Idiopathic Multicentric Hyaline Vascular-Type Castleman Disease

Abstract

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.

Figure 1

(a) CT scan showing mediastinal lymphadenopathy measuring 35.3 mm (marker); (b) CT scan showing bilateral axillary lymphadenopathies (arrows).

Figure 2

Lymph node biopsy showing features of Castleman disease, hyaline vascular type. (a) HE coloration, 10x amplification. Numerous small follicular structures, follicular dendritic type cells, and blood vessels, some with hyaline walls. (b) HE coloration, 20x amplification. Concentric layers of lymphocytes around the follicles, the mantle zone.