72 yrs old male in 2019 April addmited with abdominal, left/splenic pain and lymphocytisis in PB. Hepatosplenomegaly, abdominal lymphadenopathy in sonoscopy reveled.
CT: Active neck, mediastinal, abdominal, inguinal ltmphadenopathy and splenic involvement (Lugano III).
THREPINE: MCL, conventional variant 65% (nodular central ir peritrabecular and diffuse pattern)
IH homogenously: CD20+; IgM+ > IgD+; CyclinD1+; CD5+; CD23/CD21(-); Sox11(-); Ki67 index 5%.
FLOW PB and ASPIRATE: MCL.
BIOCHEMISTRY/SEROLOGY: ImunofiX IgM kappa monoclone.
LDH (U/L) 280 (125-243)
B2 miCroglobulin(mg/L) 12,13 (0,97-2,64)
Ig G (g/L) 33,23 (7,0-16,0)
Ig M (g/L) 4,18 (Male 0,40-2,40)
Ig kappa FREE CHAINS(BS) (mg/L) 158,08 (3,30-19,40)
Ig lambda FREE CHAINS (BS) (mg/L) 133,52 (5,71-26,30)
LN core biopsy (right inguinal):
- B MCL with EXTREMALLY plasmoid differentiation(diffuse, nodular and mantle zone pattern).
Imunofenotype 1 (MAIN MASS): CD20+; IgM+ >> IgG+ (only plasma component) > IgD+; Ig kappa+ (plasma component+); CD138(-)(plasma component+); CD5+; MNDA1+; CyclinD1+; CD23/CD21/Bcl6(-); Sox11(-); Ki67 index low 10%.
- Immunophenotype 2: Minimal Sox11+ CD20+ "MCL in situ" rings in nodules.
- Immunophenotype 3: plasma conponent (loosing some B Ags, aquiring plasma Ags).
FISH: CCND1 t(11q13.3) break in BOTH components (MLIS not visible).
MYD88 L265P mutation absent.
CLONALITY: clonal IGH FR1-3. No different othe clones present (IGH, IGK, IGL, TCRB, TCRG ir TCRD).