53 yrs male with threpine biopsy. For 2 yrs skin eruptions and cronic cough were present.  
 
CLINICS:  
The blood count tested last friday shows a slight anemia (Hg - 12,6 g/dL), WBC and PLT normal (WBC - 6,65x10*9/L, PLT - 182x10*9/L). There is a slight increase of eosinophils 0,7 x10*9/L. Mast cell in a blood 0,9%.  
tHE Bilirubin is slightly increased at 21 mkmol/l (<21), direct bilirubin - 5,3 mkmol/L (<6,5), indirect - 15,7 (<15,3). ALT (13 U/L) and AST (17 U/L) are normal. gammaGT - 50 U/L (< 36 U/L). Albumin is 46g/L.  
No C findings.  
Skin biopsy - normal, no mast cells. Erythema.  
Serum triptase > 200.  
The whole body CT shows no pathologic mass or fractures. Bone density performed by DEXa - normal.  
The patient has with allergy associated symptoms as caught and skin erythema.  
 
HISTO:  
a) Cellularity 90%, subtotal difusse epithelioid medium sized (Giemsa+) eosinophilic and pale mastoytic infiltration with reniform nuclei.  
Multiple atypical mastocytes in smear.  
b) B lymphocytic aggregates and follicles.  
 
IH:  
MAST CELLS: CD117(+++)(membr.) 100% (~60% population); CD25(+/++)(membr.) 80%; CD2(+/++)(membr.) 90%; CD68(+/-)(dim. perinuclear) 30%; Mast cell tryptase (+/-) 90% (dim. citopl.); CD30(-); Ki67 index <5% (+++); CD43(++) 100% (membr.).  
lYMHOID: CD20 (++)(citopl.) >>> CD2+; CD5(+++)(citopl.) 100%; CD23(+/++)(citopl.) 25%; IgM(++) 100% (citopl.); p27(++/+++) 100% (nuclear); IgG(-); IgD(+/++) 30% (citopl.); MNDA1(-); CD3(-); CD43(?);  
 
FLOW:  
Peripheral blood: T lymphos (CD3+) ~59%, Th:Tc=1,6; NK cells (CD3- CD56+) ~9%; ~26% B-cells: CD45+, CD19+, CD5+, CD20+ strong, CD23+het, CD22+ dim, CD79b+, CD43+dim, FMC7+dim, CD38-, HLA-DR+, kappa+, lambda-, CD11c-, CD10-, sIgM-.  
restriction Ig kappa:lambda = 9:1. Imunophenotype compatible with B-CLL/SLL.  
~0,03% aberrant mastocytic population found: CD45+dim, CD117+strong, CD33+, CD2+, CD25+dim, CD34-.  
 
MOLECS: C kit exon 17 D816V mutation in bone marrow is negative. (repeated twice).  
FIP1L1/PDGFRA del4q12 is negative. TruSight myeloid sequencing did not show any mutation.  
 
PROPOSAL: a) Systemic aggressive mastocytosis/ aleukemic mast cell leukemia: CD117+; CD2/CD25+ > CD68+; CD43+; CD30(-); Ki67 prolif. activity low <5%.  
b) INCIDENTAL B SLL/CLL (or clonal lymphocytosis) in BM: p27+; CD5+ CD20+ > CD23+; IgM+ > IgD+; MNDA1/CyclinD1/CD43(-).  
 
ThankYouBeeingCloseForYears.