71 yrs old male with hepatosplenomegaly, serous effusions in pleural and abdominal cavities hospitalized due to fatigue, physical activity intoleration, short breath. Anemia nad thrombocytopenia were found.  
BLOOD: WBC (*10e9/l) - 4,72, NEU (%) - 6,0, LYM (%) - 25,0, MON (%) - 63,6, EOS (%) - 2,5, BAS (%) - 0,6, IG (%) - 2,3, NRBC (%) - 1,8, NEU (*10e9/l) - 0,28, LYM (*10e9/l) - 1,18, MON (*10e9/l) - 3,00, EOS (*10e9/l) - 0,12, BAS (*10e9/l) - 0,03, IG (*10e9/l) - 0,11, RBC (*10e12/l) - 3,49, HgB (g/l) - 107, Hct (l/l) - 0,310, MCV (fl) - 88,8, MCH (pg) - 30,7, MCHC (g/l) - 345, RDW-SD (fl) - 51,2, P-LCR (%) - 24,6, Plt (x10e9/l) - 72.  
FLOW: CD45+, CD19+, CD22+, CD20+bl, CD79b+, HLA-DR+, CD38+, CD43+bl, CD10+bl, CD5+, CD23-, FMC7-, sIgM-. Changes are compatible with diagnosis of low grade B LPD: B mantle cell lymphoma (FMC7-,sIgM-)? CD5+ DLBCL?.  
BM: 90% infiltration of medium sized lymphos with ovoid monotonous vesiculated nuclei and hyperprominent eosinophilic nucleoli ("prolymphocytes"). Focal reticulin fibrosis MF-1.  
Molecs: JAK2(-); BRAF(-);  
IH: CD20+ (strong); CyclinD1/HCL (DBA.44)(-)(aberant?); Annexin (-/+)(aberant?); CD23(-); CD25+; IgM+; CD123(-); IgD(+/-); CD5+ (aberant?); Ki67 low 10%.  
 
Proposal: Low grade (leukaemic) B NHL.  
Dx: 1. HCL: cytopenia + splenomegaly fit, but BRAF(-); cytology "pro"; DBA44(-); CD5+; CyclinD1(-).  
2. HCLv: cytology "pro" fit, but CD5+ (aberant?);  
3. Mantle cell lympoma ("prolymphocytic"): CyclnD1(-). I cannot arrange CCND1 FISH from BM.  
4. Primary splenic MZL: not compatible IH.  
5. Primary splenic unclass NHL: not compatible IH.  
6. B prolymphocytic leukamia.  
 
Clinically a case was interpreted as B marginal zone lymphoma and treated with R-CVP.  
 
Thank you for your kind look.  

tzankov 2013-12-16 11:56

Classificatory difficulat case of a CD5+ B-cell lymphoma with lots of (para)immunloblasts. From the cytology and the negativity for CCND1, I would not favor MCL. It doese not look like HCL. To diagnose B-PLL you need the morphology of the peripheral lymphocytes, but this could be easily checked for. For the moment I would favor calling it B-cell lymphoma, unclassifiable. Unfortunately I con not open the Ki67 file.

ugnius 2013-12-28 13:10

Thank you very much.