Abstract
Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2872096831190851.Castleman disease (CD), described as a heterogeneous lymphoproliferative disorder, can be divided into different subtypes according to clinical appearance (unicentric and multicentric form) and histopathological features (hyaline vascular, plasma cell, mixed type, human herpesvirus 8–associated and multicentric not otherwise specified). Unicentric CD is known to be usually of the hyaline vascular variant, plasma cell and mixed type of this form are quite uncommon. Malignancies are mainly associated with the multicentric form. We report a rare case of unicentric mixed variant CD evolving into intrabronchial, extramedullary plasmacytoma.Intrabronchial mass with consequential obstruction of the left main bronchus, left lung atelectasis and mediastinal lymphadenomegaly was detected by chest CT in our patient suffering from cough and hemoptysis. Pulmonectomy was performed, histopathological and immunhistochemical analysis of lymph nodes revealed mixed type of CD with interfollicular monotypic plasma cell proliferation. The intrabronchial mass consisted of monotypic plasma cells confirming plasmacytoma. Systemic involvement was not confirmed by further tests.Although malignancies more often present in multicentric CD that usually belongs to the plasma cell subtype, this case confirms the neoplastic potential of the rarest, unicentric mixed variant of CD.
Highlights
Castleman disease (CD) - known as giant lymph node hyperplasia or angiofollicular lymph node hyperplasia - has a wide spectrum of appearances due to its heterogeneous pathological and clinical characteristics
HHV8 positive CD, a special subtype of multicentric CD, may be associated with a special entity named “large B-cell lymphoma arising in Human herpesvirus (HHV)-8-associated multicentric CD” or “human herpesvirus 8 (HHV-8)-positive plasmablastic lymphoma” consisting of IgM lambda expressing immunoblasts that are located in the mantle zone
Its role was suggested in the development of B cell lymphomas and plasmacytomagenesis, it may be a link between plasmacytoma and plasma cell type of CD [26]
Summary
Castleman disease (CD) - known as giant lymph node hyperplasia or angiofollicular lymph node hyperplasia - has a wide spectrum of appearances due to its heterogeneous pathological and clinical characteristics. Multicentric CD frequently associated with HIV infection and Kaposi’s sarcoma, is more aggressive and usually high risk for malignant transformation into lymphoma or other malignant lymphoproliferative diseases [1] This form often occurs with systemic symptoms (such as fever, weight loss, night sweats, splenomegaly) and abnormal laboratory results (hypergammaglobulinemia, elevated liver enzymes, anemia, thrombocytopenia and elevated interleukin (IL)-6 levels) [2,3]. Histological examination of the obtained two samples showed groups of cells with small round nuclei in the Figure 2 2 D chest CT image of the intrabronchial mass located 3 cm from the main carina. Immunohistochemistry demonstrated the plasma cells to be positive for CD 31 with immunoglobulin lambda light chain restriction (Figures 6 and 7) These findings were consistent with the mixed variant of CD associated with endobronchial plasmocytoma. Tree years after surgery the patient is asymptomatic, with no signs of recurrence of the disease
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