OBJECTIVE: To explore the clinical characteristics, diagnosis, and treatment methods of one patient with idiopathic multicentric Castleman disease (iMCD), in order to strengthen the understanding of this rare disease. METHODS: The clinical manifestations, diagnosis and treatment process, and prognosis of one patient with iMCD admitted to our hospital were retrospectively analyzed. RESULTS: The patient was a 45-year-old female with swollen bilateral orbit, edema of lower limbs, multiple serosal cavity effusion, thrombocytopenia, renal insufficiency, and multiple lymph node enlargement. Lymph node biopsy suggested mixed type of Castleman disease. Combined with pathology, imaging and laboratory examination, the patient was finally diagnosed with mixed type of iMCD. After six cycles of R-COP regimen chemotherapy, the patient achieved complete remission. CONCLUSIONS Castleman disease with renal and orbit involvement is rare in clinic and easy to be misdiagnosed. It should be distinguished from lymphoma. The patient has been treated with R-COP regimen, and obtained good short-term efficacy.
Humans ; Castleman Disease/diagnosis* ; Middle Aged ; Female ; Retrospective Studies ; Orbit/pathology* ; Kidney/pathology*

Objective To investigate the clinical characteristics,treatment responses,and prognosis of patients with idiopathic multicentric Castleman disease(iMCD)complicated by autoimmune hemolytic anemia(AIHA). Methods The patients diagnosed with iMCD in Peking Union Medical College Hospital from January 2010 to December 2023 and having complete baseline blood routine data were retrospectively enrolled in the study.The iMCD patients were further assigned into AIHA and non-AIHA groups based on baseline laboratory examinations,and the clinical characteristics and prognosis were compared between the two groups. Results A total of 341 patients with iMCD were enrolled in this study,including 277(81.2%)exhibiting anemia at baseline.Five(1.8%)patients were identified as having iMCD-AIHA,all of whom were iMCD-not otherwise specified type complicated by warm antibody-type AIHA,and two of them were simultaneously diagnosed with Evans syndrome.The timing relationship between the first onset of AIHA and iMCD diagnosis varied,with 2 patients experiencing their first hemolytic episode prior to the diagnosis of iMCD.In terms of treatment,the therapy targeting iMCD was effective in alleviating AIHA.The AIHA group had a poorer prognosis(HR=4.61,95% CI=1.08-19.80,P=0.040)and a lower 5-year survival rate(90% vs.60%,P=0.024)than the non-AIHA group.Conclusions iMCD-AIHA is clinically rare,and AIHA can occur at different stages of iMCD.Although the primary treatment principle remains targeting iMCD,great attention should be paid to the exacerbation of immune dysfunction caused by AIHA and the possible adverse prognosis it may bring.
Humans ; Anemia, Hemolytic, Autoimmune/diagnosis* ; Castleman Disease/diagnosis* ; Retrospective Studies ; Male ; Female ; Prognosis ; Adult ; Middle Aged ; Aged ; Young Adult ; Adolescent

Objective:Castleman's disease is a group of heterogeneous lymphoproliferative disorders with unclear etiology and pathogenesis.This article reports a case of Castleman's disease of the parapharyngeal space, with a comprehensive literature review of the clinical feature and typing, pathologic diagnosis, treatment, and prognosis of the disease.This case is a unicentric type, which is clinically characterized by significant enlargement of lymph nodes without systemic symptoms. The pathology was hyaline vascular type, and the treatment of unicentric type was mostly based on surgery with a better prognosis.
Adult ; Female ; Humans ; Male ; Castleman Disease/diagnosis* ; Lymph Nodes/pathology* ; Parapharyngeal Space ; Prognosis

