Rituximab Therapy for HIV-Negative Multicentric Castleman’s Disease: Seven Years’ Experience at a Single Institute

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4998-4998
Author(s):  
Makoto Ide ◽  
Yasunori Kawachi ◽  
Yoichiro Izumi
Keyword(s):  
Plasma Cell ◽  
Castleman’S Disease ◽  
Cell Type ◽  
Multicentric Castleman’S Disease ◽  
Vascular Type ◽  
Hyaline Vascular Type ◽  
Plasma Cell Type ◽  
Multicentric Castleman's Disease ◽  
Hiv Negative

Abstract Background: Multicentric Castleman’s disease (MCD) is an indolent lymphoproliferative disorder of unknown etiology, and is classified into three types: hyaline-vascular type, plasma-cell type, and mixed type. Recently, we reported the use of rituximab therapy for HIV-negative patients with MCD, after obtaining the informed consent of the patient and the approval of the Institutional Review Board (Br J Haematology 121, 818–819, 2003, Eur J Haematology 76. 119–123. 2006). Therefore, we tried to review the long-term follow-up (nine to 75 months) of all patients at a single institute. Patients and Methods: Over seven years, 5 HIV-negative patients with MCD (3 men and 2 women; median age: 39, range: 26–61 years) were referred to Takamatsu Red Cross hospital. After clinical evaluation (chest and abdominal computed tomography scans, magnetic resonance imaging and Ga67 citrate scintigrams), lymphnode biopsy was performed for histological diagnosis. Rituximab was administered intravenously at a standard dose of 375 mg/m2 weekly, for 4 or 8 times without chemotherapy, and we analyzed the clinical course of MCD after rituximab therapy. During the follow-up, clinical symptoms were recorded. At a median follow-up of 49 months, biologic parameters (complete blood count, C-reactive protein, immunoglobulin G), and cytokines (soluble interleukin-2 receptor, Interleukin-6) were determined at different time points by standard procedures. Results: Three of the five patients had histological evidence of hyaline-vascular type MCD, and two patients had plasma-cell type MCD. Two (both with hyaline vascular type) of the five MCD patients (40%) with MCD achieved almost complete remission with rituximab therapy when followed up for 50 to 75 months. Three (one with hyaline vascular type and two with plasma-cell type) of the five patients did not show clinical remission after riruximab administration. Conclusion;Rituximab treatment provides a durable response in a some MCD (especially those with hyaline-vascular type) patients, but is not always successful.

scholarly journals Abdominal localization of unicentric form of Castleman disease: A case report

2017 ◽  
Vol 74 (4) ◽  
pp. 367-370
Author(s):  
Bosko Milev ◽  
Borka Milev ◽  
Zoran Kostic ◽  
Darko Mirkovic ◽  
Nenad Perisic ◽  
...  
Keyword(s):  
Case Report ◽  
Plasma Cell ◽  
Castleman Disease ◽  
Mr Enterography ◽  
Unknown Etiology ◽  
Cell Type ◽  
Vascular Type ◽  
Hyaline Vascular Type ◽  
Plasma Cell Type ◽  
Golden Standard

Introduction. Castleman disease is a rare disease of the unknown etiology, occuring in two clinical forms: unicentric or multicentric. It is characterized by the hyperplasia of lymph glands. In literature the four pathohistological forms were described: hyaline vascular type, plasma cell type, mixed type and a recently recognized plasmablastic type. The most frequent changes are localized in the mediastinum, while the abdominal localization is with significatly rare occurrence, and that was the motive for presentation of this case. Case report. In a 41-year old male magnetic resonance (MR) enterography showed a change in the ileocecal area without the presence of subjective symptoms of digestive tract and without loss of body mass. Due to the suspicion of stromal tumor, surgical intervention was indicated. Pathohistological findings showed Castleman lymphadenopathia reactiva mesenterii (plasma cell type) which was in the unicentric form. There were present only anaemia and the increased value of sedimentation from the laboratory analyses. Conclusion. Abdominal localization of unicentric plasma cell form occurs rarely and the surgical method of treatment presents the golden standard as it was shown in the presented case.

scholarly journals Multicentric Hyaline-Vascular Castleman Disease: A Case Report

