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Lupus anticoagulant associated with primary malignant lymphoplasmacytic lymphoma of the spleen: A report of four patients
Author(s) -
Ciaudo M.,
Horellou M. H.,
De Carbonnieres C.,
Conard J.,
Samama M.,
Audouin J.
Publication year - 1991
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830380404
Subject(s) - lymphoplasmacytic lymphoma , medicine , lupus anticoagulant , splenectomy , lymphoma , splenic marginal zone lymphoma , malignancy , partial thromboplastin time , spleen , pathology , gastroenterology , immunology , antibody , waldenstrom macroglobulinemia , coagulation
Primary lymphoma of the spleen is characterized by predominant splenomegaly. Lymphoplasmacytic malignant lymphoma of the spleen, of low malignancy in the Kiel classification, low and intermediate grade in the National Cancer Institute Working Formulation (NCIWF), is rare. It is often associated with a monoclonal immunoglobulin M (IgM). Four patients presenting with primary splenic lymphoma of plasmacytic type associated with a high level of monoclonal IgM and a lupus anticoagulant (LA) are described. This association has not previously been reported. In contrast with the usual heterogeneity of LA, this LA is relatively homogeneous with an important prolongation of the prothrombin time (>18 see for a control of 12), more prolonged partial thromboplastin time (PTT) of the mixture patient + control plasma than PTT of the patient plasma. Despite the important coagulation abnormalities, none of these four patients has presented any hemorrhagic or thrombotic complications, even during major surgery such as splenectomy. The lupus‐like anticoagulant effect ran parallel with the monoclonal IgM. Survival, after splenectomy and chemotherapy, appears to be favourable: three patients are alive with survivals of ≧7 years. The follow‐up is as yet too short for the last patient.