Unusual presentation of hyaline vascular Castleman's disease.

Sir, Castleman’s disease, also known as Angiofollicular lymphnode hyperplasia is a rare disorder of lymphoreticular system with heterogenous clinical manifestations.1 Histopathologically, it has two variants1 hyaline vascular variant and plasma cell variant. While hyaline vascular variant usually present with localized yet marked lymphadenopathy2 with few constitutional symptoms, lymphoreticular involvement is more widespread in plasma cell variant with a variety of constitutional symptoms. However, there are as yet no reports of hyaline vascular variety presenting as generalised lymphadenopathy with constitutional features like splenomegaly and pancytopenia. Recently, we came across a patient of hyaline vascular Castleman’s disease presenting with atypical features of multicentricity and prominent systemic involvement. A 35yrs female, weighing 40kg presented with complaints of glandular enlargement in both sides of neck and both axillae of 4 months duration along with fever of 3 months duration. The glandular enlargement appeared simultaneously in neck and axillae. She was pale and non-icteric. Clinical examination revealed bilateral multiple cervical lymphnodes along with bilateral axillary lymphnodes with average size of 3cm × 2cm, firm in consistency, mobile, nontender, nonmatted. Examination of abdomen revealed 3cm palpable, firm, nontender splenomegaly. The laboratory investigations are given in Table 1. A diagnosis of multicentric variety of hyaline vascular variant of Castleman’s disease with systemic features was made. Patient was treated with oral prednisolone 80mg/day for 1 month with gradual tapering over next 3month with monthly follow up. The patient showed significant improvement with complete disappearance of lymphadenopathy and pancytopenia with Hb 11gm/ dl, TLC 8300/mm3, Platelets 1.3lac/mm3 after 3 months of treatment. Neither the lymphnodes nor the constitutional symptoms recurred after 8 months of follow up. Castleman’s disease1 is a rare lymphoproliferative2 disorder with two distinct variants yet described i.e. hyaline vascular variant and plasma cell variant. The presentation of hyaline vascular variant is almost always a solitary lymphnode3 mass with absence of systemic manifestations. Whereas plasma cell variant presents with multiple lymphnodes with systemic features like fever, malaise, hepatosplenomegaly and pancytopenia. The literature4 does mention a few rare cases of mixed histology usually presenting as localised mass. However, pure hyaline vascular variant presenting as multicentric disease is not yet reported in literature to the best of our knowledge. The exact nature of Castleman’s disease is unknown. Various hypothesis have been put forward including hyperplastic, reactive, hemartomatous, immune and mixed. IL-6, a potent cytokine with lymphoproliferative properties has been found to be elevated in a few observations studies. In our case too, it was elevated to 20pg/ml. The case highlights that the two variants of Castleman’s disease may be the two poles of the same disease and forms in between the two with mixture of features of both are possible. Rarely, hyaline vascular variant can have multicentric presentation as in our case. Dinesh Kumar*, Rajni** *Senior Resident; **Post-graduate Resident; Department of Medicine, Institute of Medical Sciences, Banaras Hindu University, Varanasi-221005. Received : 24.8.2006; Revised : 20.11.2006; Re-Revised : 30.1.2007; Re-Re-Revised : 22.2.2008; Accepted : 16.4.2008