Here it goes:
A boy born 1982, SLE since 3 years, treated with Imurel and steroids, but
not recently, was admitted with Hb 40 g/l, WBC 121 x 10e9/l, Plt 125x10e9/l,
lab. hemolysis. Diff. count showed that at least 50% of nucleated PB cells
were nucleated erythrocytes, but there were also 15% "blasts". Bone marrow
aspirate showed left shifted erythropoesis (like in haemolysis),
differentiating granulopoesis and 21% blast-like cells with lymphoid
cytology but not exactly LGL4s.
Flowcytometry of the bone marrow showed that 20% of bone marrow cells were:
CD2+, CD11b+, membr. and cyt.CD3-, CD5-, CD7neg/dim, CD4-, CD8-, CD19-,
CD20-, CD22-, CD10-, CD34-, CD117-, CD13-, CD65-, CD15-, CD14-, Tdt-, MPO-.
A subpopulation of these cells was CD56+ (5% of BM cells totally) and
another subpopulation (10% of BM cells) was CD33+. There seem also to be a
triple positive CD2+/CD56+/CD33+ population present (5% of BM cells totally).
These cells are on FSC/SSC plot located in the NK cell/small blast region.
The patient has lung infiltrates that are considered to be SLE related,
splenomegaly but no palpable peripheral lymph nodes.
Cytogenetics is not ready yet.
Has anybody seen such population? Is it an NK cell reaction or emerging AML
or lymphoma?
All comments / suggestions will be greatly appreciated.
Anna Porwit-MacDonald MD, PHD
Chief, Haematopathology Lab.,
Karolinska Hospital, Stockholm, Sweden
anpo@mb.ks.se