Analysis of immunophenotypic features in hyaline vascular type Castleman disease.

BACKGROUND: Studies suggested that the immunophenotype of hyaline vascular type Castleman disease (HVCD) lacked characteristics, which was easy to be confused with other diseases. METHODS: From January 2010 to June 2022, 17 cases of HVCD were selected from the Department of Pathology of Shaanxi Provincial People's Hospital and the Department of Pathology of Shaanxi Provincial Cancer Hospital. 13 cases of reactive hyperplastic lymph nodes (RHL) and 11 cases of follicular lymphoma (FL) were selected as the control group. All cases were performed CD3, CD20, CD21 and BCL2 immunohistochemical staining. RESULTS: (i) In 17 cases of HVCD, the negative area of BCL2 of germinal center was significantly smaller than the negative area of CD3 of germinal center. However, in 13 cases of RHL, the negative area of CD3 of germinal center was basically consistent with the negative area of germinal center of BCL2 of germinal center. In 11 cases of FL, in neoplastic follicles, the negative area of CD3 was basically consistent with the positive area of BCL2. The difference between HVCD group and other two groups of diseases was statistically significant (P < 0.05). (ii) In 17 cases of HVCD, the negative area of BCL2 of germinal center was significantly smaller than the follicular dendritic cell (FDC) meshworks expressed by CD21. However, in 13 cases of RHL, the FDC meshworks expressed by CD21 were basically consistent with the negative area of BCL2 of germinal center. In 11 cases of FL, in neoplastic follicles, the FDC meshworks expressed by CD21 was basically consistent with the positive area of BCL2. The difference between HVCD group and other two groups of disease was statistically significant (P < 0.05). CONCLUSIONS: HVCD has unique immunophenotypic characteristics. The negative area of BCL2 of germinal center is significantly smaller than the negative area of CD3. The negative area of the BCL2 of germinal center is significantly smaller than the FDC meshworks expressed by CD21. These two immunophenotypic features in HVCD are very important in diagnosis and differential diagnosis.

[Diagnosis and surgical treatment of a patient with Castleman disease]. / Diagnostika i khirurgicheskoe lechenie patsienta s bolezn'yu Kastlemana: klinicheskoe nablyudenie.

Castleman disease (CD) is a benign lymphoproliferative disease. Small prevalence and diverse clinical course of disease makes it difficult to standardize diagnostics and treatment. Currently, the number of CD patients has increased with improvement in the quality of examination. Therefore, differential diagnosis of this disease is important. We present a young patient with CD and retroperitoneal non-organ neoplasm. Despite a thorough preoperative examination, the final diagnosis was established only after histological examination of surgical specimen. We discuss the diagnosis and surgical treatment of a patient with unicentric type of CD.

Multicentric Castleman Disease: The Hyaline Vascular Type Without HIV Association in a Young Male With Cutaneous Manifestation and Renal Impairment.

Castleman disease (CD), a heterogenous lymphoproliferative disorder resulting from immune dysregulation, is a very rare disease in clinical practice. The clinical spectrum of Castleman disease is wide and its treatment options are mostly based on case reports and case series. To date, two clinical and four histological types have been described. It has recently been successfully demonstrated that the pathogenesis of multicentric Castleman disease (MCD) has a direct association with human immunodeficiency virus (HIV) and human herpes virus 8 (HHV-8) infection which is why further studies are necessary. Here, we report an unusual case of MCD not associated with HIV and having a histological diagnosis of the hyaline vascular type that presented with acute renal impairment and subcutaneous abnormal lymphatic proliferation.

Case report: Castleman's disease involving the renal sinus resembling renal cell carcinoma.

