Stefan Raffac, Slovak Republic
L.Pasteur University Hospital, Rafmed s.r.o, OZRO Dep.of clin. ImmunologyPresenter of 1 Presentation
NON HODGKIN LYMPHOMA IN PATIENT WITH CVID
Abstract
Background and Aims
We would like to report the specific problem of lymphoproliferation in patients with CVID.
Methods
Case report
Results
This case reports 65 years old lady with CVID, diagnosed in 2013. Later splenomegaly and decreas in thrombocytes and leukocytes were detected.Flow cytometry of peripheral blood showed patological lymphocytes. Bone marrow(BM): flow cytometry: 9.1% B-lymfocytes, 50% of B ly with pathological phenotype CD23-/+/CD5-/CD10- , mFLCKappa - dg. B chronic lymphoproliferative disease B-CLPD. Histology: NO evidence of malignant changes and NO evidence of infiltration with malignant lymohoma. Clinicaly was patient stable, with No evidance of B symtomatology. Splenomegaly did not progreeded and changes in blood count stayed mild. In terapy we administered immunoglobulins substitution regularly.
Four years later splenomegaly and lukopenia progreeded. New BM biopsy shows 15% infiltration of bone marrow with patological lymphocytes, morphologicaly consistnet with small cellular CD 20+malignant lympfoma, most likely splenic marginal zone lymphoma. Because there are porgessive leukopenia we decided to star the therapy with anti CD20 rituximab.
Date | Spleen (cm) | Lekocytes (x 109/L) | Thrombocytes (x 109/L) |
XI/2013 | 16x10 | 6,3 | 132 |
X/2015 | 18x10 | 3,0 | 80 |
VI/2017 | 17x9 | 4,5 | 110 |
III/2019 | 20x12 | 1,6 | 75 |
Conclusions
Primary immunodeficiency is complex immune system dysregulation. This can lead to broken balance of lymphocytes and formation of lymphoproliferation. Lymphocytes proliferate from polyclonal to oligoclonal and finaly to monoclonol, malignat lyphoproliferative disease. Ethiology of this proces is multifactorial. Management of lyphoproliferation is complicated and has to consider risk associated with conventional cytostatik agent, or splenectomy. Therfore is better to choose newer drug, e.g. monoclonal antibodies. Crucial is immunoglobulin substitution and infections prevention.