Preoperative Direct Puncture Embolization of Castleman Disease of the Parotid Gland: A Case Report.

BACKGROUND: Castleman disease (CD) is an uncommon benign lymphoproliferative disease characterized by hypervascular lymphoid hyperplasia. We present a unique case of unicentric CD of the parotid gland treated by preoperative direct puncture embolization. CASE PRESENTATION: A 27-year-old female patient was admitted for a right neck mass. Ultrasound examination and MRI scan documented a hypervascular mass within the right parotid gland. Preoperative embolization was performed by direct puncture technique: a needle was inserted into the core of the mass under both ultrasound and fluoroscopic guidance and SQUID 12 was injected into the mass under fluoroscopic control, achieving a total devascularization. CONCLUSION: Preoperative direct puncture embolization was safe and effective and provides excellent hemostatic control during the surgical operation, limiting the amount of intraoperative bleeding.

A case report: Castleman disease treated by the trinity of internal and external treatment of "Fuzheng, phlegm-resolving, and detoxification method".

RATIONALE: Castleman disease (CD) was first included in the CSCO lymphoma diagnosis and treatment guidelines in 2021. Its diagnosis relies on lymph node pathological examination. Observation, surgical resection of the lesion, radiotherapy, chemotherapy, and medical therapy (e.g., rituximab, siltuximab, steroids) can be used. Due to the traumatic, incurable, and recurrent nature of surgical treatment, drug therapy has many side effects and is expensive. Exploring effective traditional Chinese medicine (TCM) comprehensive treatment methods for this disease is important and necessary. PATIENT CONCERNS: The main symptom was recurrent lymphadenopathy, which had been surgically removed 5 times in the past. This time, lymph node enlargement occurred again, and the local hospital recommended surgical resection again. The patient could not tolerate another surgical treatment. Other targeted treatments are not available due to financial constraints. DIAGNOSES: The case was diagnosed as CD by pathological examination, which is an important basis for the diagnosis of this disease. INTERVENTIONS: The patient was treated with surgery in the early stage several times, later came to our hospital for the trinity of TCM integrated treatment program, which combines oral TCM with external application of TCM and intravenous drip of TCM as a syndrome of positive deficiency and phlegm-toxin internalization, and the therapeutic principle. OUTCOMES: After nearly 3 years of comprehensive treatment with TCM, the enlarged lymph nodes could not be touched, and there was no fatigue, fever, or weight loss. During this period, the patient did not undergo surgery, chemotherapy and other western medicine treatment, and lived a normal life. It not only met the patient's expectation but also confirmed that the TCM treatment was indeed effective. LESSONS: This case report confirms that TCM is safe and effective in the treatment of CD, which is worthy of promotion. In clinical practice, the individualized treatment for the patient, the duration of treatment, and the different disease states also affect the treatment outcome.

Idiopathic multicentric Castleman disease: An update in diagnosis and treatment advances.

Idiopathic multicentric Castleman disease (iMCD) is a rare disease, and it is likely underdiagnosed because of the heterogeneity of clinical manifestations and laboratory findings. While the disease leads to significant morbidity and mortality, its causes are not yet fully elucidated. There have been significant advances in diagnosis and treatment of iMCD in the past decade, including the approval of the anti-IL-6 antibody siltuximab. In this review, we provide an update of the many new developments and publications surrounding iMCD.

Clinical features and outcomes in patients with human immunodeficiency virus-negative, Castleman's disease: a single medical center study in Tunisia.

