POSTER'44'
LYMPHOMA'AND'COMMON'VARIABLE'IMMUNODEFICIENCY'I'A'CASE'
REPORT'
'
Cátia!Sol!Reis1,!Ana!Carneiro1,!Manuel!Sobrinho!Simões1,!Elsa!Fonseca2!and!Fernanda!Trigo1!
!
(1)Clinical! Haematology! Department! of! Centro! Hospitalar! e! Universitário! de! São! João,! Porto,!
Portugal,! (2)Pathological! Anatomy! Department! of! Centro! Hospitalar! e! Universitário! de! São! João,!
Porto,!Portugal!
!
Introduction:!Common!Variable!Immunodeficiency!(CVID)!is!a!rare,!heterogeneous,!primary!
immune! deficiency! disorder! characterized! by! hypogammaglobulinemia.! It! causes!
susceptibility! to! recurrent! infections,! autoimmunity,! inflammationJinduced! organ! damage!
and!lymphoproliferative!disorders.!
Objectives:!Presentation!and!discussion!of!a!clinical!case!of!lymphoma!in!a!patient!with!CVID!
Clinical' Case:!Caucasian! women,! 40JyearsJold! who! was! diagnosed! with! CVID! in! 2012! and!
subsequently!treated!with!intravenous!immunoglobulin!replacement!(IVIG).!She!experienced!
recurrent! respiratory! tract! infections! and! history! of! fluctuating! adenopathies,! with! several!
biopsies! showing! infiltration! by! a! population! of! small! polymorphic! lymphocytes.! In! 2017!
were!evidenced!the!presence!of!structural!lung!alterations!on!chest!computed!tomography!
(CT)!scan!with!subsequent!biopsy!diagnosing!a!Granulomatous!Lymphocytic!Interstitial!Lung!
Disease.! In! 2018! she! developed! gastrointestinal! complains! with! slightly! alterations! in! liver!
function,!with!liver!and!duodenal!biopsies!revealing!nodular!lymphoid!hyperplasia.!In!2019!
was! diagnosed! with! pneumocystosis! and! also! developed! a! progressive! left! axillary!
adenopathy.! Excisional! biopsy! revealed! an! EBVJnegative! diffuse! large! BJcell! (DLBC)!
lymphoma!with!abundant!T!cells.!CT!scan!and!positron!emission!tomography!(PET)!imaging!
obtained! showed! hypermetabolic! adenopathies! distributed! throughout! the! supraclavicular!
region,!thorax,!abdomen!and!pelvis.!It!was!assumed!the!diagnosis!of!DLBC!lymphoma,!stage!
IVJA,!RJIPI!2!and!was!treated!with!6!cycles!of!RJCHOP!with!continued!monthly!administration!
of! IVIG,! antiviral! prophylaxis! and! maintenance! of! coJtrimoxazol! (for! pneumocystosis!
treatment).! At! the! end! of! treatment! CT! scan! and! PET! imaging! demonstrated! complete!
remission.!Treatment!was!very!well!tolerated,!with!no!infections!or!other!complications.!
Discussion'and'Conclusions:!CVID!patients!are!at!increased!risk!of!developing!lymphomas,!
possibly!due!to!underlying!immune!dysregulation!and!sustained!activation!and!proliferation!
of!lymphocytes!during!chronic!infections.!Biopsy!evaluation!in!patients!with!CVID!to!assess!
for!lymphoma!is!challenging!and!should!be!interpreted!with!extreme!caution!because!of!the!
altered! architecture! within! lymph! nodes! and! marked! lymphoid! infiltration! in! extra! nodal!
sites.!The!patient!experienced!involvement!by!lymphoproliferative!lesions!during!the!course!
of!the!CVID!(pulmonary,!gastrointestinal,!lymph!nodes)!with!biopsies!revealing!polymorphic!
infiltrates!of!lymphoid!cells.!Once!the!diagnosis!of!lymphoma!is!made,!there!is!a!concern!for!
the! high! risk! of! infection! due! to! excessive! immunossupression! of! CVID! combined! with!
immunochemotherapy.!In!our!case,!the!treatment!proceeded!without!complications,!as!the!
patient!maintained!IVIG!administrations!and!infectious!prophylaxis!were!fulfilled.!
! !
SCIENTIFIC PROGRAMME
SESSION I
HODGKIN’S DISEASE
DEBATE I
IS THERE STILL A ROLE
FOR COMBINED MODALITY
THERAPY FOR EARLY
STAGE CHL?
SESSION II
T-CELL LYMPHOMA
ROUNDTABLE I
FUTURE DIRECTIONS IN
T-CELL LYMPHOMA
SESSION III
FOLLICULAR LYMPHOMA
DEBATE II
CAN WE AVOID
CHEMOTHERAPY IN
THE MANAGEMENT OF
FOLLICULAR LYMPHOMA?
SESSION IV
RARE LYMPHOMAS –
MARGINAL ZONE
LYMPHOMA AND
WALDENSTRÖM M
ACROGLOBULINEMIA
ROUNDTABLE II – WHERE
TO GO IN RARE B-CELL
LYMPHOMAS
SESSION V
MANTLE CELL LYMPHOMA
SESSION VI
DIFFUSE LARGE B-CELL
LYMPHOMA
DEBATE III
DO WE STILL NEED ASCT
IN MCL?
SESSION VII
NOVEL THER APEUTIC
CONCEPTS IN B-CELL
LYMPHOMAS
SELECTED ABSTRACTS
FOR AN ORAL
PRESENTATION
SELECTED ABSTRACTS
FOR A POSTER
PRESENTATION
DISCLOSURES