Multiple M components in two patients with splenic lymphoma with villous lymphocytes.
Splenic lymphoma with villous lymphocytes (SLVL) is a rare lymphoproliferative disorder characterized by the presence of typical lymphoid cells with villous projections and monoclonal immunoglobulin (M-Ig) in about 30% of patients. The simultaneous presence of more than one M-Ig in SLVL has not been reported. We present two patients with SLVL, each with three serum M components associated with the presence of rheumatoid factor (RF) and antiphospholipid antibodies (APLA) together with fatal thromboembolic events. Both patients presented with splenomegaly and typical bone marrow cytology with 30-50% infiltration of lymphoid cells that had the characteristics of villous lymphocytes. Immunohistochemistry of bone marrow histology showed CD20++, CD43-/+, CD5-, IgM+, lambda+ and kappa-. In serum, two M-IgM lambda components were combined with M-IgG lambda in case 1 and with M-IgA lambda in case 2. In both cases, M-IgM displayed RF as well as lupus anticoagulant activity and free monoclonal lambda (lambda) light chains were present. In addition to M-IgM, in case 1 M-IgG also behaved like an APLA. One patient was splenectomized. Both patients suffered thromboembolic complications and died 3 and 8 months after presentation with signs of massive pulmonary thromboembolism.
Colović
Natasa
N
Institute of Hematology, Clinical Center of Serbia, Belgrade, Serbia and Montenegro.
Miljić
Predrag
P
Colović
Milica
M
Milosević-Jovcić
Nadezda
N
D000368:Aged; D017152:Antibodies, Antiphospholipid / Q000097:blood; D015703:Antigens, CD / Q000097:blood; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007145:Immunoglobulin kappa-Chains / Q000097:blood; D007146:Immunoglobulin lambda-Chains / Q000097:blood; D008214:Lymphocytes / Q000378:metabolism / Q000473:pathology; D016393:Lymphoma, B-Cell / Q000097:blood* / Q000150:complications / Q000473:pathology / Q000601:surgery; D008875:Middle Aged; D009194:Myeloma Proteins / Q000032:analysis; D011655:Pulmonary Embolism / Q000097:blood* / Q000209:etiology / Q000473:pathology / Q000601:surgery; D013156:Splenectomy; D013160:Splenic Neoplasms / Q000097:blood* / Q000150:complications / Q000473:pathology / Q000601:surgery; D013163:Splenomegaly / Q000097:blood / Q000150:complications / Q000473:pathology / Q000601:surgery
D017152:Antibodies, Antiphospholipid; D015703:Antigens, CD; D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains; D009194:Myeloma Proteins