Description

About a quarter of patients with acute promyelocytic leukemia (APML, M3) have cells that lack the large cytoplasmic granules seen in promyelocytes. This type may be misidentified unless flow cytometry and cytogenetics is performed.


 

Presentation:

(1) high white blood cell count (may be > 100,000 per µL); this may be high enough to result in leukostasis

(2) bleeding disorder with thrombocytopenia, prolonged PT and prolonged PTT

(3) A microangiopathic hemolytic anemia may be present.

 

Features of leukemic cells:

(1) Blasts in the peripheral blood have no, few or very small cytoplasmic granules.

(2) Auer rods are usually rare or absent but often can be found; "faggot" cells are possible.

(3) The nucleus may be folded, bilobed or irregular.

(4) The cells may be misdiagnosed as another form of leukemia (monocytic, T cell, etc).

(5) Blasts in the bone marrow may show the granules of typical M3.

 

Cytochemical pattern: myeloperoxidase and Sudan Black B positive; often chloroacetate esterase positive; occasionally alpha-naphthyl butyrate esterase positive

 

Immunoperoxidase pattern:

positive: CD33, CD13

negative: HLA-DR, CD34, CD14, CD11b, CD41

variable: T-cell associated surface antigen

 

Cytogenetic finding: usually t(15;17); less often t(11;17) or t(5;17)

 

PCR: presence of PML-retinoic acid receptor alpha (RAR-alpha) fusion gene product

 


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