LYMPHOMATOID'GRANULOMATOSIS'MIMICKING'PSEUDOSARCOIDOTIC'
POSTER'36'
GRANULOMATOSIS':'A'CASE'REPORT'
'
Aznag!Mohamed!Amine!and!Raissi!Abderrahim!
!
Avicenna!Military!Hospital,!Marrakesh,!Morocco!
!
Introduction!
Described! in! 1972! by! Liebow,! lymphomatoid! granulomatosis! is! a! rare! EpsteinJBarr! virus!
(EBV)Jassociated!lymphoproliferative!disorder,!involving!the!lung,!and!often!the!skin!or!the!
central!nervous!system.!It!could!have!a!systemic!course!making!its!diagnosis!challenging.!The!
severity!varies!from!indolent!forms!to!aggressive!transformation!to!large!B!cell!lymphoma.!
Here! we! report! the! case! of! a! patient! with! lymphomatoid! granulomatosis! mimicking!
pseudosarcoidotic!granulomatosis.!
Case'report':!
A! 77Jyear! old! male,! with! no! past! medical! history,! was! admitted! to! internal! medicine!
department!for!2!months!history!of!whole!body!spread!dermoJepidermal!nodules!associated!
with!dyspnea,!productive!cough,!decreased!visual!acuity,!dry!mouth!and!weight!loss!(8!kilos!
in!2!months).!
Physical! examination! showed! hard! discoid! erythematous! lesions! with! centimetric! cervical!
and!axillary!lymphadenopathy.!
Complete!blood!count!was!normal!except!lymphopenia!(0.69!Giga/L).!Phosphocalcic,!renal,!
hepatic! and! thyroid! functions! were! unremarkable.! ESR! was! accelerated! (77! mm! at! the!
1st!hour)! and! CRP! was! moderately! elevated! (26! mg/L).! Phthysiological! assessment,!
immunological!workup,!viral!serologies!and!QuantiFERON®!came!back!negative.!Angiotensin!
converting!enzyme!was!normal.!Chest!xJray!showed!multiple!small!diffuse!alveoloJinterstitial!
and!peribronchial!foci.!Whole!body!CTJscan!showed!lobar!alveolar!condensation!associated!
with! supraJ! and! infraJdiaphragmatic! lymphadenopathy.! Bronchoalveolar! lavage! favors!
lymphocytic! alveolitis.! The! salivary! gland! biopsy! was! negative.! Skin! biopsy! showed!
inflammatory!epithelioid!granulomatosis.!
The! patient! initiated! corticosteroid! therapy! (1mg/kg! of! prednisone)! with! antibiotics.! The!
evolution!after!one!week!of!treatment!was!spectacular,!with!disappearance!of!axillary!and!
cervical!lymphadenopathy!and!regression!of!skin!lesions.!
After! 02! months! of! treatment,! while! tapering! corticosteroids,! the! patient! presented! with!
new! onset! of! fever,! dyspnea,! vesicular! rash! and! reappearance! of! cervical,! axillary! and!
inguinal! lymphadenopathy! associated! with! splenomegaly.! ThoracoJabdominoJpelvic! CT!
revealed!mediastinal,!hilar!and!axillary!lymphadenopathy!with!reticular!pulmonary!lesions.!
Anatomopathological! study! of! a! cervical! lymph! node! biopsy! concluded! on! lymphomatoid!
granulomatosis.!
The! evolution! was! rapidly! unfavorable,! with! occurrence! of! hemophagocytic!
lymphohistiocytosis!and!rapid!degradation!of!the!patient,!who!died!following!a!multiJvisceral!
failure.!
Conclusion'!
Lymphomatoid! Granulomatosis! is! a! rare! lymphoproliferative! disease.! Despite! optimal!
management,! mortality! remains! high.! Clinicians! should! be! aware! of! this! entity,! especially!
when! skin! lesions! are! associated! with! pulmonary! manifestations! and! deterioration! of! the!
general!condition.!
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