Introduction: Castleman's disease (CD) is a rare benign lymphoproliferative disease that frequently involves the mediastinal thorax and the neck lymph nodes. It rarely affects extrathoracic presentations, with even fewer presentations in the renal sinus. Patient concerns: In this report, we present a case of a 40-year-old woman with no significant past medical history who presented Castleman's disease arising in the renal sinus. Diagnosis and interventions: The patient visited our hospital with the chief complaint of left renal sinus lesion after renal ultrasonography by regular physical examination. Subsequent abdominal computed tomography urography revealed a soft tissue mass with heterogeneous obvious enhancement in the sinus of the left kidney, which was suspected to be a renal malignant tumor. Hence, the patient underwent a left radical nephrectomy. Histological examination revealed hyperplastic lymphoid follicles in the renal sinus and was finally diagnosed as Castleman's disease of the hyaline vascular type. Outcomes: Five days after the surgery procedure, the patient was discharged. Conclusion: Due to the low incidence of Castleman's disease in renal sinus, there is a strong likelihood of missed diagnosis or misdiagnosis, and it is, therefore, important to be aware of the risk. Heightened awareness of this disease and its radiographic manifestations may prompt consideration of this diagnosis. Therefore, we explored the radiologic findings to find out some radiologic features suggesting this condition to help clinicians to schedule nephron-sparing surgery in the future.

Retroperitoneal fibrosis, a rare entity with urorenal and vascular subtypes - preliminary data.

INTRODUCTION: Retroperitoneal fibrosis (RPF) is a rare disease associated with the formation of hard inflammatory and fibrous tissue in the retroperitoneum. Taking into consideration the fact that RPF is a rare disease with different subtypes, we compared the basal clinical and biochemical characteristics of the vascular and urorenal subtypes. PATIENTS AND METHODS: From January 2005 until December 2021, 27 patients were identified as vascular subtype (18 males) and 11 as urorenal subtype (9 males). RESULTS: Patients with a primary urorenal origin had significantly worse kidney function as reflected by serum creatinine and eGFR (both p < 0.001); they also had higher serum cholesterol (p < 0.01). Hypertension, diabetes, hyperlipidemia and nicotinism were significantly more prevalent in vascular subtype (all p < 0.001). CONCLUSION: Vascular subtype is more prevalent in our study with more cardiovascular risk factor present. Due to the diversity of symptoms, diagnosis of RPF becomes a challenge for specialists as well as therapy.

Hippocampal calcification and its effects on cognitive function and symptoms in dementia.

BACKGROUND: Hippocampal calcification (HC), highly prevalent in older people, has not attracted attention until recently. Despite its potential effects on cognition and behaviour, and its possible impact on the diagnosis and severity of dementia, it has not been investigated. This study aimed to evaluate the prevalence of HC and its influence on cognition and behavioural symptoms in patients with dementia. METHODS: Data from consecutive patients who visited a medical centre for dementia, for the first time between April 2016 and September 2018, were extracted and analysed. These data included the patients' demographics, the presence of HC and hippocampal thickness as measured on computed tomography, the diagnosis of dementia and its type, cognitive function measured using the Mini-Mental State Examination and the Clock Drawing Test, and the chief complaints or symptoms prompting the visit. RESULTS: A high incidence of HC (85/267 patients) was observed. There was no significant difference in the ages of patients with and without HC. Patients with HC had higher cognitive function than those without HC at their first visit. This result was contrary to our expectations as it was not explained by the chief complaints recorded at the first visit. CONCLUSIONS: Our study showed a high prevalence of HC in older patients with dementia. Patients with HC had better cognitive function than did those without HC during their first hospital visit. This study suggests that HC may not affect the cognitive functions related to dementia. However, further research is needed to evaluate the long-term consequences of dementia with HC.

Two closely spaced missense COL3A1 variants in cis cause vascular Ehlers-Danlos syndrome in one large Chinese family.

Vascular Ehlers-Danlos syndrome (vEDS) is a rare and severe hereditary connective tissue disease arising from a mutation in the type III collagen alpha I chain (COL3A1) gene, with a poor prognosis due to exceptional vascular ruptures and premature death. Herein, starting from a 36-year-old Chinese male patient with a complaint of upper abdominal pain, we collected clinical data of and performed a genetic analysis of a total of 20 family members. We identified two closely spaced COL3A1 missense variants in cis, p.Leu734Phe (c.2199_2200TC>AT) and p.Gly741Ser (c.2221G>A), as the cause of vEDS in this family. p.Gly741Ser, a glycine substitution mutation, has been previously reported, whereas p.Leu734Phe, a non-glycine substitution mutation, is novel. We analysed their independent and combined effects on the COL3A1 level in transfected skin fibroblast cells by means of Western blotting. We found that both variants independently led to a reduced COL3A1 level and, when combined, led to an even more reduced COL3A1 level compared to the wild type. Thus, each missense variant can be independently classified as a pathogenic variant, albeit with a synergetic effect when occurring together. Moreover, our genetic findings provide an explanation for four previous sudden deaths and identified two high-risk carriers in the family.

