Lupus anticoagulant associated with primary malignant lymphoplasmacytic lymphoma of the spleen: a report of four patients.

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 (greater than 18 sec 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 greater than or equal to 7 years. The follow-up is as yet too short for the last patient.

[Erythrocytic and immunologic abnormalities in myeloid splenomegaly]. / Anomalies erythrocytaires et immunitaires dans la splénomégalie myéloide.

Screening for red cell defects, and exploration of cellular and humoral immunity has been performed in 33 patients : 31 had agnogenic myelosclerosis with myeloid metaplasia, 3 had polycythemia vera with secondary myelosclerosis. No patient had the biological abnormalities characteristical of paroxysmal nocturnal hemoglobinuria (Marchiafava-Micheli). In 19 out of 21 cases, red cells had antigen i on their membrane, thus suggesting that splenic erythropoiesis could give rise to immature erythrocytes. Two patients had a monoclonal dysglobulinemia, 5 a positive Coombs test, 6 a rhumatoid factor in the serum, 3 antitissue antibodies, 1 LE cells, 3 a positive Paul-Bunnel-Davidsohn test without mononucleosis, 11 a negative skin test. Implications of the uncommon occurrence of these dissorders are discussed.

[4 cases of acquired Willebrand factor deficiency associated with monoclonal dysglobulinemia]. / Quatre cas de déficit acquis en facteur Willebrand associés á une dysglobulinémie monoclonale.

Acquired von Willebrand syndrome is reported in four patients with monoclonal IgG: benign gammapathy in three cases, multiple myeloma in one case; to our knowledge, this last association has not been previously reported. Coagulation abnormalities included a borderline bleeding time, a low platelet retention on glass beads, decreased levels of factor VIII coagulant activity (VIII: C), factor VIII related-antigen (VIII R: Ag) and ristocetin induced agglutination cofactor (VIII R: RC). The late clinical onset, the negative family history and the immunological abnormality suggest an acquired von Willebrand syndrome. After cryoprecipitate infusion the patients did not show the expected rise and there was no secondary increment in factor VIII: C. Time-dependent inhibition of factor VIII R: RC and factor VIII: C was found in one case only and was associated with qualitative abnormality of factor VIII R: Ag demonstrated by crossed-immunoelectrophoresis. It was not possible to interpret this last test in the other cases, due to the very low level of factor VIII R: Ag. The factor VIII abnormalities might be related to the binding and/or destruction of factor VIII by a circulating antibody, or to the adsorption of this factor on the malignant lymphocytes.