Castleman disease. A report of six cases.

Abstract

Background and aims:Castleman’s disease is a rare disorder situated at the boundary between reactive and neoplastic conditions. The pathogenesis is a subject of debate and the limited number of cases renders the study of the disease difficult.In our paper we present a series of six cases of Castleman disease with emphasis on the clinical presentation, pathology examination and the use of immunohistochemistry in the final diagnosis of the cases.Patients and method:The classification of the disease was based on clinical, imaging and pathological assessment. Specimens were obtained by surgical excision and were routinely processed for the pathology examination.Results:All cases were unicentric disease. Two cases were locally extensive. The clinical symptoms were related mostly to compression effects. Five case were of the hyaline-vascular type and one was included in the plasma cell variant. One case showed angiomyoid differentiation.Conclusions:We strongly believe that by understanding the pathogenesis of the precursor lesions we will gain better understanding of the pathways that lead to neoplasia and that Castleman disesase is a very interesting ”natural experiment” illustrating the progression from chronic antigen stimulation to reactive lymphoid hyperplasia and finally to overt lymphoid neoplasia.

Castleman’s disease in the HIV-endemic setting

“…The only study that we found is a descriptive retrospective study conducted at Chris Hani Baragwanath Academic Hospital (CHBAH), Johannesburg, South Africa, over a 25-year period (1990–2014). 8 On the African landscape, however, only isolated case reports have been published. Thus, we anticipate that this study will shed some light on the epidemiology of CD in the HIV-endemic setting and may direct the way in how we approach the diagnosis and staging using bone marrow biopsy of this condition.…”

HIV-associated multicentric Castleman disease: a report of 19 cases at an oncology institution

“…On physical exam, there are multiple areas of lymphadenopathy, hepatosplenomegaly, edema, pulmonary signs, and pleural and pericardial effusion. 11–14 We describe multiple lymphadenopathy in 95% of cases, systemic symptoms in 63%, and nearly 50% of the patients had hepatosplenomegaly. The reported laboratory abnormalities are those associated with chronic and inflammation-related diseases such as anemia, thrombocytopenia, elevation of inflammatory markers, and hypoalbuminemia.…”

Multicentric Plasma-Cell Type Castleman Disease Masquerading As Hodgkin Lymphoma: A Case Report

“…4 The plasma cell subtype of CD is characterized histomorphologically by sheets of mature plasma cells within the interfollicular zones of the lymph node interspersed by surrounding larger/hyperplastic germinal center with less vascularity. 1,11 The MCD form of CD is associated with this plasma cell variant. 1 It usually affects older patients and clinically it is characterized by generalized lymphadenopathy, constitutional symptoms, multisystem organ involvement, and deranged laboratory findings.…”

“…The application of IHC as an ancillary technique in this case was especially critical in deciphering this case given the diagnostic dilemma initially presented by her overlapping clinical features and the initial Hodgkin's lymphoma pathologic diagnosis; thus IHC evaluation in combination with routine histopathological evaluations has been recommended as a standard method for lymph node examination especially where routine histopathological evaluation is nonspecific. 1,5,[11][12][13][14][15]19 Surgical resection of the affected lymph node is the treatment of choice for UCD, usually with no risk of recurrence; however, the MCD variant requires multimodal therapies with cytotoxic chemotherapy or CVAD (cyclophosphamide, vincristine, doxorubicin, dexamethasone, or prednisolone)/CHOP (cyclophosphamide, vincristine, doxorubicin, dexamethasone, or prednisolone), corticosteroids (dexamethasone/prednisolone), immunomodulators (lenalidomide or thalidomide, bortezomib, interferon α), intravenous immunoglobulins, plasmapheresis, radiotherapy, monoclonal antibodies (tocilizumab, siltuximab, and rituximab) and autologous hematopoietic stem cell transplantation. 2,6,9,18,20 In accordance with these literature our patient was successfully treated with CHOP.…”

“…1,2,6,8,10 Histomorphologically CD is distinct from malignant lymphoma; however, these two lesions can coexist or even, indeed, mimic each other like in this case report. 1,6,[11][12][13][14][15][16][17][18] In such scenarios, immunohistochemical (IHC) evaluation of the lymph node specimen in addition to routine H&E histopathological evaluation becomes the gold standard in arriving at a definitive diagnosis. 1,5,11,[13][14][15]19 This case is reported because of its rarity and thus should be considered in the differential diagnosis of generalized lymphadenopathy, and also to show how important IHC is in resolving diagnostic dilemmas in lymph node pathology.…”