[Idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia (IPL) accompanied with severe thrombocytopenia and interstitial pneumonitis].
A case of generalized lymphadenopathy with severe thrombocytopenia and marked polyclonal hypergammaglobulinemia is reported. A 51-year-old man was admitted to the hospital because of nasal bleeding and generalized lymphadenopathy. Laboratory examination revealed a platelet count of 6,000/microliters, a total protein level of 11.6 g/dl, and a gammaglobulin level of 8.7 g/dl including 6,726 mg/dl IgG, 1,119 mg/dl IgA, 265 mg/dl IgM, 265 mg/dl IgD and 226 IU/ml IgE. Neither definite evidence suggesting viral infection nor an autoimmune disease was found except slightly elevated titers of anti-microsome and anti-thyroglobulin antibodies. The chest X-ray revealed diffuse reticular shadowing suggesting interstitial pneumonitis. The cervical lymph node biopsy revealed massive infiltration by IgG-, IgA-, and IgM-positive plasmocytoid cells in the paracortical T cell areas, but no destruction of normal architecture of lymph node. The treatment with prednisolone resulted in a decrease in the severity of the lymphadenopathy, thrombocytopenia and polyclonal hypergammopathy. He is still well; however, interstitial pneumonitis, mild thrombocytopenia, and polyclonal hypergammopathy have persisted for 4 years. The histological findings in this case are similar to those in the case of Castleman lymphoma of a plasma cell type. Thus, considering the clinical data and clinical course, the diagnosis in this case is likely to be idiopathic plasmacytic lymphadenopathy with polyclonal hyperglobulinemia as a subtype of the plasma cell type of Castleman lymphoma. However, the accompanying interstitial pneumonitis and the severe thrombocytopenia, which was caused by excessive platelet destruction or by the suppression of megakaryocytes by proliferated plasma cells, are unique and are not found in any of the previously reported cases.
Tsutani
H
H
First Department of Internal Medicine, Fukui Medical School.
Kamiya
K
K
Domae
N
N
Kagawa
D
D
Uchida
M
M
Nakamura
T
T
D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications* / Q000276:immunology; D007074:Immunoglobulin G / Q000032:analysis; D008206:Lymphatic Diseases / Q000150:complications* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000150:complications*; D013921:Thrombocytopenia / Q000150:complications*
D007074:Immunoglobulin G