Clinical and Pathological Characteristics of Hyaline-Vascular Type Unicentric Castleman Disease: A 20-Year Retrospective Analysis.

The first case of hyaline vascular type of unicentric Castleman disease (HV-UCD) was reported more than six decades ago. Since patients with HV-UCD are often asymptomatic and this condition is generally discovered incidentally on imaging tests, most of the previous reports were of mediastinal origin detected by chest radiography. In recent years, improved access to imaging modalities has provided new insights in the diagnosis of this condition. In this study, we reviewed the detailed clinical and pathological findings of 38 HV-UCD cases (20 males and 18 females, mean age: 42.8 years). The most common site involved was the abdominal cavity (34.2%), followed by mediastinum (23.7%) and retroperitoneum (15.8%). In the abdominal cavity, mesenteric origin was the most common. The mean size of masses was 4.8 cm. Pathologically, thick hyalinized collagen fibers surrounding large blood vessels and calcification were observed (81.6% and 23.7%, respectively). Multinucleated giant cells resembling Warthin-Finkeldey cell were also observed in occasional cases (23.7%). This is a unique paper that summarizes detailed clinical and pathological findings of a large series of a rare disease. The clinical information presented in this paper is more plausible than previous views and is useful for accurate diagnosis and understanding of the disease.

Hyaline Vascular Type of Castleman Disease: Diagnostic Pitfalls on Cytology and Its Clinical Relevance.

Castleman disease (CD) is an uncommon cause of lymphadenopathy. The role of fine-needle aspiration cytology (FNAC) as a diagnostic modality in this disease is not well established. Cytological features of CD have a considerable overlap with many reactive conditions. It has subtle morphological features; which if overlooked, may miss the diagnosis. A two-year-old girl presented with cervical lymphadenopathy. FNAC of the cervical lymph node showed features of granulomatous lymphadenitis. Excision biopsy revealed the hyaline vascular type of CD. Cytological smears were reviewed carefully and revealed indicators of CD. These included capillary fragments with adherent reactive lymphoid cells, plump endothelial cells and pale pink material admixed with germinal center cells. The collections of plump endothelial cells had been misinterpreted as granulomas previously. This report highlights the subtle cytomorphological pointers of CD. Careful scrutiny for these features could aid the cytologist in differentiating CD from other reactive and neoplastic disorders, thus avoiding cytodiagnostic pitfalls. This case study reiterates an important fact that for a lymph node lesion, histopathology plays a crucial role in differentiating mimickers and renders an accurate diagnosis.

Establishment of visual assessment for the severity of dark circles in Chinese Han women.

OBJECTIVE: To establish a visual assessment scale for the severity of dark circles' pigmented and vascular type in Chinese women. MATERIALS AND METHOD: A total of 269 healthy Chinese women from Shanghai with different degrees of dark circles', both pigmented and vascular types, were evaluated by visual assessment. Photographs of their dark circles were analyzed by image analysis. RESULTS: The visual assessment evaluation on classification and severity showed a favorable agreement between the successive measured results. Significant differences from very slight to severe dark circles for pigmented type and vascular type were observed. The severity level by visual assessment was significantly positively correlated with ΔE values while negatively correlated with ΔL values (P < .01) in both pigmented and vascular types. Besides, Δa values of vascular type were significantly positively correlated with the ΔE values for dark circles' vascular type. Values between ΔE and ΔL also showed a significant negative correlation (P < .01). CONCLUSION: The five-point visual assessment scale for dark circles of vascular and pigmented types was verified and proved to have good repeatability. The image analysis's objective result proved significant and consistent with the visual assessment and color parameters. This scale could be a useful and effective tool in diagnosing dark circles' severity.

