POSTER'27'
INTRAVASCULAR'LARGE'B'CELL'LYMPHOMA'PRESENTED'AS'
HEMOPHAGOCYTIC'LYMPHOHISTIOCYTOSIS'
'
Victoria!Irigoin,!Ana!Carolina!Oliver!and!Gabriela!De!Galvez!
!
CASMU,!Montevideo,!Uruguay!
!
61! yearJold! male! with! no! comorbidities.! Daily! fever! over! a! month,! no! evidence! of! an!
infectious! origin.! Cultures! and! viral! serological! tests! and! specific! microorganis! search! were!
negative,! PET/TC! scan! was! normal.! Fever! persisted! despite! wide! spectrum! antibiotics!
empirically! administered! with! development! of! multiorgan! disfunction! syndrome;! liver! and!
renal! disfunction,! coagulation! test! alteration! and! cytopenias.! Ferritin! level! was! >! 10.000!
ng/ml,! hypertrigliceridemia! and! high! LDH! were! observed! which! led! to! the! suspicion! of!
hemophagocytic!lymphohistiocytosis.!Bone!marrow!aspirate!was!inconclusive,!however,!ILJ2!
soluble! receptor! (sILJ2r)! was! 17.000! U/ml.! Due! to! the! severity! of! the! clinical! situation!
empirical! treatment! with! HLHJ94! protocol! was! started.! Liver! and! renal! dysfunction!
corrected,!cytopenias!improved,!coagulation!tests!normalized,!fever!disappeared!and!ferritin!
levels! decreased.! sILJ2r! after! treatment! was! started! was! 1350! U/ml.! After! 3! weeks! on!
treatment,!fever,!anemia!and!increase!in!ferritin!levels!reappeared!and!bone!marrow!biopsy!
showed!infiltration!by!Intravascular!Large!B!cell!Lymphoma.!He!received!RJCHOP!x!6!cycles!
with! normalization! of! laboratory! parameters! and! intrathecal! methotrexate! as! SNC!
prophylaxis!achieving!complete!remission!and!will!be!consolidated!with!autologous!stem!cell!
transplantation.!
Discussion:! Intravascular! large! BJcell! lymphoma! (IVLBCL)! is! a! rare,! aggressive! lymphoma!
characterized!by!an!almost!exclusive!growth!of!large!cells!within!the!lumen!of!all!sized!blood!
vessels.! Median! age! at! diagnosis! is! 70! years! (34J90)! without! sex! prevalence.! The! most!
common!symptom!is!fever!of!unknown!origin,!and!a!rapid!deterioration!of!general!condition!
is! very! frequent.! Three! main! variants! are! described:! classical,! cutaneous! and!
Hemophagocytic!syndrome–associated.!The!last!one!shows!an!acute!aggressive!onset!with!
median! survival! of! 2J8! months! in! the! pre! Rituximab! era.! Treatment! of! choice! is!
chemoimmunotherapy!as!in!DLBCL.!The!addition!of!Rituximab!has!significantly!changed!the!
evolution!of!IVLBCL.!In!western!world!RJCHOP!leads!to!CR!in!88%!and!3Jyear!OS!of!81%.!In!
Japan,!2Jyear!progressionJfree!survival!and!OS!is!56%!and!66%!respectively.!CNS!is!the!most!
common! site! of! relapse! therefore,! prophylaxis! is! recommended.! The! role! of! Autologous!
Stem! Cell! Transplantation! as! consolidation! is! not! well! established,! but! it! is! an! option! in! fit!
patients.!
Summary:!this!is!a!very!rare!and!aggressive!entity!where!a!high!grade!of!suspicion!is!needed!
and! highlights! the! importance! or! performing! a! bone! marrow! biopsy! in! cases! of! fever! of!
unknown!origin.!The!association!with!hemophagocytic!syndrome!is!associated!with!a!poorer!
prognostic!than!the!classical!variant.!