scholarly journals An Unusual Presentation of Idiopathic Multicentric Castleman Disease: The Great Masquerader

2020 ◽  
Vol 7 (9) ◽  
pp. C119-122
Author(s):  
Sujaya Mazumder ◽  
Raji Tejas Naidu ◽  
Susan Cherian ◽  
Sruthi Mayura ◽  
Uma Pankaj Chaturvedi
Keyword(s):  
Lymphoproliferative Disorder ◽  
Rare Case ◽  
Young Female ◽  
Castleman Disease ◽  
Unusual Presentation ◽  
Multicentric Castleman Disease ◽  
Specific Symptoms ◽  
Work Up ◽  
Idiopathic Disease

Castleman disease is an uncommon, non-clonal, lymphoproliferative disorder characterized by lymphadenopathy and symptoms related to hypercytokinemia. Clinically it is classified as unicentric and multicentric disease. Multicentric disease is further subclassified as HHV- 8 associated disease and idiopathic disease, which is the rarest subtype. The incidence of idiopathic disease is estimated to be 5 per million person years. The diagnosis of Idiopathic Multicentric Castleman disease is complicated by an array of clinical mimics and non-specific symptoms. We report a rare case of Idiopathic Multicentric Castleman disease in a young female where a detailed pathological work up helped to secure the diagnosis and exclude its mimics.

scholarly journals KSHV- and EBV-associated germinotropic lymphoproliferative disorder

Blood ◽  
2002 ◽  
Vol 100 (9) ◽  
pp. 3415-3418 ◽  
Author(s):  
Ming-Qing Du ◽  
Tim C. Diss ◽  
Hongxiang Liu ◽  
Hongtao Ye ◽  
Rifat A. Hamoudi ◽  
...  
Keyword(s):  
Lymphoproliferative Disorder ◽  
Gene Rearrangement ◽  
Primary Effusion Lymphoma ◽  
Kaposi Sarcoma ◽  
Castleman Disease ◽  
Lymphoproliferative Disorders ◽  
Multicentric Castleman Disease ◽  
Tissue Sections ◽  
Response To Chemotherapy ◽  
Ig Heavy Chain

Abstract Kaposi sarcoma–associated herpesvirus (KSHV) is known to be associated with 3 distinct lymphoproliferative disorders: primary effusion lymphoma (PEL), multicentric Castleman disease (MCD), and MCD-associated plasmablastic lymphoma. We report 3 cases of a previously undescribed KSHV-associated lymphoproliferative disorder. The disease presented as localized lymphadenopathy and showed a favorable response to chemotherapy or radiotherapy. Histologically, the lymphoproliferation is characterized by plasmablasts that preferentially involved germinal centers of the lymphoid follicles, forming confluent aggregates. They were negative for CD20, CD27, CD79a, CD138, BCL6, and CD10 but showed monotypic κ or λ light chain. Clusters of CD10+CD20+ residual follicle center cells were identified in some of the follicles. The plasmablasts were positive for both KSHV and EBV, and most of them also expressed viral interleukin-6 (vIL-6). Unexpectedly, molecular analysis of whole tissue sections or microdissected KSHV-positive aggregates demonstrated a polyclonal or oligoclonal pattern of immunoglobulin (Ig) gene rearrangement. The plasmablasts showed somatic mutation and intraclonal variation in the rearranged Ig genes, and one case expressed switched Ig heavy chain (IgA), suggesting that they originated from germinal center B cells. We propose calling this distinctive entity “KSHV-associated germinotropic lymphoproliferative disorder.”

scholarly journals A Rare Case of HHV-8 Associated Hemophagocytic Lymphohistiocytosis in a Stable HIV Patient

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Nonso Osakwe ◽  
Diane Johnson ◽  
Natalie Klein ◽  
Dalia Abdel Azim
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Rare Case ◽  
Viral Infections ◽  
Rare Condition ◽  
Lymph Node Biopsy ◽  
Castleman Disease ◽  
Human Herpes Virus 8 ◽  
Case Presentation ◽  
Multicentric Castleman Disease ◽  
History Of