2015 ◽  
Vol 16 (1) ◽  
pp. 48-50
Author(s):  
Aparna Das ◽  
Sanjana Tarannum ◽  
Tahera Kona ◽  
Santanu Kumar Saha ◽  
MA Kahhar
Keyword(s):  
Lymph Node Biopsy ◽  
Castleman Disease ◽  
Whole Body ◽  
High Dose ◽  
Vascular Type ◽  
Hyaline Vascular Type ◽  
Plasma Cell Type ◽  
Generalized Lymphadenopathy ◽  
Hiv Negative

Castleman disease is a rare lymphoproliferative disorder. Contrary to its closest differential of lymphoma, the disease tends to run a benign course. However the multicentric variety, which is usually of the plasma cell type, has a more sinister prognosis. It is commonly associated with HIV infection. We report a case of a 65 year old man presenting with recurrent episodes of swelling of the whole body and diarrhoea for 3 years. Physical examination revealed generalized lymphadenopathy with hepato-splenomegaly and ascites. Lymph node biopsy revealed histopathological changes consistent with hyaline-vascular type of Castleman disease (multicentric). He was HIV negative. Patient was treated with high dose corticosteroids and discharged with follow up advice. DOI: http://dx.doi.org/10.3329/jom.v16i1.22402 J MEDICINE 2015; 16 : 48-50

Pediatric Intraparotid Castleman's Disease

2003 ◽  
Vol 112 (9) ◽  
pp. 813-816 ◽  
Author(s):  
Daniel S. Samadi ◽  
Neil G. Hockstein ◽  
Lawrence W. C. Tom
Keyword(s):  
Head And Neck ◽  
Clinical Course ◽  
Castleman’S Disease ◽  
Castleman's Disease ◽  
Cell Type ◽  
Radiographic Findings ◽  
Vascular Type ◽  
Hyaline Vascular Type ◽  
Plasma Cell Type ◽  
Histologic Types

Castleman's disease (CD) is a rare, benign lymphoepithelial disease of unknown cause that presents most commonly in the mediastinum. There are 2 histologic types of CD: the hyaline vascular type and the plasma cell type. In the head and neck, 98% of these lesions are of the hyaline vascular type. The differential diagnosis of CD includes reactive lymphadenopathy, lymphoproliferative disorders, lymph node metastasis, and other conditions. Approximately 80 cases of CD have been reported in children; head and neck involvement in children is extremely rare. A case of a child with intraparotid CD is presented; the clinical course, histopathologic presentation, radiographic findings, and management of CD are reviewed.

Clinical, Biochemical, and Radiographic Responses to Rituximab Combined with Liposomal Doxorubicin (R-Dox) in HIV-Infected Patients with Severe Kaposi Sarcoma-Associated Herpes Virus (KSHV) – Associated Multicentric Castleman's Disease.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1651-1651
Author(s):  
Thomas Uldrick ◽  
Mark N. Polizzotto ◽  
Deirdre O'Mahony ◽  
Karen Aleman ◽  
Kathy Wyvill ◽  
...  
Keyword(s):  
Liposomal Doxorubicin ◽  
Castleman’S Disease ◽  
Biochemical Response ◽  
Additional Patient ◽  
Castleman's Disease ◽  
Multicentric Castleman’S Disease ◽  
Radiographic Response ◽  
Additional Therapy ◽  
Multicentric Castleman's Disease