INTRODUCTION: Castleman's disease (CD), known as angiofollicular lymph node hyperplasia, is an uncommon condition. The two most common histological subtypes are hyaline vascular and plasma cell. We performed a retrospective analysis to define the clinic-pathological features and survival of CD, which is quite rare focusing on the particularities of our series with a review of the recent literature. METHODS: This is a retrospective study conducted in the department of internal medicine of Hedi Chaker hospital in Sfax, Tunisia over 25 years. The disease was histologically confirmed in all patients. For each file, we collected a set of data by filling in a pre-designed form. RESULTS: 18 patients were included. There were 8 men and 10 women with a mean age of 42.8 years. CD was monocentric in 5 cases (28%) and multicentric in 13 cases (72%). Clinically, peripheral adenopathy was present in 77.7% of patients and deep adenopathy in 72.2%. Systemic signs were found in 13 patients, including general condition (4.4%), fever (16.6%), serositis (27.7%), and skin involvement (33.3%). A biological inflammatory syndrome accompanied the clinical picture in 66% of patients. Abnormalities in the blood count were found in 12 cases (66%), with anemia in 11 cases, thrombocytosis in 3 cases, and hypereosinophilia in 3 cases. Cutaneous Kaposi's sarcoma was associated with Castleman's disease in 2 cases, Hodgkin's lymphoma, angioimmunoblastic T-cell lymphoma, and lymph node T-cell lymphoma were found in 1 case respectively. 3 of the patients had associated connective tissue diseases such as Sjögren's syndrome in 2 cases and rheumatoid arthritis in 1 case. HHV8 serology was positive in 1 case with a multicentric plasma cell form. Histologically, the plasma cell form represented 50% of cases, hyaline-vascular (39% of cases), and mixed (11% of cases). Therapeutically, high-dose corticosteroid therapy was initiated in 13 cases. As a second-line treatment, MOPP chemotherapy was used in 1 case due to transformation into Hodgkin's lymphoma, and biotherapy (rituximab) was used in 2 cases in the multicentric form. Surgical removal of superficial adenopathy was performed in 2 patients with monocentric CD. CONCLUSION: : Castleman's disease (CD) is a non-malignant lymphoproliferation of localized or multicentric form with a wide and heterogeneous clinical spectrum. Diagnosis can be difficult due to the lack of clinical and radiological specificity. Management depends on the clinical form involving surgical and/or medical management.

Retrospective cohort evaluation of non-HIV Castleman disease from a single academic center in Beijing, China.

The purpose is to ascertain the clinical impact of Castleman disease (CD) by reassessment of the real-world data from Peking University First Hospital (PKUFH). The results will contribute to the standardization of diagnosis and treatment on CDs. Based on the last 15-year retrospective real-world data from Peking University First Hospital (PKUFH), we reclassified and re-evaluated the clinical and pathological information of patients with pathologically suspected diagnosis of CD. A total of 203 patients were included in our study, in which the diagnosis of CD was confirmed in 189 cases, including 118 patients with unicentric CD (UCD, n = 118, 62.4%) and 71 patients with multicentric CD (MCD, n = 71, 37.6%). A total of 44.1% (n = 52) of UCDs in our cohort were complicated with paraneoplastic pemphigus (PNP). The treatment of UCD is primarily surgical, with a 5-year overall survival (OS) of 88.1%. Patients with PNP had a poorer prognosis than those without PNP (82.9% (95% CI 123-178) vs 92.8% (95% CI 168-196), log-rank p = 0.041). The rate of concurrent systemic symptoms was 74.6% (n = 53), and renal involvement occurred in 49.3% (n = 35) MCD patients. The MCD treatments were mainly chemotherapy regimens, with a 5-year OS of 77.6% (95% CI, 143-213). Patients with UCD demonstrate a better overall prognosis than patients with MCD. But the prognosis of those complicated with PNP was poor. The differential diagnosis of MCD is extensive. MCD treatment in China is heterogeneous. The inaccessibility of anti-IL-6-targeted drugs in China may contribute to the poor prognosis for patients with MCD.A preprint has previously been published (Guo et al. 34).

Castleman disease- demographics, associations, and outcomes: an analysis of adult 791 cases.

