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MPN/MDS in transformation (958871) » Eosinophilic BM HE.jpg
Filename: Eosinophilic BM HE.jpg
[MPN/MDS in transformation]
CASE IS OPEN (40x added).  
 
65 yrs female with threpine biopsy.  
CLINICS: general weakness, nausea and night sweats. from 2017 Nov. Due to leukocytosis and thrombocytopenia was sent to hemato-consult.  
 
HISTO (PHOTOS): Threpine with 100% cellularity, diffuse architecture, predominant NASDE+ granulopoiesis with left shift and dispersed normal and megalo- erythroids. Moderate large megas with lobated and hypolobated nuclei up to 8 HPF. Prominent immature granulopoiesis with various vesiculated and reniform nuclei, eosinophilic maturing cytoplasm, single eosinophilic macronucleoli (as blast equivalents not detected by IH/CD34(-)(?).  
CD68+ phagocytosis/haemohagocytosis prominent. CD68+ monocytoid/blastoid elements present and clustered.  
 
IH: "Blastoid/immature population": CD68(+/++)(cytopl.)25%; Bcl2(-); MPO (+++)(cytopl) 100%; Pax5(-); CD123(-); CD117(+)(cytopl) 10%; CD163(-); GlycophorinC(-); vWF(-); CD11c(-); CD2(-);  
CD34/HLA DR+ clustering population up to <5%; TdT+ hematogones singles <3%.  
POSSIBLY blastic population might be CD34(-)???  
 
BLOOD:  
WBC (*10e9/l) 48,62(Normal value 4,0-9,8)  
NEU (*10e9/l) 44,20 (Normal value 1,5-6,0)  
MON (%) 1,2 ( Normal value 2-10)  
RBC (*10e12/l) 2,70 (Normal female value 4,1-5,1)  
HgB (g/l) 76 (Normal female value 117-145)  
Plt (x10e9/l) 50 (Normal value 140-450)  
 
ASPIRATE FLOW:  
Immature monocytes (CD45+bl, HLA-DR+, CD38+, CD33+ high, CD64+, CD13-/+ weak, IREM-2 -, CD14-, CD36-, CD34-, CD117-) ~0,4 %. Mature monocytes (CD45+bl, HLA-DR+, CD38+, CD33+ryš, CD64+, CD13+, IREM-2+, CD14+, CD36+, CD34-, CD117-)~0,5%  
~7 % mieloblasts: CD45+weak, CD117+het, CD34+, CD13+weak, CD33+het, CD38+weak, CD133+bl, HLA-DR+, CD11a+weak, cMPO-/+het, CD123-/+het, CD99-/+weak, CD7-, CD15-, CD96-, CD11b-, CD64-, CD14-, CD36-, cTdt-, cCD3-, cCD79a-. Abberations: weaker CD13+, heterogenenous CD33+,  
 
MOLECS:  
BCR/ABL1 t(9;22)(q34;q11) NEGATIVE  
SNP: del 1p (standard CYTOGENETIC RISK)  
JAK2 V617F and Ex12 NEGATIVE  
PML/RARA t(15;17)(q22;q21)NEGATIVE  
AML1/ETO t(8;21)(q22;q22) NEGATIVE  
CBFB/MYH11 inv(16)(p13;q22) NEGATIVE  
DEK/NUP214 t(6;9)(p23;q34) NEGATIVE  
NPM1 NEGATIVE  
FLT3 ITD NEGATIVE  
 
SRSF2 mutation Pro95-Arg102del  
CSF3R ex14 mutation T618I. Ex17 mutation: NEGATIVE.  
 
PROPOSAL:  
a) MPN/MDS NOS (SRSF2 ir CSF3R Ex14 mutated; del 1p) with blast excess (7% flow; 12% threpine imprint)/in possible transformation: DIFFERENTIALS/"OVERLAP" BETWEEN CMML (not prominent monocytosis) and CNL (contrary: dyserythropoiesis and dysgranulopoiesis) and aCML (mutations more specific for CNL and CMML).  
High risk of transformation into AML.  
b) Hemophagocytosis (histologically in BM): systemic disease HLH? (NOT FOCCUSSED THERE)  
 
PROBLEM: CD34+ POPULATION NOT EXCEEDS 10% (MDS like), BUT REAL BLAST POPULATION VISUALLY SEEMS TO BE MUCH HIGHER... (probably mono/CD68+). Unfortunatelly Bcl2 not helps.  
 
P.S. Follow up after diagnosis (clinically cured as MPN/MDS NOS EB2): Rapid deterioriation, blasts in peripheral blood appear (transformation). Exitus.  
 
ThankYouBeeingClose.  
 
 
Sender: ugnius
2018-04-07 17:23
Haematopathology Forum

Last modified: 2018-04-07 17:23:15