38 yrs old male with resected CNS tumor (headaches and CT diagnostics). EXTERNAL material (IH quality suboptimal).  
HISTO: on the slides: dense, predominatelly T small-medium lymhoid angiocentric infiltrate with ovoid and irregular/atypical nuclei. Perifocal necrosis with histiocytic reaction and haemophagocytosis signs. Plasmacytic admixture.  
IH: CD3(+/+++)100%; CD20(-); CD2(+++)100%; CD5(++)80%; CD7 (-)(?)(about 30% of population +: reactive?); CD4(-); CD8(+++)100%; Granzym B(++/+++)100%; TIA1(+++)(100%; CD30(-); CD56(-); CD57(+/+++)70%; Toxoplasma(-); EBER(-); PanCK(-); CD68(-)(Histiocytes +); Ki67(+++)iki 70%; CMV/polyoma (SV40)(-); CD23/CD21(-)(FDC absent).  
CD138+ clusters: Ig kappa/lambda+ (politypic).  
 
MOLECS: CLONAL TCRB V-D-J1/2, D-J1/2 ir TCRG V-J .  
IGH ir IGK lokusuose NON CLONAL.  
 
PROPOSAL: Atypical EBER(-) T lymhoid cytotoxic lymphoproliferation of CNS with abberant immunophenotype: CD3/CD2/CD8/CD57+ > CD5+; CD7/CD4(-); CD56/CD30(-); TIA1/Granzym B+; EBER(-); Ki67 high up to 70%. Hemophagocytosis. Prominent arteriosclerosis/atheromatosis.  
Clinical correlation is essential: viral infections? LDH? leukemic? extranodal/nodal lesions? due to possible T LPD: T large granular lymphocytic leukaemia?

tzankov 2013-10-15 11:46

The morphology (angocentricity, destructive process, cytologic atypia of the lymphocytes), the phenotype with possible Cd7 loss and the clonality allow the diagnosis of T-cell neoplasm.     The exact classification seems more difficult. For principal issues ALK1 must be stained. Does the patient have a clinical hemophagocytic syndrome? Are there LGL in the peripheral blood? How is the skin and penis status? Is the spleen enlarged? Is there ahistory of any disorer incl. sprue?

ugnius 2013-10-15 12:12

Thnx. The patient from outside just to be hospitalized for hemato evaluation. So I cannot add smth else to description and for me too its requires some clarity. I'll be back with details.