BACKGROUND: Castleman disease (CD), also known as angiofollicular lymph node hyperplasia or large lymph node hyperplasia, is a rare medical condition. Despite its rarity, it exhibits diverse clinical presentations and outcomes, which pose challenges for comprehensive understanding and management. This study aims to shed light on the demographics, associations, and outcomes of CD by conducting a retrospective analysis. METHODS: The National Inpatient Sample (US) was used to identify patients with the diagnosis of Castleman disease using ICD-10 diagnosis code D47.Z2, during the years 2016-2019. Data was collected on demographics, associated diagnoses, treatments and outcomes. Data analysis was performed using STATA Version 17, College Station, TX: Stata Corp LLC. RESULTS: Our study identified 791 hospitalizations involving adult CD patients. The mean age of these patients was 52.4 years, with a male predominance (56.1%). Whites comprised the largest racial group affected (50.1%). Most patients were covered by Medicare (39.6%). The majority received treatment in urban teaching hospitals (84.0%) and large-bed size facilities (62.5%). In-hospital mortality was low at 2.8%, with an average length of stay of 7.5 days and average total charges of $109,308. Common associations included acute kidney injury (27.0%), congestive heart failure (17.1%), sepsis (16.4%), and acute respiratory failure (12.6%). Hematological and lymphatic associations featured anemia (47.5%), thrombocytopenia (12.2%), and other conditions. Red blood cell transfusions were administered to 11.1% of patients. CONCLUSION: This study contributes valuable insights into CD, a rare and clinically heterogeneous disease. It underscores the importance of recognizing its associations and complications. Additionally, it highlights the need for further research and improved diagnostic and treatment guidelines to address the complexity of this condition.

Clinical features and treatment outcomes of Castleman disease in children: a retrospective cohort in China.

Castleman disease (CD) is a rare lymphoproliferative disorder of undetermined etiology. Unicentric CD (UCD) and multicentric CD (MCD) are two phenotypes of CD diagnosed by the histopathology of lymph nodes. We attempted to describe a pediatric CD cohort to optimize the management of this disease. We reviewed the medical records of pediatric patients diagnosed with CD between April, 2004, and October, 2022, at the Children's Hospital of Fudan University. Prognosis information was collected in January, 2023, by telephone inquiry. Twenty-two patients with UCD and 2 patients with MCD were identified, all with hyaline vascular (HV) type. The median ages at diagnosis were 10.75 years (IQR 8, 12.81) for UCD and 14.42 years (IQR 13.42, 15.42) for MCD. The most common lesion location of UCD was the neck (9/22, 40.91%) and abdomen (9/22, 40.91%). Systematic symptoms occurred on 10/22 (45.45%) patients with UCD and 1/2 (50%) patients with MCD, and abnormal laboratory indexes were detected in both. Resection and biopsy were performed on all patients. One out of two patients with MCD also received rituximab for upfront therapy. After a median of 4 years (IQR 1.5, 6) of follow-up time, the overall survival was 100% and the complete remission rate in UCD was 63%. There was no relapse or progression. CONCLUSIONS: Our series demonstrated that HV-UCD was the most common type in children. Resection and biopsy were used for both deterministic diagnoses and treatments. Despite the high possibility to develop systematic inflammation, children with CD showed promising outcomes. WHAT IS KNOWN: • Castleman disease is a rare lymphoproliferative disorder with limited cohort studies, especially in pediatrics. • The ubiquity of delayed confirmations and misdiagnoses points to a lack of knowledge about etiology and characteristics, which is a prerequisite for novel therapeutics. WHAT IS NEW: • We retrospectively reviewed and analyzed the clinical and pathological symptoms, laboratory and imaging features, and treatment outcomes of a Chinese pediatric cohort with Castleman disease. • Our work may improve the recognition and optimize the management of this rare disease in children.

[POEMS-syndrome]. / POEMS-sindrom.