Castleman's disease of the left parotid gland: a case report.

Castleman's disease is a very rare heterogeneous group of lymphoproliferative disorders characterized by non-neoplastic growths. It is unknown about the pathophysiology of the Castleman's disease. Previous studies demonstrated that Castleman's disease can be divided into two groups according to clinical classification, including unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). The hyaline vascular type is most common in the head and neck, and is abbreviated as UCD. In the present case, a woman complained that a mass in her parotid gland was growing and it was painless seven months ago. The computed tomography (CT) showed that the superficial lobe of the parotid gland on the left had an elliptical soft tissue density shadow, about 2.5×3.5 cm, with clear boundaries and no obvious abnormalities in the surrounding bone. The CT scan showed no obvious abnormalities in the shape and density of the right parotid and bilateral submandibular glands. After the operation, combined with the results of immunohistochemistry, the final diagnosis was Castleman tumor. The patient recovered smoothly, after the operation and during follow-up. The patient maintained good health without recurrence or metastasis.

Outcomes of vitrectomy for retinal detachment in a patient with Ehlers-Danlos syndrome type IV: a case report.

BACKGROUND: The Ehlers-Danlos syndrome (EDS) is a group of connective tissue disorders characterized by fragile blood vessels and an increased tendency for bleeding and scarring. Here, we report the outcome of a pars plana vitrectomy for the treatment of rhegmatogenous retinal detachment in a patient with EDS type IV (vascular type). CASE PRESENTATION: A 40-year-old Slovenian man with high myopia, unilateral bullous retinal detachment, and vitreous hemorrhage was referred for surgery. The patient had a history of colon perforation, muscle and arterial rupture in both lower limbs, and recurrent shoulder joint luxations. Genetic testing revealed a pathogenic mutation in the COL3A1 gene. The patient underwent a 25-gauge three-port pars plana vitrectomy. The tendency for bleeding during surgery was prevented by endodiathermy applied to the edges of the retinal breaks. Endolaser photocoagulation was performed under air. The surgical procedure was completed with the injection of gas tamponade, followed by the patient remaining for a few days in a face-down position. Mild postoperative vitreous hemorrhage was resorbed in first week after the surgery. Postoperative extensive pigment dispersion on the posterior lens face persisted for several weeks. After the gas tamponade had resorbed, the retina was flat and remained attached during the follow-up period. Eight months after the surgery, visual acuity continued to improve from a preoperative 6/38 to 6/6.6 (Snellen chart) at the last checkup. CONCLUSION: A small-gauge pars plana vitrectomy with gas tamponade and laser photocoagulation under air may successfully achieve reattachment of the retina in patients with high myopia with EDS type IV and restore visual acuity.

A case of vascular Ehlers-Danlos syndrome with a ruptured hepatic artery after surgical treatment of peritonitis caused by the perforation of the colon.

BACKGROUND: Ehlers-Danlos syndrome (EDS) is an inherited disorder that causes connective tissue fragility. The vascular type of EDS (vEDS) caused by defective collagen type III production accounts for 5%-10% of all EDS cases. Patients can develop gastrointestinal or arterial ruptures, which cause poor prognosis. We report a case of a patient who experienced colonic rupture, which was immediately followed by arterial rupture. CASE PRESENTATION: A 40-year-old man who had been genetically diagnosed with vEDS 6 years previously was admitted to our hospital with ischemic colitis. After 3 days of conservative treatment, his abdominal pain worsened, and computed tomography (CT) revealed free air in the abdominal cavity. Pan-peritonitis due to perforation of the sigmoid colon was diagnosed. Intraperitoneal lavage and drainage and Hartmann's operation were urgently performed. Because the patient had confirmed vEDS, we performed the surgery in a protective manner. The postoperative course was initially good, and he was transferred to the general ward 3 days after surgery. However, 5 days after surgery, massive intra-abdominal hemorrhage suddenly occurred, and contrast-enhanced CT showed an aneurysm in the common hepatic artery that had ruptured; this aneurysm was not present before surgery and was far from the surgical field. Although we considered an emergency operation, the patient suddenly experienced cardiac arrest and was unresponsive to resuscitation. CONCLUSIONS: In cases of vEDS, vascular rupture can occur immediately after surgery for intestinal rupture. We recommend paying special attention to vascular complications in patients in their forties, as such complications are the most common causes of death.