OBJECTIVE: To investigate the clinical characteristics, diagnosis, and treatment of one patient with TAFRO syndrome, and to strengthen the understanding of this rare type. METHODS: The clinical manifestations, diagnosis and treatment process, and prognosis of the patient admitted in Gansu Provincial People's Hospital were retrospectively analyzed. RESULTS: Combined with laboratory tests, bone marrow examination, imaging, pathology, etc, the patient was diagnosed with TAFRO syndrome. After three cycles of treatment with pomalidomide (2-3 mg/d, d1-21), cyclophosphamide (300 mg/m², 0.54 g once a week) and dexamethasone (20 mg/d, two days a week), platelet count, serum creatinine and procalcitonin returned to normal, the systemic edema disappeared, and the patient's condition was alleviated. The therapeutic effect was good. CONCLUSION TAFRO syndrome is rare, involves multiple systems, progresses rapidly, and has a worse prognosis. The choice of the "Pomalidomide+cyclophosphamide+dexamethasone" regimen is help to improve the survival prognosis of patient with TAFRO syndrome.
Humans ; Thrombocytopenia ; Retrospective Studies ; Castleman Disease/diagnosis* ; Dexamethasone ; Cyclophosphamide/therapeutic use*

Objective: To investigate the value of plasma cells for diagnosing lymph node diseases. Methods: Common lymphadenopathy (except plasma cell neoplasms) diagnosed from September 2012 to August 2022 were selected from the pathological records of Changhai Hospital, Shanghai, China. Morphological and immunohistochemical features were analyzed to examine the infiltration pattern, clonality, and IgG and IgG4 expression of plasma cells in these lymphadenopathies, and to summarize the differential diagnoses of plasma cell infiltration in common lymphadenopathies. Results: A total of 236 cases of lymphadenopathies with various degrees of plasma cell infiltration were included in the study. There were 58 cases of Castleman's disease, 55 cases of IgG4-related lymphadenopathy, 14 cases of syphilitic lymphadenitis, 2 cases of rheumatoid lymphadenitis, 18 cases of Rosai-Dorfman disease, 23 cases of Kimura's disease, 13 cases of dermal lymphadenitis and 53 cases of angioimmunoblastic T-cell lymphoma (AITL). The main features of these lymphadenopathies were lymph node enlargement with various degrees of plasm cell infiltration. A panel of immunohistochemical antibodies were used to examine the distribution of plasma cells and the expression of IgG and IgG4. The presence of lymph node architecture could help determine benign and malignant lesions. The preliminary classification of these lymphadenopathies was based on the infiltration features of plasma cells. The evaluation of IgG and IgG4 as a routine means could exclude the lymph nodes involvement of IgG4-related dieases (IgG4-RD), and whether it was accompanied by autoimmune diseases or multiple-organ diseases, which were of critical evidence for the differential diagnosis. For common lesions of lymphadenopathies, such as Castleman's disease, Kimura's disease, Rosai-Dorfman's disease and dermal lymphadenitis, the expression ratio of IgG4/IgG (>40%) as detected using immunhistochemistry and serum IgG4 levels should be considered as a standard for the possibility of IgG4-RD. The differential diagnosis of multicentric Castleman's diseases and IgG4-RD should be also considered. Conclusions: Infiltration of plasma cells and IgG4-positive plasma cells may be detected in some types of lymphadenopathies and lymphomas in clinicopathological daily practice, but not all of them are related to IgG4-RD. It should be emphasized that the characteristics of plasma cell infiltration and the ratio of IgG4/IgG (>40%) should be considered for further differential diagnosis and avoiding misclassification of lymphadenopathies.
Humans ; Castleman Disease/pathology* ; Plasma Cells/pathology* ; Immunoglobulin G4-Related Disease ; China ; Lymphadenopathy/pathology* ; Inflammation/pathology* ; Lymph Nodes/pathology* ; Diagnosis, Differential ; Lymphadenitis/pathology* ; Immunoglobulin G/metabolism*

Humans ; Sjogren's Syndrome/diagnosis* ; Castleman Disease/diagnosis* ; Case-Control Studies

Aged ; Castleman Disease ; immunology ; pathology ; Diagnosis, Differential ; Humans ; Immunoglobulin G ; metabolism ; Lymphatic Diseases ; immunology ; pathology ; surgery ; Lymphoma ; pathology ; Male ; Plasma Cells ; immunology ; Pseudolymphoma ; immunology ; pathology

OBJECTIVETo investigate the clinical characteristics and treatment of localized Castleman's disease (CD), and review the literatures to improve the diagnosis and management of this disease.

