primary immunodeficiency-associated lymphoproliferative diseases

Lymphoproliferative disorders (LPDs) are a severe complication in primary immunodeficiency and post-transplant patients. In primary immunodeficiency patients, LPDs are not well-known.

PIALPDs are frequently extranodal, EBV-associated, and are more commonly pleomorphic in primary immunodeficiency than in post-transplant patients.

A low T-cell count and abnormal T-cell function indicate bad prognosis.

Polymorphic LPDs (PLPDs) are most frequent, whereas lymphomas are rare, and pseudo-tumoral lymphoid hyperplasias were observed only in primary immunodeficiencies.

Comparative p53/bcl-2 staining reveals a p53 overexpression in lymphomas compared with PLPDs; CD20/CD79a show a similar staining in lymphomas, whereas PLPD expressed mainly CD20.

TCR and IgH rearrangements do not help in distinguishing PLPDs from lymphomas, but detection of IgH clonality by Southern blot indicate poor prognosis, whereas oligoclonality by Southern blot regardless of PCR clonality and especially a polyclonal profile by Southern blot and PCR indicate a relatively good prognosis.

The studies document the pleomorphism of LPDs in primary immunodeficiency compared to post-transplant children, even if some LPDs are similar in both groups (PLPDs).

No criteria are useful enough to ascertain the diagnosis of malignancy in this series.

Some molecular biological criteria help to predict the clinical outcome which, nevertheless, seems to depend more on the degree of immunosuppression and on T-lymphocyte presence and function.

Examples

 CVID-associated lymphoproliferative diseases

See also

 immunosuppression-associated lymphoproliferative diseases (ISALPDs)

• post-transplantation lymphoproliferative diseases (PTLDs)

 AIDS-associated lymphoproliferative diseases (ALDs)
 lymphoproliferative diseases (LPDs)
 lymphoproliferation (LP)
 immunosuppression-associated lymphomas (ISALs)

References

 Lymphoproliferative disorders in children with primary immunodeficiencies: immunological status may be more predictive of the outcome than other criteria. Canioni D, Jabado N, MacIntyre E, Patey N, Emile JF, Brousse N. Histopathology. 2001 Feb;38(2):146-59. PMID: 11207828