[Multicentric Castleman disease-like case characterized by lymphadenopathy and polyclonal hypergammaglobulinemia associated with so-called Mikulicz disease].
A 73-year-old male was admitted in January 1999 with hyperimmunoglobulinemia with a serum IgG level of 6530 mg/dl, bilateral eyelid tumors, bilateral submandibular swelling, and swelling of the superficial lymph nodes. A left submandibular gland biopsy showed severe chronic sialoadenitis with fibrosis. A left cervical lymph node biopsy showed invasion by many mature lymphocytes and plasma cells, but no lymphoma cells. The patient was diagnosed as having so-called Mikulicz disease associated with a disease similar to multicentric Castleman disease (MCD) characterized by multicentric lymphadenopathy and polyclonal hyperimmunoglobulinemia. Steroid therapy (prednisolone, 20 mg/day) was effective in reducing the symptoms, the bilateral eyelid tumors and the swelling of the bilateral submandibular glands and superficial lymph nodes. The lack of any increase of serum IL-6 suggested that this case had a hitherto unknown etiology, other than MCD.
Ohta
Tadanobu
T
Department of Internal Medicine, Seichoukai Fuchu Hospital.
Furukawa
Yoshio
Y
Aoyama
Yasutaka
Y
Kumura
Takeo
T
Mugitani
Atsuko
A
Nakamae
Hirohisa
H
Koh
Kyron
K
Yamane
Takahisa
T
Hino
Masayuki
M
D000368:Aged; D005871:Castleman Disease; D003937:Diagnosis, Differential; D006801:Humans; D006942:Hypergammaglobulinemia / Q000188:drug therapy / Q000209:etiology*; D007074:Immunoglobulin G / Q000097:blood*; D015850:Interleukin-6 / Q000097:blood; D008206:Lymphatic Diseases / Q000188:drug therapy / Q000209:etiology*; D008297:Male; D008882:Mikulicz' Disease / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D011239:Prednisolone / Q000627:therapeutic use; D016896:Treatment Outcome
D007074:Immunoglobulin G; D015850:Interleukin-6; D011239:Prednisolone