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Klinische Wochenschrift

, Volume 65, Issue 18, pp 852–859 | Cite as

Lupus anticoagulant associated syndrome in benign and malignant systemic disease — Analysis of ten observations

  • U. Dührsen
  • D. Paar
  • C. Kölbel
  • A. Boekstegers
  • U. Metz-Kurschel
  • R. Wagner
  • W. Kirch
  • P. Meusers
  • E. König
  • G. Brittinger
Originalien

Summary

The clinical and laboratory findings in seven female patients with primary autoimmune diseases, one female patient with lymphoplasmacytoid (LP) immunocytoma and IgM paraproteinemia, and two male patients with multiple myeloma are described. The common denominator in all patients was a lupus anticoagulant or a closely related coagulation disorder. Recurrent thrombosis was observed in six patients with autoimmune diseases and in two patients with malignant monoclonal gammopathies. Other clinical manifestations included cerebral disorders (four patients with autoimmune disease/two patients with monoclonal gammopathy), repeated obstetric complications (6/1), asymptomatic valvular heart disease (6/1), renal dysfunction (6/2), hepatic involvement (2/2), and arthropathy (2/0). Laboratory investigations revealed a biologic false-positive serological test for syphilis in six patients with autoimmune disease and one with monoclonal gammopathy, antinuclear antibodies (4/0), antibodies against DNA (4/1), and a positive direct Coombs test (3/1) which was accompanied by hemolytic anemia in two patients (1/1). Additionally slight leucocytopenia (2/1) and thrombocytopenia (6/2) were observed; abnormal bleeding was only seen in one patient with severe thrombocytopenia. Other complications characteristic of LP immunocytoma or multiple myeloma were missing.

The obvious similarities between the patients with autoimmune diseases and the patients with malignant monoclonal gammopathies suggest analogous pathogenetic mechanisms. Since the clinical syndrome associated with the lupus anticoagulant in patients with autoimmune disorders has been proposed to arise from the action of autoantibodies against phospholipids, it is attractive to hypothesize that the findings in the patients with malignant diseases were caused by an autoantibody activity of the monoclonal immunoglobulin. This assumption is substantiated by the observation that in one patient the paraprotein level correlated with the prolongation of the coagulation times and the severeness of the clinical perturbations. Although paraproteins mimicking the laboratory alterations of the lupus anticoagulant have been reported before, the corresponding clinical features have not yet been described in malignant monoclonal gammopathies. The syndrome may be juxtaposed to other systemic disorders complicating paraproteinemias, and is possibly more frequent than is as yet known.

Key words

Lupus anticoagulant Paraproteinemia Systemic lupus erythematosus Multiple myeloma Lymphoproliferative diseases Lymphoma 

Abbreviations

aPTT

Activated partial thromboplastin time

BFP-STS

Biologic false-positive serological test for syphilis

DNA

Deoxyribonucleic acid

GN

Chronic glomerulonephritis

IC

Lymphoplasmacytoid immunocytoma

LA

Lupus anticoagulant

LP immunocytoma

Lymphoplasmacytoid immunocytoma

MM

Multiple myeloma

PN

Panarteritis nodosa

POEMS syndrome

Polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes

PT

Prothrombin time

SLE

Systemic lupus erythematosus

VDRL

Venereal Disease Research Laboratory

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Copyright information

© Springer-Verlag 1987

Authors and Affiliations

  • U. Dührsen
    • 1
    • 2
  • D. Paar
    • 1
  • C. Kölbel
    • 1
  • A. Boekstegers
    • 1
  • U. Metz-Kurschel
    • 1
  • R. Wagner
    • 1
  • W. Kirch
    • 1
  • P. Meusers
    • 1
  • E. König
    • 1
  • G. Brittinger
    • 1
  1. 1.Medizinische Klinik und Poliklinik der Universität (GHS) EssenGermany
  2. 2.The Walter and Eliza Hall Institute of Medical Research Cancer Research Unit Post OfficeThe Royal Melbourne HospitalVictoriaAustralia

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