Systemic Castleman’s disease

Sir,

Castleman’s disease (CD) is a rare atypical lymphoproliferative disorder characterized by enlarged hyperplastic lymph nodes with striking vascular proliferations. CD represents a morphologically distinct form of lymph node hyperplasia rather than a neoplasm or a hamartoma. It is characterized by enlarged hyperplastic lymph node with striking vascular proliferation. ^[1] CD can be either localized or disseminated (multicentric or systemic) and further subdivided into hyaline-vascular, plasma cell or mixed histopathological patterns. ^[2] The multicentric or systemic form is nearly always of the plasma cell type, although occasional examples of the hyaline vascular type are on record. ^[3]

A 70-year-old female was admitted with complaints of bilateral axillary and inguinal lymph node swellings, itching all over the body, and generalized weakness since 15 days. General examination revealed pallor, bilateral axillary and inguinal lymph node enlargement, and mild splenomegaly.

She was human immunodeficiency virus (HIV) and rapid plasma reagin (RPR) nonreactive. Her erythrocyte sedimentation rate (ESR) was 80 mm/h. Her peripheral smear findings were unremarkable. Ultrasonography revealed splenomegaly, para-aortic, and mesenteric lymphadenopathy. Fine-needle aspiration cytology (FNAC) of the right and left axillary lymph nodes [Figure 1] and [Figure 2] showed polymorphous population of lymphoid cells along with histiocytes in the background of blood suggesting reactive lymphadenitis. A lymph node biopsy was advised.

Right inguinal lymph node biopsy was received in the form of single, well-circumscribed, grayish white, firm oval tissue bit of size 3 cm × 2 cm × 1 cm with focal areas of congestion.

Lymph node histopathology [Figure 3] and [Figure 4] revealed numerous small involuted follicular centers evenly distributed throughout the node parenchyma. The follicles were surrounded by concentric layers of lymphocytes and had prominent central vessels, some of which have a hyaline wall and prominent endothelial cells. The interfollicular areas showed small vessels, absence of sinuses, and variable numbers of lymphocytes, plasma cells, eosinophils, and immunoblasts. Impression of angiofollicular hyperplasia of lymph node was kept on histopathological examination.

Immunohistochemistry (IHC) was advised in view of generalized lymphadenopathy, mild splenomegaly, skin changes in the form of itching, and raised ESR to confirm systemic form of CD and to rule out small cell lymphoma which was the closest differential diagnosis on histopathological examination.

On IHC, germinal center cells [Figure 5] showed weak CD10 immunopositivity. Immunostains for BCL2 [Figure 6] and CD20 [Figure 7] showed expected distribution while cyclin D1/desmin [Figure 8] was negative. There was no evidence of malignancy or lymphoma. Impression on IHC was regressive transforming germinal centers with features of CD.

Castleman’s disease typically presents in patients’ ages 50-60 years, but for those who are HIV positive, it presents at younger age. ^[4] The first case of multicentric CD (MCD) which presented as generalized lymphadenopathy with systemic manifestations of fever, night sweats, weight loss, and fatigue was reported in 1978. ^[5] Pathogenesis includes infections, autoimmunity, and dysregulated cytokine expression causing lymphoid proliferations. Treatment can range from curative surgery for the hyaline form of CD to the use of chemotherapy for MCD. It is important to consider CD in the differential diagnosis for patients presenting with extensive lymphadenopathy, especially those with acquired immunodeficiency. Factors found to be associated with CD are HIV infection/AIDS, hypoalbuminemia, POEMS (polyneuropathy, organomegally, endocrinopathy, monoclonal protein, and skin changes) syndrome, hypergammaglobulinemia, fever of unknown origin, and night sweats. Our patient demonstrated generalized lymphadenopathy, mild splenomegaly, and increased ESR. She was HIV and RPR nonreactive. Her FNAC and histopathological diagnosis were reactive lymphadenitis and angiofollicular hyperplasia (CD of hyaline vascular type) of lymph node, respectively. IHC was done to rule out lymphoma and to confirm the diagnosis of MCD.

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1755-6783.149505

[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]