POEMS-syndrome (polyneuropathy - P, organomegaly - O, endocrinopathy - E, M-protein - M, skin changes - S) is a paraneoplastic syndrome caused by underlying dyscrasia of plasma cells. The main criteria of the syndrome are polyradiculoneuropathy, clonal proliferation of plasma cells, sclerotic bone lesions, elevated levels of vascular endothelial growth factor and the presence of Castleman disease. Additional signs include organomegaly, endocrinopathy, characteristic skin changes, edema of the optic disc, extravascular volume overload (edema) and thrombocytosis. The diagnosis is often made late, because the syndrome is rare and is often mistaken by specialists for other neurological disorders, most often for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We present two cases of POEMS-syndrome. The description is based on the principle of differential diagnosis with a number of similar neurological disorders. The goal facing the neurologist is to carry out the most complete diagnostic measures for early diagnosis, which further determines timely therapeutic tactics. Hematologists are engaged in specific therapy of POEMS-syndrome. A brief description of possible therapeutic options is presented. On the example of these cases, we demonstrate possible variants of the therapeutic response based on the developed system of risk stratification of patients with POEMS-syndrome.

[Clinical Features and Prognosis of Patients with Castleman's Disease].

OBJECTIVE: To analyze the clinical features and prognosis of patients with Castleman's disease (CD) and improve the diagnosis and treatment of CD. METHODS: Clinical data of patients diagnosed with CD by pathological biopsy in Gansu Provincial Hospital from January 2009 to November 2020 were retrospectively analyzed. According to clinical classification, the patients were divided into two groups: UCD (unicentric CD) group (n=20) and MCD (multicentric CD) group (n=9). The clinical manifestations, laboratory examination, treatment regimens, pathological examination and follow-up data were statistically analyzed. RESULTS: There were no significant differences in average age and gender ratio between UCD group and MCD group. In UCD patients, 80.0% were hyaline vascular type, and 20.0% were plasma cell type. In MCD patients, 33.3% were hyaline vascular type, 55.6% were plasma cell type, and 11.1% were mixed type. There was significant difference in pathological classification between the two groups (P=0.039). The UCD patients usually presented asymptomatic single lymph node enlargement with mild clinical symptoms, while the MCD patients were characterized by multiple superficial and deep lymph node enlargement throughout the body. The incidences of asthenia, splenomegaly, serous effusion in MCD group were higher than those in UCD group (P<0.05). Meanwhile, the incidences of anemia, hypoproteinemia, increased ESR, elevated serum globulin and elevated ß2-microglobulin were significantly higher than those in UCD group too (P<0.05). There was no significant difference in the incidences of abnormal WBC, PLT and elevated LDH between the two groups (P>0.05). Among 20 patients with UCD, 13 cases reached complete remission (CR), 1 case achieved partial remission (PR). Among 9 patients with MCD, 3 cases received CR and 4 cases received PR. CONCLUSION: Patients with CD requires pathological examination for diagnosis. Patients with UCD show mild clinical symptoms, good surgical treatment effect and good prognosis. Patients with MCD have diversified clinical manifestations and relatively poor prognosis, and these patients require comprehensive treatment.

TAFRO syndrome: A disease that known is half cured.

Thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly (TAFRO) syndrome is rare in clinical practice. It is a systemic inflammatory disease caused by a cytokine storm. Its clinical manifestations include thrombocytopenia, systemic edema, fever, bone marrow fibrosis, renal insufficiency, and organ enlargement. The high mortality rate of TAFRO syndrome is due to the difficulty of acquiring biopsy samples for diagnosis and the rapid disease progression. This disease is poorly understood by clinicians. Early detection, accurate diagnosis, and timely treatment play key roles in prolonging the survival of the patients. This review summarizes the latest progress in the pathogenesis, diagnostic criteria, and treatment regimens of TAFRO syndrome, aiming to help clinicians better understand TAFRO syndrome and improve its diagnosis and treatment.

Recent Advances in Castleman Disease.

