Publication

Abstract : Background Castleman’s disease ( angiofollicular lymph node hyperplasia), is a lymphoproliferative disorder. It has association with HIV and HHV-8and is associated with a number of malignancies. Disease presents in two forms: Unicentric with local symptoms and the multicentric form with systemicfeatures. Case report A 65 year old male presented with four months history of fatiguability, low grade fever and swellings in the neck. On evaluation he had anaemia with generalized lymphadenopathy and hepatosplenomegaly. Peripheral smear revealed microcytic hypochromic anaemia with marked rouleaux formation and lympho-plasmacytoid cells. Serum LDH was high and direct coombs test was positive. Lymph node biopsy revealed effacement of nodal architecture with paracortical expansion by plasma cells and lymphocytes. Bone marrow had 22% plasma cells and immunophenotyping was positive for CD 2, 4, 5, 8, 45, 38. Beta 2 Microglobulin- 13399 ug/l (510- 1470) S. Immunoglobulin- IgG- 9565 mg/dl (700-1600). PET CT was suggestive of generalized lymphadenopathy and splenomegaly. Final diagnosis of multicentric plasma cell rich castleman’s disease was made.Patient received steroids followed by 6 cycles of CHOP regime. He continued to be in remission for 8 months and then and was lost to followup. Case two: A 24 year old man presented with three year history of gradually progressive swellings in the neck, fever and night sweats. Examination revealed bilateral multiple cervical lymphadenopathy and rest of the examination was unremarkable. Bone marrow had 5% plasma cells . CT scan revealed multiple cervical lymphadenopathy and lymph node biopsy was suggestive of hyaline vascular variant of castleman disease. Patient has received 2 cycles of CHOP and his lymphadenopathy has completely regressed, he continues to be in followup. Conclusion There are few case reports of this disease from India. Treatment options for disease variants are different.