POSTER'8'
HEMOPHAGOCYTIC'LYMPHOHISTIOCYTOSIS'AS'A'PRESENTATION'OF'
INTRAVASCULAR'LYMPHOMA'
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Dino!Andrade!Luís,!Telma!Nascimento,!André!Barbosa!Ribeiro,!José!Pedro!Carda!and!Marília!Gomes!
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Clinical!Haematology!Department,!Centro!Hospitalar!e!Universitário!de!Coimbra,!Coimbra,!Portugal!
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Intravascular! Large! BJCell! Lymphoma! B! (ILBJCL)! is! a! rare! and! aggressive! extranodal!
lymphoma,! characterized! by! a! selective! growth! of! lymphoma! cells! in! the! lumen! of! blood!
vessels.! The! form! associated! with! the! macrophage! activation! syndromeJhemophagocytic!
lymphohistiocytosis!(MASJHLH),!named!Asian!variant,!is!normally!associated!with!multiorgan!
dysfunction,!hepatosplenomegaly!and!pancytopenia,!with!an!even!more!adverse!prognosis.!
Clinical'Case:48JyearJold!woman,!admitted!due!to!night!sweats,!fever!and!significant!weight!
loss,! with! 1week! duration.! In! the! physical! exam! was! detected! a! massive! hepatoJ
splenomegaly.! Analytically,! she! presented! pancytopenia,! elevation! of! cytocholestasis!
parameters! and! LDH>4LSN,! hyperferritinemia! and! hypertriglyceridemia;! a! bone! marrow!
aspirate!was!performed,!which!revealed!frequent!figures!of!hemophagocytosis,!allowing!the!
diagnosis!of!HLH!to!be!confirmed,!according!to!HLHJ2004/HJscore!criteria.!PET/CT!show!only!
massive!hepatosplenomegaly!with!diffusely!increased!uptake!of!FDGJF18,!suggesting!diffuse!
hepatosplenic! lymphomatous! involvement.The! liver! biopsy! showed! “diffuse! and! intra!
sinusoidal! involvement! by! cells! with! large! size;! immunohistochemistry! showed! strong!
positivity! for! CD20,bcl2,bcl6,MUM1,CD5andCD15! and! unmarked! for! CD3,cyclinD1!
andSOX11”;!the!molecular!study!did!not!reveal!cJMyc!rearrangement.!Thus,!the!diagnosis!of!
ILBJCL,! Asian! subtype,! stageIVJBS,RJIPI2,CNSJIPI2,! was! established.!During! hospitalization,!
there!was!a!significant!worsening!of!general!condition,!with!multiorgan!dysfunction,!namely!
liver! (ChildJPugh! C)! and! heart! (Ejection! Fraction! 43%)! failure,! encephalopathy! (grade! 3J4)!
and! severe! coagulopathy,! and! need! of! aminergic! support.! She! was! proposed! for!
chemotherapy! according! to! the! RJCHOEPprotocol,! since! this! protocol! includes! etoposide,!
which! could! be! beneficial! in! the! treatment! of! HLH.! However,! due! to! cardiac! and! hepatic!
dysfunction,! she! was! treated! in! first! cycle,! only! Rituximab,! 50%! of! the! dose! of!
cyclophosphamide!and!prednisolone.!In!D7!of!the!1stcycle!there!a!significant!improvement!in!
the! general! condition! was! observed,! with! a! 50%! reduction! in! the! parameters! of!
cyto/cholestasis!and!coagulation!times,!with!descending!bilirubin!profile!and!normalization!
of!albumin,!prothrombin!time,!factor!V!and!ammonia.!In!D21!it!was!possible!to!treat!with!full!
doseRJCHOP.! The! patient! completed! 6! cycles! ofRJCHOP.! In! the! final! PET/CT! she! met! the!
criteria!of!complete!remission!according!to!the!Lugano!criteria,!maintaining!no!evidence!of!
recurrence!6!months!after!the!end!of!treatment.!
Conclusion:The!ILBJCL!is!a!highly!aggressive!lymphoid!neoplasm!with!low!response!rates!to!
conventional! chemotherapy,! especially! Asian! variant.!With! the! present! clinical! case,! the!
authors!intend!to!describe!the!diagnostic!challenge!that!constitutes!the!presentation!of!this!
lymphoma! in! the! form! of! hemophagocytic! lymphohistiocytosis,! but! also! the! difficulty! in!
choosing!the!most!appropriate!therapy!in!face!of!severe!multiorgan!dysfunction.!
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