BACKGROUND: Castleman disease (CD) encompasses a spectrum of rare disorders with characteristic histopathological features. Unicentric CD (UCD) is a benign, local hyperplasia of lymphoid tissue that is usually curable. Multicentric CD (MCD) manifests as a potentially life-threatening systemic disease with complex symptomatology which is mostly due to an overproduction of interleukin-6 (IL-6) or dysregulation of IL-6-related signaling pathways. From a therapeutic perspective, it is important to distinguish idiopathic MCD (iMCD) from those cases that are associated with the human herpesvirus-8 (HHV-8 + MCD). SUMMARY: During recent years, it has become increasingly clear that even HHV-8-negative MCD is not a homogeneous entity and that there are clinically distinct variants. International consensus guidelines for diagnosis and treatment have been developed for iMCD and UCD. KEY MESSAGES: We herein summarize recent advances in diagnosis, treatment, and novel insights into the pathogenesis of this disease.

Immunotherapy for KSHV-associated diseases.

Kaposi sarcoma herpesvirus (KSHV)-associated diseases (Kaposi sarcoma, multicentric Castleman disease, primary effusion lymphoma, and KSHV inflammatory cytokine syndrome) are associated with immune suppression and dysregulation and loss of KSHV-specific immunity. These diseases are most frequent in people living with HIV as well as those with primary or iatrogenic immune deficiencies. KSHV itself can modulate the immune system via viral homologs of host cytokines or downregulation of immune-surface markers altering host immune surveillance. These factors make KSHV-associated diseases prime targets for immunotherapy approaches. Several agents have been studied or are under investigation in KSHV-associated diseases, including monoclonal antibodies, immunomodulatory agents, and therapeutic cytokines. Here, we review the role of immunotherapies in KSHV-associated diseases.

[The clinical features of Castleman disease in the head and neck].

Objective:To investigate the clinicopathological features, treatment and prognosis of Castleman disease in the head and neck. Methods:The clinical and pathological data of 18 patients with Castleman disease of the head and neck in Nanjing Drum Tower Hospital from 2007 to 2021 were retrospectively analyzed. There were 14 cases of unicentric type and 4 cases of multicentric type. The clinical characteristics, treatment and prognosis were analyzed. Results:Among the 18 cases of Castleman disease in the head and neck, 1 case was located in the parotid gland, 1 case was behind the ear, 1 case was in the parapharyngeal space, 3 cases were in the neck region Ⅰ, 2 cases were supraclavicular, 2 cases were in the neck region Ⅲ, the rest were located in more than two subregions of the neck. In patients with unicentric type, no tumor recurrence and progression were found in the postoperative re-examination with neck Doppler ultrasound and CT; in the multicentric type, multiple organ dysfunction, such as edema of both lower extremities, hepatosplenomegaly, and cough, were found. Of the 4 patients with multicentric type, only 1 patient received chemotherapy, and the remaining 3 patients refused chemotherapy and only received symptomatic treatment. All patients survived during follow-up, but the disease of multicentric patients progressed significantly, and the number of involved lymph nodes increased, and hepatosplenomegaly were found in some patients. Conclusion:Castleman disease of the head and neck is mostly unicentric type, which is manifested as multiple asymptomatic enlarged lymph nodes in the neck. The surgical resection is effective and the prognosis is good. Multicentric Castleman disease of the head and neck has complex clinical symptoms and involves multiple organs over time, requiring follow-up treatment.

