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2014 ; 30
(Suppl 1
): 126-30
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Multicentric, Hyaline Vascular Variant of Castleman s Syndrome
#MMPMID25332557
Mukta V
; Susmitha C
; Kar R
; Basu D
; Das AK
Indian J Hematol Blood Transfus
2014[Sep]; 30
(Suppl 1
): 126-30
PMID25332557
show ga
Castleman's disease is a nonneoplastic adenopathy, also known as angiofollicular
or giant lymphnode hyperplasia. Its multicentric form presents with wide spread
lymphadenopathy and systemic symptoms. POEMS syndrome (polyneuropathy,
organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) is observed in
11-30 % of the patients with Castleman's disease (Dispenzieri et al., Blood
101:2496-2506, 2003). Tuberculosis and lymphoma are the common diagnosis in
patients with clinical features of weight loss, lymphadenopathy,
hepatosplenomegaly and lymphocyte predominant ascites. We had a similar
presentation in a young male, who was a diabetic, with clinical features
suggestive of probable tuberculous infiltration of abdomen and adrenals causing
addisonian features, lymphocyte predominant ascites, hepatosplenomegaly and
generalized lymphadenopathy. However, a pathological diagnosis of hyaline
vascular variant of Castleman's disease was obtained on lymphnode biopsy, which
propelled us into doing a skeletal survey and bonemarrow biopsy. A final
diagnosis of multicentric Castleman's disease with reactive plasmacytosis was
achieved. Patient had osteosclerotic lesion in the right femur with evidence of
polyneuropathy, organomegaly, endocrinopathies and skin hyperpigmentation. He has
improved on chemotherapy with melphan and dexamethasone. Hyaline vascular variant
of Castleman's disease rarely presents with systemic symptoms. Follow up is
essential, as they can manifest with complications like POEMS and lymphoma.