Abstract Abstract 1651 Poster Board I-677 Background KSHV-associated multicentric Castleman's disease (MCD) is a rare lymphoproliferative disorder characterized by fever, splenomegaly, adenopathy, hypoalbuminemia, hyponatremia, cytopenias, elevated inflammatory markers, and a waxing and waning course. Most MCD arising in HIV-infected patients is KSHV-associated. Historically, prognosis has been poor. There is no standard therapy, although benefit has been reported with cytotoxic chemotherapy, interferon-á, retinoic acid and ganciclovir. Rituximab has reported activity in KSHV-MCD, but may not be sufficient as monotherapy in patients with severe disease, and can be associated with worsening of Kaposi's sarcoma (KS). Within a natural history study of KSHV-MCD, we evaluated the treatment effects of R-Dox on correlates of disease activity in patients with severe MCD or MCD with concurrent KS. Methods Patients with biopsy confirmed MCD that was severe or accompanied by severe KS were treated with liposomal doxorubicin 20mg/m2 plus rituximab 375 mg/m2 every 21 days until substantial clinical improvement or disease progression. Post R-Dox therapy, discussed below, was used to consolidate or maintain responses. Clinical, biochemical and radiographic response were evaluated individually using protocol-defined criteria. Overall complete responses (CR) required normalization of all clinical, laboratory or radiographic abnormalities attributed to MCD lasting at least 3 weeks. Results Twelve patients (1 woman, 11 men) have been treated with R-Dox to date. Patient characteristics: median (med) age 43 (range 34-55); all were on HAART, med CD4 331 cells/μL (21-1598), HIV viral load <50 copies/mL in 10 patients. Med number of prior therapies 2 (0-8); concurrent KS (5); dependent on steroids (3); patients hospitalized during first cycle (6). Med baseline values for biochemical response parameters: C-reactive protein 9.7 mg/dL (0.4-21.0), albumin 2.7 mg/dL (1.5-3.4), sodium 133 mEq/L (126-140), platelets 70 K/uL (10-377), hemoglobin 9.4 g/dL (6.8-12.0). 11 had diffuse adenopathy and all had splenomegaly, med spleen 18.5 cm (12.5-28 cm). Patients received med 4 cycles (3-9) of R-Dox. All patients met criteria for clinical CR after a med 2 cycles (range 1-5). Best biochemical response was CR in 9 (75%) and partial response (PR) in 1 (8%); 2 (17%) had stable biochemical parameters. Best radiographic response was CR in 6 (50%) and PR in 6 (50%) with a med 5 cm (+0.5 cm, -10 cm) decrease in spleen size. Best biochemical response was achieved after med 3 cycles (1-7), and best radiographic response after med 3 cycles (2-5). 2 of 12 had an overall CR at completion of R-Dox. Post R-Dox therapy included: IFNá (8), high-dose AZT + valganciclovir (2), additional liposomal doxorubicin (1). To date, another 6 patients achieved overall CR with additional therapy. Concurrent KS improved in 4 of the 5 patients affected. With a median potential follow-up of 25.5 months (actual follow-up range from 5.5+ to 41+ months), 9 of 12 patients have had no MCD relapse after starting R-Dox, 2 patients had recurrent MCD flares (months 7 and 17) that responded to additional therapy and 1 patient had progressive MCD during cycle 6 associated with worsening KS, and died at month 6 of central pontine myelinolysis. He was found to have primary effusion lymphoma at autopsy. An additional patient died of pneumonia (month 17). Toxicity was minimal. 9 patients had infusion reactions (Gr. 1 = 3, Gr. 2 = 4, Gr. 3 = 2) with the first dose of rituximab. 9/55 cycles were complicated by neutropenia (Gr. 2 = 7, Gr. 3-4 = 2). There were no infectious complications. Conclusions R-dox is highly effective in heavily pretreated patients with severe KSHV-MCD or MCD with concurrent severe KS. Further evaluation of R-Dox in patients with severe KSHV-MCD is ongoing. Disclosures Off Label Use: Rituximab and Liposomal Doxorubicin are being explored in the treatment KSHV-MCD.

scholarly journals Mixed-type Multicentric Castleman's Disease Developing during a 17-year Follow-up of Sarcoidosis

2012 ◽  
Vol 51 (21) ◽  
pp. 3061-3066 ◽  
Author(s):  
Nobuyasu Awano ◽  
Minoru Inomata ◽  
Keisuke Kondoh ◽  
Kohta Satake ◽  
Hiroyuki Kamiya ◽  
...  
Keyword(s):  
Mixed Type ◽  
Castleman’S Disease ◽  
Castleman's Disease ◽  
Multicentric Castleman’S Disease ◽  
Multicentric Castleman's Disease

Complete remission in a pancytopenic HIV negative, HHV-8 positive patient with multicentric Castleman’s disease induced with anti-CD20

2006 ◽  
Vol 8 (7) ◽  
pp. 540-541 ◽  
Author(s):  
José Manuel Cervera Grau ◽  
Gaspar Galiana Esquerdo ◽  
Cristina Llorca Ferrándiz ◽  
Hugo Briceño Garcia ◽  
Manuel Díaz Castellano ◽  
...  
Keyword(s):  
Complete Remission ◽  
Castleman’S Disease ◽  
Positive Patient ◽  
Castleman's Disease ◽  
Multicentric Castleman’S Disease ◽  
Anti Cd20 ◽  
Multicentric Castleman's Disease ◽  
Hiv Negative
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