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

OBJECTIVETo investigate the clinical characteristics of children with Castleman's disease and to improve doctors' awareness of this disease.

METHODSClinical characteristics of 5 cases with Castleman's disease were observed and analyzed and relevant reports in literature were reviewed.

RESULTS(1) All the five patients' histories were long, and the first symptoms of them were painless lymphnode enlargement, and all of them were at school age; 3 patients' abdominal lymphnodes were enlarged, mediastinum lymphnodes enlarged in 3 cases, cervical lymphnodes were involved in 3 cases; (2) The clinical subtypes: the disease in 3 cases was localized Castleman's disease (LCD), all of their pathological subtype was hyaline vascular variant (HV). The rest of them were multicentric Castleman's disease (MCD), whose pathology was plasma cell variant (PC), and both of them had a febrile symptoms; (3) The white blood cells, C-reactive protein and ferritin levels were all elevated to different extents. Four of them had viral infections, and their cellular immune function was abnormal; (4) The LCD patients' prognosis was good after the complete resection. There is no standard therapy for MCD, the available therapies include antiviral, immune modulatory regimens, CD20 B cell monoclonal antibody and chemotherapy, but the prognosis was worse than that of LCD.

CONCLUSIONSCastleman's disease is rare in children, which can be misdiagnosed because it has no specific manifestations. The prognosis depends on the subtype.

Castleman Disease ; diagnosis ; Child ; Female ; Humans ; Male ; Prognosis ; Retrospective Studies

Adult ; Anemia, Hemolytic, Autoimmune ; complications ; Castleman Disease ; diagnosis ; surgery ; Female ; Humans ; Laparoscopy ; Retroperitoneal Space ; pathology ; surgery

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*

Castleman disease is a relatively rare entity, with the hyaline-vascular type the predominant form. Although the plasma cell type is uncommon, it still comprises approximately 10% of cases of localized diseases. In addition, the abdomen is a rare site for involvement and localized Castleman disease of the plasma cell type in the abdomen is rare. The radiologic features of localized plasma cell type in the abdomen are mostly limited to case reports. In addition to the conventional imaging findings, we present some new imaging findings of localized plasma cell type in the abdomen.
Abdomen ; pathology ; Aged ; Castleman Disease ; diagnosis ; diagnostic imaging ; Female ; Humans ; Male ; Middle Aged ; Tomography, X-Ray Computed

OBJECTIVESTo investigate the clinical and pathological subtypes of Castleman's disease (CD) and their relationship with complications.

METHODSThe clinical complications of 53 patients with CD and the relationship of these complications with clinical and pathological subtypes were analyzed retrospectively.

RESULTSAmong 53 CD patients, 32 (60.4%) were classified as uni-centric type and 21 (39.6%) multicentric type. Histopathological examination showed that 37 cases (69.8%) were hyaline vascular variants (HV), 9 (17.0%) plasmacytic variants (PC), and 7 (13.2%) mixed cellular variants (Mix). Complications were identified in 32 (60.4%) patients, including the involvements of skin, internal organs and hematopoietic system. Some complications were closely associated with the clinical subtype of CD: the majority of complications in the 32 uni-centric CDs were paraneoplastic pemphigus (PNP) and bronchiolitis obliterans (BO), and those in 21 multi-centric CDs were the involvements of kidney and hematopoietic system. The complications were different among the three kinds of histopathological subtypes: PNP and BO were the predominant complications of HV variants, while the internal organ and hematopoietic system involvements were those of PC and Mix variants. The clinical and histopathological classification of CD patients with PNP were different obviously from other subtypes of CDs. In Kaplan-Meier survival analysis, the survival rate of those with complications was significantly lower than those without complication (P = 0.028).

CONCLUSIONThe clinical complications of CDs are related to their clinical and histopathological subtypes. CD patients with PNP should be considered as a unique entity to tailor the therapy. The presence of clinical complications is an independent prognostic factor in CD patients.

Adolescent ; Adult ; Aged ; Castleman Disease ; complications ; diagnosis ; pathology ; Child ; Female ; Humans ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Young Adult

Abdomen ; pathology ; surgery ; Anemia ; complications ; Castleman Disease ; complications ; diagnosis ; Child ; Humans ; Lymph Nodes ; pathology ; Male ; Treatment Outcome

OBJECTIVETo discuss the clinical findings and treatment of paraneoplastic pemphigus (PNP) with Castleman's disease.

METHODSTo investigate the clinical, histopathologic and CT findings of 8 cases paraneoplastic pemphigus with Castleman's disease.

RESULTSAll of 8 patients were diagnosed PNP first and were found Castleman's tumor incidently during routine examination. All 8 cases showed severe erosion or ulcer of the oral mucosa with various skin lesions. Histopathologically, there were intraepidermal acantholytic vesicle, basal cell liquefaction, necrotic keratinocytes in the epidermis and lymphocyte infiltration in the upper dermis. CT scan appeared solitary mass in these patients. Some of them were attacked by bronchiolitis obliterans. All 8 patients were failed by use of predisone. Obvious relief of PNP and pulmonary lesion occurred after tumor was rescted.

CONCLUSIONSParaneoplastic pemphigus with Castleman's disease is a rare disease. The key step is to find and resect the tumor in abdomen. CT scan should be used to detect the tumor in patients with PNP, especially, when predisone was failed in treatment.

Adolescent ; Adult ; Castleman Disease ; complications ; diagnosis ; therapy ; Combined Modality Therapy ; Female ; Humans ; Male ; Paraneoplastic Syndromes ; complications ; diagnosis ; therapy ; Pemphigus, Benign Familial ; complications ; diagnosis ; therapy ; Retrospective Studies

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*

OBJECTIVETo explore the clinical features and surgical treatment of thoracic Castleman's disease.

METHODSThe clinical symptoms, pathological, laboratory, CT findings and results of surgery in 32 patients with Castleman's disease from June 1996 to November 2008 were evaluated. Among the 32 patients, there were 14 male and 18 female, aged from 16 to 48 years old with a mean age of 34.2 years old. Thirteen cases had symptoms including short of breath, irritable cough, or chest pain, while 14 cases had no symptoms. Mediastinal or hilar tumors were found by CT examination.

RESULTSTumor was surgically removed in all the 32 patients except one died with anesthetic accident. Castleman's disease was conformed by pathology. Five cases were diagnosed as with paraneoplastic pemphigus, 3 of them were attacked by bronchiolitis obliterans. All 5 cases were failed by the use of prednisone. The signs of PNP were dissolved after operation, but pulmonary lesions failed to improve. There was no recurrence in all cases.

CONCLUSIONSPNP and lung abnormalities are the rare and severe complications of thoracic Castleman's disease. Surgical resection of the tumor is the first choice for treatment.

Adolescent ; Adult ; Castleman Disease ; diagnosis ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Thoracic Diseases ; diagnosis ; pathology ; surgery ; Treatment Outcome ; Young Adult