scholarly journals Idiopathic Multicentric Hyaline Vascular-Type Castleman Disease

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Adelaide Moutinho ◽  
Rita Gamboa Cunha ◽  
Sheila Koch Jamal ◽  
Marta Meleiro Lisboa ◽  
Sandra Tavares

Castleman disease is a rare lymphoproliferative disorder presenting with localized or disseminated lymphadenopathy and systemic symptoms. It can be categorized clinically as unicentric or multicentric, histopathologically as hyaline vascular, plasma cell, or mixed variant, and etiologically, considering the subtypes based on causative viral agents and associated syndromes. The multicentric type can mimic other haematological malignancies, ranging from asymptomatic to multiple organ involvement. Although its pathophysiology is not well known, the current approved treatments are directed towards interleukin-6, CD-20, and viral agents. The authors present an 82-year-old leucodermic man presented with a 2-week history of constitutional symptoms. Examination revealed pallor, hepatosplenomegaly, and palpable left axillary lymphadenopathy. Investigation showed anaemia, thrombocytopenia, polyclonal hypergammaglobulinemia, hypoalbuminemia, and high acute phase reactants, with image study revealing multiple axillary, mediastinal, inguinal, and pelvic lymphadenopathies. The lymph node biopsy was consistent with hyaline vascular-type Castleman disease without human herpersvirus-8 markers. He started prednisolone with initial improvement evolved poorly on a short term. Castleman disease has a broad spectrum of clinical manifestations, associations, and complications that bring a diagnostic challenge, requiring a multidisciplinary approach. Clinicians should be familiar with its features because proper diagnosis and aggressive targeted treatment are the pillars of proper management of these patients.

2015 ◽  
Vol 16 (1) ◽  
pp. 48-50
Author(s):  
Aparna Das ◽  
Sanjana Tarannum ◽  
Tahera Kona ◽  
Santanu Kumar Saha ◽  
MA Kahhar

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


2019 ◽  
Vol 4 (4) ◽  

Castleman Disease (CD) is a lymphoproliferative disorder characterized by enlarged hyperplastic lymph nodes with regressed follicles surrounded by expanded mantle zones of small lymphocytes, and interfollicular vascular proliferation in the hyaline-vascular type. There are two types: unicentric and multicentric. Unicentric, hyalinevascular type of Castleman’s disease can be treated successfully with complete surgical resection with monitoring for reoccurrence. Here we report a case of a patient originally diagnosed with sarcoidosis who was found to have Unicentric Castleman’s disease.


2017 ◽  
Vol 74 (4) ◽  
pp. 367-370
Author(s):  
Bosko Milev ◽  
Borka Milev ◽  
Zoran Kostic ◽  
Darko Mirkovic ◽  
Nenad Perisic ◽  
...  

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.


2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Jacob P. Smeltzer ◽  
Matthew T. Howard ◽  
Wilson I. Gonsalves ◽  
Thomas E. Witzig

Epstein-Barr virus (EBV) is a ubiquitous virus that commonly affects children and adolescents. In addition to causing a viral illness, it is also associated with various malignancies in particular B cell lymphomas and lymphoproliferative disorders. Differentiating between the two processes can be a diagnostic challenge. Here, we present a case of an atypical EBV infection in an elderly patient with severe systemic symptoms, multiorgan involvement, lymphadenopathy, and negative EBV serology. Excisional lymph node biopsy demonstrated features of a lymphoproliferative process involving EBV. Despite supportive care, she experienced continued clinical deterioration and was successfully treated with rituximab. This case illustrates the diagnostic challenges of these cases particularly in the elderly who may have age related immunosenescence, the utility of EBV PCR testing, and the clinical efficacy of rituximab in clearing the infected cells.


2000 ◽  
Vol 25 (2) ◽  
pp. 207-209 ◽  
Author(s):  
L. A. Singletary ◽  
T. J. Karcnik ◽  
H. Abujudeh

2016 ◽  
Vol 10 (2) ◽  
pp. 57-59
Author(s):  
S Gon ◽  
D Mallik ◽  
A Bhattacharya ◽  
B Majumdar ◽  
M Sengupta

Primary retroperitoneal tumours pose a diagnostic dilemma and therapeutic challenges because of their location and late detection. Localized Castleman Disease (CD) in retroperitoneal location is a rare occurrence, with incidence of only 4% cases occurring in diverse locations like retroperitoneum. A rare case of unicentric localized Castleman Disease localized in broad ligament is hereby reported which was clinically & radiologically suspected as broad ligament leiomyoma but finally diagnosed as Castleman’s Disease, hyaline vascular type in a young female posing a diagnostic challenge both for the pathologist and clinician. Definite diagnosis is essential as complete surgical excision in case of unicentric Castleman’s Disease has good prognosis after resection.   


2007 ◽  
Vol 6 (5) ◽  
pp. 455-459 ◽  
Author(s):  
Michael A. Finn ◽  
Meic H. Schmidt

✓ Castleman disease is a rare lymphoproliferative disease of unknown cause. In most cases, afflicted patients present with a mediastinal mass although the disease may manifest in numerous other sites, including intracranially and rarely intraspinally. The authors report on the case of a 19-year-old woman who presented with a large paraspinal mass emanating from the T7–8 neural foramen. The morphological and signal characteristics revealed on magnetic resonance imaging were similar to those of nerve sheath tumors. The patient underwent open biopsy sampling of the lesion, and results of a pathological evaluation revealed hyaline-vascular type Castleman disease. She underwent radiotherapy and remains symptom-free with a radiographically stable lesion 1 year later. Although the disease has been reported to mimic a meningioma when encountered in intracranial locations, to the authors' knowledge, this is the first case of the disorder mimicking a nerve sheath tumor. When the diagnosis of Castleman disease is made, good results can be obtained with partial resection and radiotherapy.


2018 ◽  
Vol 4 (4) ◽  
Author(s):  
Nazia Rashid ◽  
Aamna Hassan ◽  
Noreen Akhter ◽  
Abdul Hameed

A 27-year-old male underwent total thyroidectomy for thyroid swelling. Histopathology showed papillary thyroid carcinoma [T3 - 6.0 cm] with extra-thyroidal extension. The patient was treated with 150 mCi radioactive iodine(RAI) as adjuvant ablative therapy. Radioiodine refractory disease was identified 1-year post-RAI therapy with elevated thyroglobulin levels and negative I-131 whole body scan. F-18 FDG positron emission tomography/computedtomography scan showed activity in the right thyroid bed and multilevel right cervical nodes. Right-sided modified neck dissection was done, which showed Castleman disease (hyaline vascular type) in right cervical nodes. The most probable cause of elevated tumour markers was found out to be 0.6 cm right thyroid bed nodule on follow-up ultrasonography. Our patient also had coexistent conditions as; osteopoikilosis and Hepatitis C along with thyroid carcinoma.Key words: Castleman disease, lymph node, radioiodine, thyroid cancer


2007 ◽  
Vol 127 (3) ◽  
pp. 465-468 ◽  
Author(s):  
Nicole A. Mah ◽  
Samuel J. Peretsman ◽  
Chris M. Teigland ◽  
Peter M. Banks

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