Enfermedad de Castleman multicéntrica en pacientes HIV positivos. Reporte de dos casos y revisión de la literatura / Multicentric Astleman disease in HIV positive patients. Two cases. Report and literature review

La enfermedad de Castleman (EC) es un proceso linfoproliferativo poco frecuente que se caracteriza por hiperplasia de los ganglios linfáticos. Existen dos variedades histológicas bien diferenciadas la hialino-vascular y la plasmocelular, que a su vez pueden ser localizadas o multicéntricas. La forma hialino-vascular suele ser asintomática y localizada en mediastino mientras que la plasmocelular se presenta frecuentemente con signo-sintomatología sistémica y suele ser difusa o multicéntrica. En el contexto de la enfermedad debida al virus de la inmunodeficiencia humana (VIH), la EC se asocia en su patogenia a la infección por el herpes virus humano tipo-8 (HHV-8). La mayoría de los casos corresponden a la variante hialino-vascular (80/90%) en tanto un pequeño porcentaje (10/20%) son de la variante plasmocelular. En algunos pacientes, el patrón histopatológico puede ser mixto. Se describen dos casos de enfermedad de Castleman multicéntrica HHV8- positiva en pacientes con enfermedad HIV/SIDA.

Castleman's disease (CD), is a rare hematological condition of uncertain etiology, involves a massive proliferation of lymphoid tissues and typically presents as mediastinal masses. This is considered as a distinct type of lymphoproliferative disorder associated with inflammatory symptoms. In the context of human immunodeficiency virus (HIV) infection, CD is associated with human herpesvirus-8 (HHV8) infection. Most cases of CD represent either the hyaline vascular variant (80­90% of cases) or the plasma cell variant (10­20%); a small percentage present with a mixed histologic appearance. Two cases of Castleman's disease associated with HHV-8 and HIV/AIDS infection are described

Multicentric Castlemans disease. Symptoms, diagnostics and therapy.

Castleman disease (CD) describes a group of heterogeneous hematologic disorders with characteristic histopathological features. CD can present with unicentric (UCD) or multicentric (MCD) regions of lymph node enlargement. Some cases of MCD are caused by human herpesvirus-8 (HHV-8), whereas others are HHV-8-negative/idiopathic (iMCD). Treatment of iMCD is challenging, and outcomes can be poor. In this paper, we briefly report about symptoms of iMCD and about the International, evidencebased consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease and International evidence based consensus treatment guidelines for idiopathic multicentric Castleman disease.

The clinicopathological features and survival of Castleman disease: a multicenter Turkish study.

OBJECTIVE: In this study, we aimed to investigate the clinicopathological features and survival of CD, which is quite rare and has many unknowns. PATIENTS AND METHODS: This study was conducted by retrospectively evaluating patients diagnosed with CD in six different centers in Turkey. RESULTS: The median age of 33 patients included in the study was 49 and 51.5% (n = 17) of these patients were women. 18 (54.5%) patients were in the hyaline vascular subtype and most of the patients were UCD (n = 20, 60.6%). The most common involvement region was head and neck (n = 19, 57.5%). The UCD group was younger than the MCD group (p=0.027). Visceral lymph node involvement was higher in MCD than in UCD (p=0.001). Similarly, it was observed that there was more hepatomegaly (p=0.035) and splenomegaly (p=0.013) in the MCD group. During the median 19.5 months follow-up period, there were no patients who died. CONCLUSIONS: It was observed that UCD and MCD are different clinical entities. Promising survival times can be achieved with surgical and systemic treatments in both subtypes of this extremely rare disease. However, this result should be supported by well-designed prospective comprehensive studies.

[Clinicopathological and therapeutic analysis of Castleman's disease in children].

