[A patient with lambda type light-chain disease associated with Crow-Fukase syndrome and autoimmune thrombocytopenia].
A 67-year-old woman, who presented polyneuropathy, pleural effusion, ascites and sclerosing changes in the ribs, was admitted to our hospital on June 17, 1987. On admission, cerebrospinal examination showed a marked protein-cell dissociation and a delay in nerve conduction velocity. Bence-Jones protein was detected in urine, and the immunohistochemical study of biopsied bone marrow of the rib revealed lambda-chain positive plasmacytoma. Serum immunoelectrophoresis, however, showed no monoclonal gamma-globulinemia. From the findings described above, she was diagnosed as having Crow-Fukase syndrome associated with lambda-type light chain disease. Even with a therapy by prednisolone, platelet counts progressively declined to 10,000/ml3. Bone marrow aspiration showed normal number of megakaryocytes. Since platelet-associated IgG was increased to 452 ng/1.0 x 10(8) plt, a diagnosis of autoimmune thrombocytopenia was considered. Melphalan and cyclophosphamide to plasmacytoma resulted in a marked improvement of platelets. In addition, the level of platelet-associated IgG returned to normal range. Polyneuropathy, however, didn't respond to those therapies. It was suggested that both Crow-Fukase syndrome and thrombocytopenia were closely concerned with plasmacytoma but developed in a different manner.
Honda
Y
Y
First Department of Internal Medicine, Kurume University School of Medicine.
Murai
I
I
Aramaki
M
M
Shoji
H
H
Oizumi
K
K
D000368:Aged; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications*; D007074:Immunoglobulin G / Q000378:metabolism; D007147:Immunoglobulin Light Chains; D007146:Immunoglobulin lambda-Chains; D008558:Melphalan / Q000627:therapeutic use; D016878:POEMS Syndrome / Q000150:complications*; D010265:Paraproteinemias / Q000150:complications; D013921:Thrombocytopenia / Q000188:drug therapy / Q000209:etiology*
D007074:Immunoglobulin G; D007147:Immunoglobulin Light Chains; D007146:Immunoglobulin lambda-Chains; D003520:Cyclophosphamide; D008558:Melphalan