Cold agglutininemia with acrocyanosis in multicentric Castleman's disease: a case report

We report the first case of multicentric Castleman's disease with acrocyanosis due to cold agglutinins. A 75-year-old female patient presented with fever, weight loss, general malaise and multiple lymphadenopathy for 2 months. During admission, supportive treatment was given and several episodes of acrocyanosis were relieved by oxygen inhalation. Cold agglutination was demonstrated when screen cells and the patient's own RBC were incubated with her serum initially at room temperature with weak positively, at 4 degrees C with 4+ reaction strength, and later dissolved at 37 degrees C. Histopathological examination of the cervical lymph node showed a dense interfollicular plasmacytosis around hyperplastic follicles with inconspicuous capillary proliferation. The plasma cells were mature-appearing with polyclonal immunoglobulin production as shown by immunostaining. These findings were consistent with a plasma cell variant of Castleman's disease. She died of cardiac arrhythmia and subsequent decompensation on the day of the lymphadenectomy, four months after the presentation of apparent symptoms.