Surgical resection for pelvic retroperitoneal Castleman's disease: A case report and review literature.

Castleman's disease (CD) is a rare atypical lymphoproliferation disorder first reported in 1954. Clinically, CD is classified as unicentric or multicentric CD based on anatomical distribution. Unicentric CD primarily affects the mediastinum, and rarely affects the retroperitoneal location. The standard treatment for unicentric CD is complete surgical resection; however, this can be complicated by a high degree of attachment with other organs or hypervascularity. Preoperative angiography and embolization of the arteries that feed the problematic mass can reduce intraoperative bleeding in cases of CD with hypervascularity. In the present case report, a 44-year-old man who was found to have a pelvic retroperitoneal mass with calcification based on abdominal imaging results is discussed. Due to the hypervascularity of the mass, preoperative embolization was performed. The mass was completely resected without any complications. Additionally, a review of the literature on pelvic CD and preoperative embolization of CD was performed to provide an up-to-date reference on the management and outcomes of patients with CD.

Endoplasmic reticulum stress and collagenous formation anomalies in vascular-type Ehlers-Danlos syndrome via electron microscopy.

Vascular-type Ehlers-Danlos syndrome (vEDS) is an autosomal-dominant inherited disorder caused by a deficit in collagen III. It results from heterogeneous mutations in the α1 collagen III gene (COL3A1) and is associated with life-threatening complications, even in younger patients. However, the details of the pathogenesis underlying the COL3A1 mutation causing vEDS remain unclear. Here, we focus on anomalies in collagen fiber size and the endoplasmic reticulum (ER) stress response in patients with vEDS using electron microscopy (EM). We discovered that although the infants did not have vEDS, collagenous formations were similar to their samples in vEDS. Moreover, we examined the expression of activating transcription factor 6 (ATF6) as an ER stress marker and cartilage oligomeric matrix protein (COMP) as a binding partner protein for collagen fibrils in the dermis and COL3A1. The expression levels of ATF6 in the vEDS group were significantly higher than in infants and controls; COMP and COL3A1 levels were significantly lower. The fragile collagen fibrils in vEDS might form as a result of ER stress and that small, newly formed collagen fibrils may appear. This research revealed a novel prospect regarding an issue that has been unclear for a long time, which is the reason for the abnormal sizes of collagenous fibrils in vEDS.

Celiprolol Treatment in Patients with Vascular Ehlers-Danlos Syndrome.

OBJECTIVE: Vascular Ehlers-Danlos syndrome (vEDS) is a rare monogenetic disease caused by pathogenic variants in procollagen 3A1. Arterial rupture is the most serious clinical manifestation. A randomised controlled trial, the Beta-Blockers in Ehlers-Danlos Syndrome Treatment (BBEST) trial, reported a significant protective effect of the beta blocker celiprolol. The aim was to study the outcome of celiprolol treatment in a cohort of Swedish patients with vEDS. METHODS: Uppsala is a national referral centre for patients with vEDS. They are assessed by vascular surgeons, angiologists, and clinical geneticists. Family history, previous and future clinical events, medication, and side effects are registered. Celiprolol was administered twice daily and titrated up to a maximum dose of 400 mg daily. Logistic regression was used to analyse predictors of vascular events. RESULTS: Forty patients with pathogenic sequence variants in COL3A1 were offered treatment with celiprolol in the period 2011-2019. The median follow up was 22 months (range 1-98 months); total follow up was 106 patient years. In two patients, uptitration of the dose is ongoing. Of the remaining 38, 26 (65%) patients reached the target dose of 400 mg daily. Dose uptitration was unsuccessful in six patients because of side effects; one died before reaching the maximum dose, and five terminated the treatment. Five major vascular events occurred; four were fatal (ruptured ascending aorta; aortic rupture after type B dissection; ruptured cerebral aneurysm; and ruptured pulmonary artery). One bled from a branch of the internal iliac artery, which was successfully coiled endovascularly. The annual risk of a major vascular event was 4.7% (n = 5/106), similar to the treatment arm of the BBEST trial (5%) and lower than in the control arm of the same trial (12%). No significant predictor of vascular events was identified. CONCLUSION: Treatment with celiprolol is tolerated in most patients with vEDS. Despite fatal vascular events, these observations suggest that celiprolol may have a protective effect in vEDS.