Background. Hemophagocytic lymphohistiocytosis (HLH) is a rare condition associated with viral infections including HIV. Cases have been reported mainly in advanced HIV/AIDS. This is a rare case that reports HLH associated with human herpes virus-8 (HHV-8) associated multicentric Castleman disease in a stable HIV patient. Case Presentation. A 70-year-old Asian male patient with history of stable HIV on medications with CD 4 cell count above 200 presented with cough and fever and was initially treated for pneumonia as an outpatient. Persisting symptoms prompted presentation to the hospital. The patient was found to have anemia which persisted despite repeated transfusion of packed red cells. A bone marrow biopsy to investigate anemia revealed hemophagocytosis. A CT scan revealed multiple enlarged lymph nodes and hepatosplenomegaly. An excisional lymph node biopsy revealed HHV-8 associated multicentric Castleman disease. The patient deteriorated despite initiation of treatment. Conclusion. HLH can occur at any stage of HIV, rapid diagnosis to identify possible underlying reactive infectious etiology and prompt initiation of treatment is crucial to survival.

scholarly journals Chronic Myelomonocytic Leukemia following Multicentric Castleman Disease

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Feng Li ◽  
Xiaomei Zhang ◽  
Yanting Guo ◽  
Yuandong Zhu ◽  
Yicun Wu ◽  
...  
Keyword(s):  
Lymphoproliferative Disorder ◽  
Pluripotent Stem Cell ◽  
Underlying Mechanism ◽  
Castleman Disease ◽  
Chronic Myelomonocytic Leukemia ◽  
Cell Precursor ◽  
Multicentric Castleman Disease ◽  
Night Sweats ◽  
Systemic Symptoms ◽  
Myelomonocytic Leukemia

Multicentric Castleman disease (MCD) is a rare nonmalignant lymphoproliferative disorder presenting systemic symptoms such as fever, night sweats, fatigue, anemia, effusions, and multifocal lymphadenopathy. The etiology of MCD has not been clarified to date. The coexistence of MCD with chronic myelomonocytic leukemia (CMML) has been rarely reported. Although the pathogenesis remains unclear, this association probably reflects an incidental and fortuitous finding rather than the alteration of a common pluripotent stem cell precursor. Herein, we report on one case of MCD coexisting with CMML and elucidate the underlying mechanism of pathology in some aspects.

High levels of human herpesvirus 8 viral load, human interleukin-6, interleukin-10, and C reactive protein correlate with exacerbation of multicentric Castleman disease in HIV-infected patients

Blood ◽  
2000 ◽  
Vol 96 (6) ◽  
pp. 2069-2073 ◽  
Author(s):  
Eric Oksenhendler ◽  
Guislaine Carcelain ◽  
Yoshiyasu Aoki ◽  
Emmanuelle Boulanger ◽  
Anne Maillard ◽  
...  
Keyword(s):  
Viral Load ◽  
Interleukin 6 ◽  
Lymphoproliferative Disorder ◽  
Human Herpesvirus ◽  
Castleman Disease ◽  
Human Herpesvirus 8 ◽  
C Reactive Protein ◽  
Herpesvirus 8 ◽  
Multicentric Castleman Disease ◽  
Reactive Protein

Abstract Multicentric Castleman disease (MCD) is a distinct type of lymphoproliferative disorder associated with inflammatory symptoms and interleukin-6 (IL-6) dysregulation. In the context of human immunodeficiency virus (HIV) infection, MCD is associated with human herpesvirus 8 (HHV8) infection. In a prospective study of 23 HIV-infected patients with MCD, clinical symptoms of MCD were present at 45 visits, whereas patients were in chemotherapy-induced clinical remission at 50 visits. Symptoms were associated with a high level of serum C reactive protein, high HHV8 viral load in peripheral blood mononuclear cells, and high plasma human IL-6 and IL-10 levels. Strong correlations between plasma IL-6 and plasma IL-10 with the HHV8 viral load suggest that both cytokines may be involved in the pathogenesis of this virus-associated lymphoproliferative disorder.

scholarly journals Castleman Disease in the Buccal Space Mimicking Lymphoma: A Case Report

2019 ◽  
Vol 7 (3) ◽  
pp. 23
Author(s):  
Mohsen Hashemi ◽  
Javad Yazdani ◽  
Amir Hossein Bani Shah Abadi ◽  
Tannaz Abdollah Zade Baghai ◽  
Behrouz Shokouhi
Keyword(s):  
Case Report ◽  
Lymphoproliferative Disorder ◽  
Rare Case ◽  
Systemic Treatment ◽  
Surgical Excision ◽  
Castleman Disease ◽  
Unknown Etiology ◽  
Histopathological Features ◽  
Buccal Space

Castleman disease is a benign lymphoproliferative disorder of unknown etiology, more commonly appearing in the mediastinum. Craniofacial involvement is rare. Surgical excision is the only intervention needed for the unicentric type, while systemic treatment would be necessary for the multicentric type. Here, we report a rare case of unicentric castleman disease of the buccal space of a 23-year-old woman. Additionally, we review the radiological and histopathological features of this lesion.

