66yrs old Lady with Acute Myelo Leukaemia diagnosis 1 yer before. First course of standard chemo was realised.  
FISH t;21). Progression and neuroleukaemia in suspitio.  
ASPIRATE: 49% blasts; MPO+ only 5%.  
FLOW: ~35% blasts in BM: CD34+, CD33+,CD7+ dim, CD61+ dim, CD41+ dim, CD42-, CD13-, HLA-DR-, CD117-, cMPO-, CD14-, CD64-, CD56-, CD11c-, CD7-, CD2-, CD1a-, CD4-, CD8-, CD22-, CD19-, CD20-, CD10-, CD15-, cTdT-, cCD3-, cCD79a-. AML M7 POSSIBLE.  
BLOOD: WBC (*10e9/l) 30,8; HgB (g/l) 99; Plt (x10e9/l) 422.  
HISTO: in the virtual slides and snapshots. Pleomorphic blasts with multilobated nuclei vWF+ closed to more conventional blasts.  
IH: CD117+/-; CD34+; vWF+; Pax-5+ (possibly asociated with t(8;21); CD7+; TdT/CD68/CD4/CD56/MPO(-); CD2/CD5(-).  
 
PROPOSAL: Acute myeloleukaemia, 85% BM. Fe deposition.  
QUESTION: 1. The possibility of subclassification (M7 like: vWF+; CD61- partially? or additional genetic changes in suspition?). 2. Usually t(8;21) asoc with good prognosis and Pax5+ (it does not take place there).

SergeyN 2009-10-19 09:12	A very unusual case, really. Did she have t(8;21) at diagnosis?     Just a small comment on blast evaluation: there could actually be more megakaryoblasts, for megakaryocyte loses CD34 and acquires vWF while maturing. Do you stain for CD61? It may be interesting to compare.   Some blasts could be of red origin, too.
SergeyN 2009-10-19 09:20	There is another question that doesn't quite pertain to this specific case.     Sometimes, there are discrepancies between flow and IHC that are difficult to explain by purely technical issues, like CD117 negativity here. Sometimes it even causes diagnostic problems.     Could experts comment on that and on correct way of dealing with the discrepancy?
ugnius 2009-10-19 10:09	Thanx for comment. CD61 (IH) negative. Hb negative too (for sure it's not a best erythroid marker).
hurwitz 2009-10-20 18:13	I like Sergey's diagnosis: this is an unusual case. Looking at the morphology, and the results of immunophenotyping there seems to be a mixed population consisting of myeloblasts with evidence of myeloid maturation and expression of lymphoid markers (PAX-5) consistent with AML with (8;21) and a second population showing morphologic and immunophenotypic evidence of megakaryocytic differentiation.   Is there any possibilty to see the material taken at diagnosis? Another question is which kind of treatment did the patient receive?
erber 2009-10-20 20:38	Based on the bone marrow histology, immunocytochemistry and flow cytometry data available, I favour "acute myeloid leukaemia". I find it difficult on the information available to determine whether this is "pure" myeloblastic leukaemia, megakaryoblastic or mixed myeloid / megakaryoblastic. At this stage it makes no difference to the current management as this is now relapse after 1 year, and with leukaemic meningitis.
ugnius 2009-10-21 13:18	Thanx. PRIMARY slides will be transfered soon. Therapy first round: daunorubicin and citosar. 2nd round: HAM protocol(citosar and mitoxantron).
diane.c.farhi 2009-10-21 22:34	I would call this acute megakaryoblastic leukemia, since this is clearly the cell line most affected. I think it's beautifully illustrated. It may be clonally related to the original AML or an example of therapy-related AML occurring within a short time-frame, a phenomenon that has been reported. It is true that IHC and flow results do not always correlate, and this is also true of morphology and flow. I don't think there is any official consensus on this. My feeling is that morphology is better than flow for enumerating blasts, as promyelocytes and myelocytes may show up on flow as CD34+ but would not be counted in a WBC differential as blasts. Regarding flow vs IHC, flow is definitely more sensitive and I would trust flow results over IHC in enumerating the percentage of positive cells in a population.
hurwitz 2009-10-28 15:39	There is no consensus in this case, which is more or less normal in such unusual cases.   I like Dr.Erber's pragmatic approach in calling it simply AML, however, megakaryocytic differentiation, as evidenced by flow cytometry and immunocytochemistry, as well as t(8;21) with aberrant PAX5 expression cannot be overlooked.   Therefore my proposed diagnosis:   Acute myeloid leukemia, with t(8;21)and evidence of megakaryocytic differentiation.
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