Hyaline vascular Castleman disease associated with POEMS syndrome and cerebral infarction

Abstract

Dear Editor, In 2006, a 42-year-old Chinese woman was admitted to the Endocrine Department of our hospital because of diarrhea for 5 months and chilly for 1 month. She was diagnosed of hyperthyroidism 2 months before admission and was treated with antithyroid drugs. Her prior medical history was unremarkable, with no history of diabetes, smoking, alcohol, or HIV. On admission she was suffering from diarrhea, was experiencing progressive fatigue and chill, night sweats, weight loss, and edema. Positive physical examination revealed multiple small peripheral lymph nodes, hepatomegaly, splenomegaly, hyperpigmentation, paraesthesia in four limbs and pitting edema of the lower extremities. Laboratory tests on admission showed HbsAg (+), HbcAb (+), HbeAb (+) and HIVAb (−). The fibrinogen level was 0.35 g/dl (normal 0.2–0.4 g/dl). Blood biochemistry indicated hypoalbuminemia and hyperglobulinemia. Immunoelectrophoresis showed a polyclonal increase in serum immunoglobulin (IgG 22.7 g/l, IgA 48.3 g/l, IgM 0.66 g/l) with a monoclonal IgA kappa. Ultrasonic examination indicated pericardial effusion. Needle electromyogram test was performed. Slow conduction velocity on sensory nerve and motor nerve was confirmed. Blood routine was normal. Cervical lymph node biopsy diagnosed of Castleman disease (hyaline vascular type) (Fig. 1). Then she was admitted to the Hematology Department because of Castleman disease associated with POEMS syndrome. One day after admission, the patient suffered from mental anomaly, gradually occurred left hemiplegia, and coma. Findings of physical examination revealed fever, hyperpigmentation, ascites, dysarthria, tetraparesis, and areflexia of both legs. The fibrinogen level was 0.412 g/dl. Neither polycythemia nor thrombocythemia was detected. The MRI and DWI scans showed a large area of acute cerebral infarction including right frontal lobe and parietal lobe, left frontal lobe, extending to the cortex and subcortex (Fig. 2). After treatment with dexamethasone 10 mg/day, cerebrospinal fluid pressure lowered; after supportive treatment for 7 days, the consciousness of the patient improved. She became drowsy and could respond to our call. Physical examination showed no fever or ascites, no edema or lymphadenectasis; she could raise her right limbs by herself. The fibrinogen level dropped to normal (0.29 g/dl). Ultrasonic examination indicated no pericardial effusion or splenohepatomegalia. Castleman disease is a rare pathologic process of unknown etiology, characterized by reactive proliferation of lymphoid tissue [1]. The clinical features of Castleman disease are classified into two categories, localized and multicentric. The localized form usually presents in young adults with localized masses in the mediastinum, neck, or, less commonly, intra-abdominal masses. Systemic symptoms are rare with localized Castleman disease (LCD). In contrast, multicentric Castleman disease (MCD) presents with polylymphadenopathy and frequently multi-organ involvement and is associated with systemic features. MCD is less common than LCD and follows a more aggressive natural history. The histology of Castleman disease is similarly divided into two subgroups: the hyaline vascular type and the plasma cell type. The former is found in 90% of LCD, but rarely in MCD and associated with systemic clinical manifestations. The latter is found in only Ann Hematol (2007) 86:59–61 DOI 10.1007/s00277-006-0188-z