Objective: To investigate the clinical features, pathological phenotype, treatment and prognosis of Castleman's disease in children. Methods: Clinical data of 15 children diagnosed with Castleman's disease in Henan Provincial People's Hospital and the First Affiliated Hospital of Zhengzhou University from May 2010 to October 2019 were analyzed retrospectively. The clinical characteristics, laboratory examination and histopathological data were analyzed. Results: Among the 15 Castleman's disease patients, 12 were males and 3 females. The age of first visit was 12 (10, 15) years. The time from mass discovery to pathologic diagnosis was 9.0 (2.0, 13.0) months. The majority of patients were unicentric (13 cases), and the histopathological type was hyaline vascular (11 cases). Unicentric lesions were most common in the neck (11 cases), all 13 patients received complete surgical resection of the lesions, the follow-up time was 20.0 (13.5, 50.5) months, and the prognosis was good. Two cases were multicentric type, the pathological types were mixed variant, meeting the criteria of idiopathic Castleman's disease, the two children underwent partial surgical resection, one was treated with rituximab and prednisone and the other was treated with thalidomide and prednisone. The follow-up time was 32 months and 10 month, both of them had good prognosis. Conclusions: Most cases of Castleman's disease in children are diagnosed late, and the unicentric type is dominant. The most common pathological type is hyaline vascular, which is characterized by painless lymphadenopathy, while multicentric type has systemic symptoms and both of them have a good overall prognosis.

Castleman Disease: A Multicenter Case Series from Turkey

Objective: Castleman disease (CD) is a rare disease also known as angiofollicular lymph node hyperplasia. The two main histological subtypes are the hyaline vascular and plasma cell variants. It is further classified as unicentric CD (UCD) or multicentric CD (MCD) according to the anatomical distribution of the disease and the number of lymph nodes involved. The aim of this multicenter study was to evaluate all cases of CD identified to date in Turkey to set up a national registry to improve the early recognition, treatment, and follow-up of CD. Materials and Methods: Both adult (n=130) and pediatric (n=10) patients with lymph node or involved field biopsy results reported as CD were included in the study. Patients' demographic information, clinical and laboratory characteristics, imaging study results, treatment strategies, and clinical outcomes were evaluated retrospectively. Results: A total of 140 patients (69 male and 71 female) with a diagnosis of UCD (n=73) or MCD (n=67) were included. The mean age was 39 years in the UCD group and 47 years in the MCD group. Female patients were more common in the UCD group. The most common histological subtype was hyaline vascular for both UCD and MCD patients. Asymptomatic patients were more common in the UCD group. Anemia, elevations of acute phase reactants, and hypoalbuminemia were more common in the MCD group. The most commonly used treatment strategies for UCD were surgical excision, rituximab, and radiotherapy, respectively. All UCD patients were alive at a median of 19.5 months of follow-up. The most commonly used treatment strategies for MCD were methyl prednisolone, R-CHOP, R-CVP, and rituximab. Thirteen MCD patients had died at a median of 34 months of follow-up. Conclusion: This study is important in presenting the patient characteristics and treatment strategies for CD from Turkey, with the potential of increasing awareness about CD. Treatment data may help in making decisions, particularly in countries that do not have access to siltuximab. However, larger prospective studies are needed to make definitive conclusions.

Multicentric Castleman disease: A single center experience of treatment with a focus on autologous stem cell transplantation.

Castleman disease (CD) is a rare lymphoproliferative disease characterized by diverse clinical and pathologic features. Due to its rarity, there are limited studies comparing currently available therapies. The role of autologous stem cell transplantation (ASCT) in CD has not yet been established. In this paper, we describe the clinical characteristics, treatment choices, and outcomes in 34 Mayo Clinic patients diagnosed with multicentric CD from July 1, 2003 to April 30, 2018. Eighteen patients (53%) also met the criteria for POEMS, including 14 with the osteosclerotic variant. The first-line treatments included: steroid monotherapy (4), cytotoxic chemotherapy (6), rituximab alone (8) or with chemotherapy (2), anti-IL6 treatment (3), and ASCT (10). The median follow-up was 4.8 (range: 0.1-15.2) years. The 5- and 10-year overall survival rates were 84% and 71%, respectively. Sixteen patients received high-dose chemotherapy followed by ASCT during their disease course. Among those, 14 had multicentric CD associated with POEMS. There were no transplant-related deaths. All patients had at least a partial response to ASCT, most of whom achieved a complete response. The favorable outcomes seen with ASCT in this cohort suggest that transplantation may have a role in multicentric CD, particularly for patients with multicentric CD associated with POEMS.