Lab Med Online.  2017 Jul;7(3):103-110. 10.3343/lmo.2017.7.3.103.

Frequency and Distinct Characteristics of Acute Myeloid Leukemia Lacking HLA-DR and CD34 Expression: Features Intermediate between Typical Acute Myeloid Leukemia and Acute Promyelocytic Leukemia

• Lee HY
• Cho YU
• You E
• Jang S
• Seo EJ
• Park CJ

• KMID: 2383088
• DOI: http://doi.org/10.3343/lmo.2017.7.3.103

Abstract

BACKGROUND
The objective of this study was to investigate the frequency and characteristics of HLA-DR⁻/CD34⁻ acute myeloid leukemia (AML) also known as acute promyelocytic leukemia (APL)-like AML.
METHODS
This study included 683 newly diagnosed patients with AML. After exclusion of 211 patients with recurrent genetic abnormalities, one with acute panmyelosis with myelofibrosis, two with myeloid leukemia associated with Down syndrome, and two devoid of metaphase cells, we classified the remaining 467 patients as follows: group 1, HLA-DR⁺/CD34⁺ (typical AML); group 2, HLA-DR⁺/CD34⁻ or HLA-DR⁻/CD34⁺; group 3, APL-like AML.
RESULTS
Group 1 comprised 294 patients, group 2 comprised 133, and group 3 comprised 40. Therefore, the frequency of APL-like AML among 683 unselected patients with AML was 5.9%. Group 3 patients had significantly higher leukocyte counts and bone marrow (BM) blast percentages, higher frequencies of normal karyotypes and NPM1 mutation, higher fractions of CD33-positive cells, higher concentrations of fibrin degradation products and D-dimers, lower frequencies of complex karyotypes, monosomal karyotypes and poor cytogenetic risk, lower fractions of CD13-positive cells, and lower fibrinogen concentrations, compared with group 1 patients. The values of the BM blast percentage, number of CD33-positive cells, and DIC score of the patients with APL-like AML were intermediate between those of the patients with typical AML and APL.
CONCLUSIONS
This study demonstrates that APL-like AML is not uncommon, and it has characteristics distinguishable from those of typical AML. APL-like AML may have some pathophysiological relationships with APL, which need further investigation.

Keyword

promyelocytic leukemia; Acute myeloid leukemia; HLA-DR; CD34; Immunophenotype; DIC; NPM1 mutation

MeSH Terms

Bone Marrow
Cytogenetics
Dacarbazine
Down Syndrome
Fibrin Fibrinogen Degradation Products
Fibrinogen
HLA-DR Antigens*
Humans
Karyotype
Leukemia, Myeloid
Leukemia, Myeloid, Acute*
Leukemia, Promyelocytic, Acute*
Leukocyte Count
Metaphase
Primary Myelofibrosis
Dacarbazine
Fibrin Fibrinogen Degradation Products
Fibrinogen
HLA-DR Antigens

Figure

• 

  Fig. 1. The distribution and frequency of cases with high bone marrow blasts, CD33-positive cells, and DIC score. Patients with APL-like AML (group 3) showed an intermediate feature between HLA-DR+/CD34+ (group 1) and true APL cases. The cutoff values for bone marrow blasts (76.0%) and CD33-positive cells (91.4%) were derived from values corresponding to the 75 percentile of patients with group 1. The cutoff value for DIC score (5) was based on the ISTH consensus proposal. The frequencies of BM blast ≥76.0% were 25.2% in group 1, 22.6% in group 2, 45.0% in group 3, and 69.1% in APL. The frequencies of CD33-positive cell ≥91.4% were 25.2% in group 1, 52.6% in group 2, 70.0% in group 3, and 96.3% in APL. The frequencies of ISTH DIC score ≥5 were 12.3% in group 1, 23.6% in group 2, 51.5% in group 3, and 70.4% in APL. All of these three variables (BM blast, CD33-positive cell, DIC score) were significant (each, P<0.001) by Chi-square test for trends.

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