meticulous hierarchical order of investigations leads the way to final diagnosis with the aid of existing literature. Extra-oral biopsy, decortication and antibiotic therapy proved to be an effective treatment with no recurrence at 1 year follow-up. Conclusion The clinical and radiological features of Early Onset Primary Chronic Osteomyelitis are deceptively complex throwing an array of differential diagnosis including malignant tumors whilst histopathology reveals only chronic inflammation making this entity an enigma. This disease entity should be included in the differential diagnosis for a pediatric posterior mandibular swelling that occurs without an infectious nidus, pus discharge and fistula formation.

scholarly journals Utility of Direct Fast Scarlet Staining in the Histopathological Diagnosis of Eosinophilic Esophagitis: A Short Report

2020 ◽  
Vol 2 (4) ◽  
pp. 448-455
Author(s):  
Takuji Tanaka ◽  
Naoki Watanabe ◽  
Tomohiro Kato ◽  
Ryogo Aoki ◽  
Tomio Ogiso ◽  
...  
Keyword(s):  
Eosinophilic Esophagitis ◽  
Histopathological Examination ◽  
Clinical Manifestations ◽  
Atopic Disease ◽  
Final Diagnosis ◽  
Histopathological Diagnosis ◽  
Short Report ◽  
Esophageal Mucosa ◽  
Pharmacological Agents ◽  
Biopsy Specimens

Eosinophilic esophagitis (EoE), an atopic disease of the esophagus, has become increasingly recognized over the last 15 years. The epidemiology of EoE has now been reported from many countries around the world. While the clinical diagnosis of this disease depends on the patient’s clinical manifestations, the final diagnosis should be made based on the histopathological examination of esophageal mucosal biopsies. In the diagnosis of EoE, to facilitate the appropriate treatment of patients, it is extremely important to precisely recognize the presence of eosinophils in biopsy specimens of the esophageal mucosa. If eosinophils are present, EoE patients should be referred to an allergist for appropriate management with dietary modification, pharmacological agents (including corticosteroids), and/or mechanical dilation of the esophagus. We herein present and recommend the use of direct fast scarlet staining for the easy and precise recognition of eosinophils in biopsy specimens of the esophageal mucosa, a technique that has been routinely used in our laboratory.

scholarly journals Clinical and morphological characteristics of lichen planus and lichenoid drug eruption of the skin

2020 ◽  
Vol 23 (3) ◽  
pp. 157-164
Author(s):  
Denis V. Zaslavsky ◽  
A. A. Sidikov ◽  
A. I. Sadykov ◽  
I. N. Chuprov ◽  
D. V. Kozlova ◽  
...  
Keyword(s):  
Histopathological Examination ◽  
Histological Study ◽  
Correct Diagnosis ◽  
Clinical Manifestations ◽  
Morphological Characteristics ◽  
Final Diagnosis ◽  
Response To Therapy ◽  
Lichen Ruber Planus ◽  
Microscopic Features ◽  
Classic Form

Background: Lichen ruber planus (LP) and lichenoid skin reaction (LSR) are clinically and histologically similar. The performance of histological diagnosis in these diseases remains controversial. Materials and methods: We prospectively studied 33 patients with clinical manifestations and histological signs of the classic form of LP and LSR to assess the accuracy of an isolated histological LP and LSR examinations and to identify a variety of microscopic features. Each histological study was conducted by a pathomorphologist, who was blinded to the patients clinical characteristics and diagnosis. Results: Isolated histopathological examination made it possible to make a correct diagnosis in 25 (75%) of 33 patients: in particular, the diagnosis of LRC was established in 10 (30%), CPL-in 15 (45%) cases. Based on a combined assessment of histological and clinical data and response to therapy, the final diagnosis was established in 30 (91%) of the 33 patients who were divided into two groups. The first group comprised 18 patients diagnosed with LSR, and the second group comprised 12 patients diagnosed with the classic form of LP. Conclusions: Through this investigation, some differences in these diseases based on their clinical and pathomorphological features were identified. The diseases were characterized by different typical localizations and lesion sizes. The pathomorphology of both diseases is represented by lichenoid type of interface dermatitis.

Next generation sequencing panel in undifferentiated autoinflammatory diseases identifies patients with colchicine-responder recurrent fevers

Rheumatology ◽  
2019 ◽  
Vol 59 (2) ◽  
pp. 344-360 ◽  
Author(s):  
Riccardo Papa ◽  
Marta Rusmini ◽  
Stefano Volpi ◽  
Roberta Caorsi ◽  
Paolo Picco ◽  
...  
Keyword(s):  
Next Generation Sequencing ◽  
Clinical Manifestations ◽  
Final Diagnosis ◽  
Chronic Inflammatory Disease ◽  
Autoinflammatory Diseases ◽  
Molecular Diagnostic ◽  
Recurrent Fever ◽  
Next Generation ◽  
Next Generation Sequencing Ngs ◽  
Generation Sequencing

Abstract Objectives The number of innate immune system disorders classified as systemic autoinflammatory diseases (SAID) has increased in recent years. More than 70% of patients with clinical manifestations of SAID did not receive a molecular diagnosis, thus being classed as so-called undifferentiated or undefined SAID (uSAID). The aim of the present study was to evaluate a next-generation sequencing (NGS)-based clinically oriented protocol in patients with uSAID. Methods We designed a NGS panel that included 41 genes clustered in seven subpanels. Patients with uSAID were classified into different groups according to their clinical features and sequenced for the coding portions of the 41 genes. Results Fifty patients were enrolled in the study. Thirty-four patients (72%) displayed recurrent fevers not consistent with a PFAPA phenotype. Sixteen patients displayed a chronic inflammatory disease course. A total of 100 gene variants were found (mean 2 per patient; range 0–6), a quarter of which affected suspected genes. Mutations with a definitive diagnostic impact were detected in two patients. Patients with genetically negative recurrent fevers displayed a prevalent gastrointestinal, skin and articular involvement. Patients responded to steroids on demands (94%) and colchicine, with a response rate of 78%. Conclusion Even with a low molecular diagnostic rate, a NGS-based approach is able to provide a final diagnosis in a proportion of uSAID patients with evident cost-effectiveness. It also allows the identification of a subgroup of genetically negative patients with recurrent fever responding to steroid on demand and colchicine.

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