METHODSThe clinical symptoms, histopathology, CT, MRI findings and results of surgery in 20 patients with localized CD were evaluated retrospectively.

RESULTSThe average age of the patients was 37.7 years. The lesions were located in the retroperitoneal space (9 cases), mediastinum (7 cases), pelvic cavity (1 case), neck (1 case), upper arm (1 case), and axillary (1 case). All patients underwent surgical resection, including 9 cases for retroperitoneal resection (6 cases had open operation and 3 cases laparoscopic resection) and 7 cases for mediastinal resection (open operation in 5 cases and thoracoscopic resection in 2 cases). The Castleman's disease was confirmed by histopathology. There were hyaline vascular type of CD in 17 cases, plasma cell type of CD in 1 case, and mixed cellularity type of CD in 2 cases. The duration of follow-up ranged from 12 to 165 months for 16 cases. Among them 15 patients were alive without recurrence, and 1 case had recurrence in the primary site at 47 months after the operation.

CONCLUSIONSPatients with Castleman's disease have no typical clinical symptoms and have normal laboratory results. The majority of patients are of hyaline vascular type of the disease. Imaging examination is helpful to diagnosis, and the final diagnosis depends on pathologic examination. Complete surgical resection of the tumor is the best treatment for localized Castleman's disease.

Adult ; Castleman Disease ; diagnosis ; diagnostic imaging ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Male ; Mediastinum ; Middle Aged ; Recurrence ; Retroperitoneal Space ; Retrospective Studies ; Tomography, X-Ray Computed ; Treatment Outcome ; Young Adult

ADP-ribosyl Cyclase 1 ; metabolism ; Adult ; Brain Neoplasms ; metabolism ; pathology ; surgery ; CD79 Antigens ; metabolism ; Castleman Disease ; metabolism ; pathology ; Diagnosis, Differential ; Humans ; Leukocyte Common Antigens ; metabolism ; Lymphoma, Large B-Cell, Diffuse ; metabolism ; pathology ; surgery ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Male ; Melanoma ; metabolism ; pathology ; Parietal Lobe ; Plasma Cells ; metabolism ; pathology

To evaluated the multi-detector CT (MDCT) and magnetic resonance imaging (MRI) features of localized Castleman's disease (CD), we retrospectively analyzed the clinical data, MDCT and MRI findings of 13 patients with CD proved pathologically. All patients underwent plain MDCT scan, 11 underwent enhanced CT, and 2 MRI. 14 lesions were detected in the 13 patients, and all of them were hyaline-vascular type (HV-CD) histopathologically. On plain MDCT scans, all lesions were homogeneously attenuated soft tissue mass; intra-tumoral calcification with punctate and "arborizing" patterns was detected in the center of 2 lesions each. Of the patients with enhanced MDCT, all lesions showed obvious enhancement homogeneously except two lesions with central stellate and 1 lesion with dotted low attenuation. Tortuous vessels could be revealed at the periphery of 8 lesions. Of the 2 patients with MRI, the lesions showed slightly hyper-intensity on T1WI, hyper-intensity on T2WI and marked homogenous enhancement. In addition, one of them showed signal void appearance in the center on unenhanced MRI and large supplying artery with tortuous vessels at the periphery on enhanced MRI. In a word, Localized HV-CD usually demonstrated as soft tissue mass with obvious enhancement on MDCT and MRI. Central stellate area of low attenuation and calcification with punctate or "arborizing" pattern may also be present.
Adolescent ; Adult ; Castleman Disease ; diagnosis ; diagnostic imaging ; pathology ; Female ; Humans ; Image Enhancement ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Multidetector Computed Tomography ; Retrospective Studies ; Young Adult