Table 3

 Clinical, laboratory, and histological features of patients developing lymphoproliferative neoplasms

CharacteristicsPatient No
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*Evaluated at diagnosing pSS; †developing during the course of disease before lymphoma development, but not present or not analysed at first visit; ‡biopsy in this case contained insufficient glandular tissue for evaluation. §CD4-penia is defined as number of CD4 <300 cells/μl, % of CD4 <30, or ratio of CD4/CD8 <0.8; ¶+  =  focus score >1; **this patient had had a pseudolymphoma in a submandibular gland before her pSS was diagnosed (18 years before the large B cell lymphoma). At re-evaluation of the tissue the lymphoepithelial lesion without full blown malignancy was confirmed.
ND, not done; BM, bone marrow; LN, lymph node.
Age at pSS diagnosis755269546766595052532557
Age at lymphoma/myeloma onset765872617873715765593367
61
Death/age at death+++++++
82627974576139
Symptom duration before pSS diagnosis10162112180618251124
SexFemaleMaleMaleFemaleFemaleFemaleFemaleFemaleFemaleFemaleFemaleFemale
Lymphoma type (WHO)Small lymphocytic B cell/chronic lymphatic leukaemiaSmall lymphocytic B cell+MyelomaFollicular B cellSmall lymphocytic B cell  =  Waldenström MGDiffuse large B cellDiffuse large B cellDiffuse large B cell**Anaplastic large T cellDiffuse large B cellDiffuse large B cellDiffuse large B cellMyeloma + diffuse large B cell
GradeLowLowLowLowHighHighHighHighHighHighHigh(High)
Primary localisationBM, LNsBM, LNsSalivary glandsBMLN groinRight knee hollowSalivary gland? LNs neck, intra-abdominal, BMLNs, liver spleen, BMLung parenchymaBM, LNsSalivary glands,LNsLNs
Salivary gland swelling−*+*−*+†+*+*+*−*+†+†+*−*
Palpable purpura/skin vasculitis−*+*−*−*−*−*+*+*+†+†+*+†
Peripheral neuropathy−*+*−*−*+†+†−*+†+†+†+†−*
Enlarged LN or spleen−*−*−*−*−*−*−*+†+†+−*+*
+†
Concomitant lymphoma predisposing conditionSquamous cell cancer*Psoriasis†Psoriasis* H pylori† Chronic gastritisBasal cell cancer†Hashimoto†H pyloriSquamous + basal cell cancer†Coeliac disease* H pylori† Hashimoto*Sibling with psoriasisBasal cell cancer†
RF+*+*−*+*+†+*+*−*+*+*+*
SSA/SSB−†+*+†+*+*+†−*+†+†+*+*+*
ANA+*+*+*+*+*+*+*+†+*+*+*+*
CryoglobulinaemiaND+†NDNDNDND−*−*+*ND+*ND
Lymphopenia−*−*−*−*−*+*+*+†+*−*+*−*
Anaemia−*−*−*−*−*+*−*−†+*−*+*−*
C3 (g/l)0.70*0.55*0.98*0.83*0.84*0.75*0.50*0.83*0.78*1.01*0.81†0.73*1.06†
C4 (g/l)0.36*0.13*0.27*0.29*0.14*0.16*0.01*0.16*0.18*0.37*0.12†0.06*0.23†
CD4-penia§ND+*+*−*+*+*−†+*+*−*+*+*
IgG (g/l)8.2*14.0*11.9*13.8*16.0*27.0*11.3*23.0*34.1*14.4*36.0*24.6*
IgA (g/l)1.72*2.20*2.20*2.14*2.82*1.38*1.29*1.00*0.13*2.30*3.31*2.98*
IgM (g/l)0.63*2.30*1.76*1.54*0.86*2.03*1.11*1.03*1.33*0.40*1.98*1.51*
Monoclonality−*+* IgG−*+† IgM+† IgM−*, oligo†−*−*−*−*+* IgMOligo*
Salivary gland biopsy¶+*+*+*+*+*+*+*+*+*+*ND