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)
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.