scholarly journals Suprarenal unicentric Castelman’s disease: A rare case report

2021 ◽  
Vol 2 (5) ◽  
Author(s):  
Mohammed Arafath Ali ◽  
◽  
Ramachandra C ◽  
Uday Karjol ◽  
Ravi Arjunan ◽  
...  
Keyword(s):  
Ct Scan ◽  
Complete Resolution ◽  
Lymphoproliferative Disorder ◽  
Rare Case ◽  
Clinical Presentation ◽  
Castleman Disease ◽  
Malignant Diseases ◽  
Rare Case Report ◽  
Suprarenal Mass

Castleman Disease (CD) is a rare non-clonal lymphoproliferative disorder. The clinical presentation of CD often overlaps with autoimmune, infectious, or other malignant diseases. The diagnosis is confirmed by a biopsy of the affected lymph-node tissue. A 39-year-old gentleman was evaluated for intractable abdominal pain fatigue of 2 months duration. CT scan showed a 4 X 4 X 5 cm right suprarenal mass. Laparotomy and near total excision was performed which showed reactive B cells expressing CD20 and PAX5, favoring CD. Patient received adjuvant 6 cycles of Rituximab and steroids. A follow up CT scan after 3 months showed a near complete resolution.

scholarly journals Multicentric Castleman Disease - An Unusual Presentation

2013 ◽  
Vol 19 (1) ◽  
pp. 53-57
Author(s):  
Arunabha Chakravarti ◽  
Meenakshi Mishra ◽  
Anita Nangia ◽  
Jatinder Kumar Sahni
Keyword(s):  
Case Report ◽  
Cervical Lymphadenopathy ◽  
Castleman Disease ◽  
Unusual Presentation ◽  
Neck Swelling ◽  
Multicentric Castleman Disease ◽  
Presenting Symptoms ◽  
Histopathologic Diagnosis ◽  
Planning Treatment ◽  
Insight Into

Castleman disease is a rare clinicopathological disorder associated with lymphoproliferation. We report the case of a 54 year old gentleman initially presenting with dysphagia followed by bilateral cervical lymphadenopathy, and later diagnosed as multicentric Castleman disease. To the best of our knowledge no case of Castleman disease presenting as dysphagia along with neck swelling has been previously described in the literature. This case report highlights a very unusual presentation of this rare disease. It also provides insight into the fact that because of the lack of any specific presenting symptoms and distinguishing radiographic features, an accurate histopathologic diagnosis and careful staging become crucial to planning treatment for this disease. DOI: http://dx.doi.org/10.3329/bjo.v19i1.12041 Bangladesh J Otorhinolaryngol 2013; 19(1): 53-57

High levels of human herpesvirus 8 viral load, human interleukin-6, interleukin-10, and C reactive protein correlate with exacerbation of multicentric Castleman disease in HIV-infected patients

Blood ◽  
2000 ◽  
Vol 96 (6) ◽  
pp. 2069-2073 ◽  
Author(s):  
Eric Oksenhendler ◽  
Guislaine Carcelain ◽  
Yoshiyasu Aoki ◽  
Emmanuelle Boulanger ◽  
Anne Maillard ◽  
...  
Keyword(s):  
Viral Load ◽  
Interleukin 6 ◽  
Lymphoproliferative Disorder ◽  
Human Herpesvirus ◽  
Castleman Disease ◽  
Human Herpesvirus 8 ◽  
C Reactive Protein ◽  
Herpesvirus 8 ◽  
Multicentric Castleman Disease ◽  
Reactive Protein

Multicentric Castleman disease (MCD) is a distinct type of lymphoproliferative disorder associated with inflammatory symptoms and interleukin-6 (IL-6) dysregulation. In the context of human immunodeficiency virus (HIV) infection, MCD is associated with human herpesvirus 8 (HHV8) infection. In a prospective study of 23 HIV-infected patients with MCD, clinical symptoms of MCD were present at 45 visits, whereas patients were in chemotherapy-induced clinical remission at 50 visits. Symptoms were associated with a high level of serum C reactive protein, high HHV8 viral load in peripheral blood mononuclear cells, and high plasma human IL-6 and IL-10 levels. Strong correlations between plasma IL-6 and plasma IL-10 with the HHV8 viral load suggest that both cytokines may be involved in the pathogenesis of this virus-associated lymphoproliferative disorder.

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