Current treatment of vascular Behcet′s disease / 国际外科学杂志

Behcet′s disease is a chronic, multisystemic, inflammatory disease characterized by recurrent episodes of mucous membranes, eyes, musculoskeletal, blood vessels, central nervous system, and gastrointestinal tract.The treatment of Behcet′s disease varies according to the degree of organ involvement, gender and age of the patient, and there is no standard treatment. Behcet′s disease can be divided into vascular type, gastrointestinal type and neural type. Vascular type often leads to high mortality and disability rate.Glucocorticoids, azathioprine and cyclophosphamide are still recommended as first-line treatments for vascular Behcet′s disease.However, with the use of tumor necrosis factor inhibitors, they are an acceptable option for the treatment of refractory vascular Bezier′s disease.This article reviews the current treatment of vascular Behcet′s disease.

[Clinicopathological Features of Follicular Dendritic Cell Sarcoma].

Objective To explore the clinicopathological and immunohistochemical characteristics of follicular dendritic cell sarcoma(FDCS)and the expressions of IgG and IgG4. Methods We retrospectively analyzed the clinicopathological and immunohistochemical data of 9 pathologically confirmed FDCS cases in Peking Union Medical College Hospital from January 2005 to December 2018.Immunohistochemical staining of IgG and IgG4 were performed,and Epstein-Barr virus(EBV)-encoded RNA(EBER)in situ hybridization were carried out. Results Nine cases of FDCS included 4 men and 5 women aged 16-53 years [mean(38.2±9.7)years].The clinical manifestations included masses,lymph node enlargement,rash,and fever.The tumors were located in lymph node,retroperitoneal region,adrenal gland,neck,axillary region,and liver,respectively.Ultrasound showed clear boundary cystic or solid mass with maximum diameters of 1.5-15.0 cm.Microscopically,the spindle tumor cells were arranged in solid and storiform patterns with abundant and slightly stained cytoplasm,vacuolated nuclei,and small nucleoli.The mitosis was 1-3/10 high power fields,and necrosis was found in 5 cases.Immunohistochemically,the tumor cells were positive for CD21(6/9),CD35(6/9),and CD23(7/9). Conclusions FDCS is a rare malignant tumor,which is easy to be missed.The combination of CD21,CD35,and CD23 is helpful for diagnosis.Hyaline-vascular type Castleman's disease may be the precursor of FDCS,and there may be only a small number of IgG4-positive plasma cells in FDCS.Surgical resection remains the main treatment for FDCS.

New local treatments for different types of melasma: Vascular type vs nonvascular type. A randomized polycentric study.

Melasma is an acquired circumscribed hyperpigmented disorder seen mainly on the malar area of face and other parts of body. We evaluated the safety and efficacy of two different creams for melasma. Sixty volunteers with melasma were evaluated in this study, divided in three groups: A, B, and C. Patients in groups A and B were treated with same creams with the exception of addition of Tranexamic acid in cream B. Patients in group C were treated with placebo cream containing petrolatum only, respectively, twice daily for 10 weeks. Melasma Areas and Severity Index (MASI) score, melanin index (MI) and adverse events were evaluated every 4 weeks. MASI scores declined significantly in groups A and B compared to group C (P < .05). Cream B, containing tranexamic acid, resulted superior to cream A in subjects with hypervascular melasma. No adverse reactions were observed in all groups. Both active creams are safe and effective for melasma and should be selected according to normal or hypervascular type of melasma. Cream B (containing tranexamic acid) should be prescribed instead of cream A (not containing tranexamic acid) to subjects with hypervascular melasma.