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A variant acute promyelocytic leukemia with t(11;17) (q23;q12); ZBTB16-RARA showing typical morphology of classical acute promyelocytic leukemia.

Han SB, Lim J, Kim Y, Kim HJ, Han K - Korean J Hematol (2010)

Bottom Line: The leukemic cells expressed CD13, CD33, CD117, human leukocyte antigen (HLA)-DR, and cytoplasmic-MPO on the immunophenotyping study.The diagnosis was confirmed by cytogenetic and molecular studies.To distinguish variant APL cases from classical APL cases, regardless of whether morphologically the findings are consistent with those of classical APL, combining morphologic, immunophenotypic, cytogenetic, and molecular studies before chemotherapy is very important.

View Article: PubMed Central - PubMed

Affiliation: Department of Laboratory Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.

ABSTRACT
A subgroup of acute leukemia with morphology resembling acute promyelocytic leukemia (APL) shows variant translocations involving RARA and has a different morphology from that of classical APL. The variant APL with t(11;17)(q23;q12); ZBTB16-RARA subgroup has been reported to have leukemic cells with regular nuclei, many granules, absence of Auer rods, an increased number of Pelgeroid neutrophils, strong myeloperoxidase (MPO) activity, and all-trans-retinoic-acid (ATRA) resistance. Here, we report a case of variant APL with t(11;17)(q23;q12); ZBTB16-RARA showing typical morphological features of classical APL, including numerous Auer rods and faggot cells. The leukemic cells expressed CD13, CD33, CD117, human leukocyte antigen (HLA)-DR, and cytoplasmic-MPO on the immunophenotyping study. The diagnosis was confirmed by cytogenetic and molecular studies. To distinguish variant APL cases from classical APL cases, regardless of whether morphologically the findings are consistent with those of classical APL, combining morphologic, immunophenotypic, cytogenetic, and molecular studies before chemotherapy is very important.

No MeSH data available.


Related in: MedlinePlus

In the first PCR (A) for screening of leukemia-associated genes, there is a positive band in lane 8 (between 250-300 bp). In the split-out reaction (B) using a single specific primer pair, a t(11;17)(q23;q21); ZBTB16-RARA fusion (285 bp) is confirmed, as revealed by the bands in lane 8A.
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Figure 2: In the first PCR (A) for screening of leukemia-associated genes, there is a positive band in lane 8 (between 250-300 bp). In the split-out reaction (B) using a single specific primer pair, a t(11;17)(q23;q21); ZBTB16-RARA fusion (285 bp) is confirmed, as revealed by the bands in lane 8A.

Mentions: In January 2010, a 52-year-old man was referred to our hospital because of pancytopenia. Complete blood count (CBC) showed pancytopenia with white blood cells, 1,620/µL (segment neutrophils, 12.3%; lymphocytes, 60.5%; monocytes, 27.2%; eosinophils, <1%; basophils, <1%; leukemic cells, 8%); hemoglobin, 8.8 g/dL; and platelets, 98,000/µL. Bone marrow biopsy revealed about 100% cellularity, and that 80% of the nucleated elements were leukemic cells. The leukemic cells showed medium to large size, irregular shape, finely chromatinized nuclei with distinct nucleoli, and moderate amount of blue cytoplasm with azurophilic granules. The leukemic cells showed multiple Auer rods and frequently showed faggot cells (Fig. 1A). They were strongly positive to MPO stain (Fig. 1B). The leukemic cells expressed CD13, CD33, CD117, human leukocyte antigen (HLA)-DR, and cytoplasmic-MPO, as revealed by the immunophenotyping study. ZBTB16-RARA translocation was detected on the leukemia gene screening test by reverse transcription-nested polymerase chain reaction (Fig. 2; Hemavision, DNA technology, Denmark). Cytogenetic study revealed t(11;17)(q23;q21) (Fig. 3). The patient is being treated with chemotherapy without ATRA.


A variant acute promyelocytic leukemia with t(11;17) (q23;q12); ZBTB16-RARA showing typical morphology of classical acute promyelocytic leukemia.

Han SB, Lim J, Kim Y, Kim HJ, Han K - Korean J Hematol (2010)

In the first PCR (A) for screening of leukemia-associated genes, there is a positive band in lane 8 (between 250-300 bp). In the split-out reaction (B) using a single specific primer pair, a t(11;17)(q23;q21); ZBTB16-RARA fusion (285 bp) is confirmed, as revealed by the bands in lane 8A.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2983015&req=5

Figure 2: In the first PCR (A) for screening of leukemia-associated genes, there is a positive band in lane 8 (between 250-300 bp). In the split-out reaction (B) using a single specific primer pair, a t(11;17)(q23;q21); ZBTB16-RARA fusion (285 bp) is confirmed, as revealed by the bands in lane 8A.
Mentions: In January 2010, a 52-year-old man was referred to our hospital because of pancytopenia. Complete blood count (CBC) showed pancytopenia with white blood cells, 1,620/µL (segment neutrophils, 12.3%; lymphocytes, 60.5%; monocytes, 27.2%; eosinophils, <1%; basophils, <1%; leukemic cells, 8%); hemoglobin, 8.8 g/dL; and platelets, 98,000/µL. Bone marrow biopsy revealed about 100% cellularity, and that 80% of the nucleated elements were leukemic cells. The leukemic cells showed medium to large size, irregular shape, finely chromatinized nuclei with distinct nucleoli, and moderate amount of blue cytoplasm with azurophilic granules. The leukemic cells showed multiple Auer rods and frequently showed faggot cells (Fig. 1A). They were strongly positive to MPO stain (Fig. 1B). The leukemic cells expressed CD13, CD33, CD117, human leukocyte antigen (HLA)-DR, and cytoplasmic-MPO, as revealed by the immunophenotyping study. ZBTB16-RARA translocation was detected on the leukemia gene screening test by reverse transcription-nested polymerase chain reaction (Fig. 2; Hemavision, DNA technology, Denmark). Cytogenetic study revealed t(11;17)(q23;q21) (Fig. 3). The patient is being treated with chemotherapy without ATRA.

Bottom Line: The leukemic cells expressed CD13, CD33, CD117, human leukocyte antigen (HLA)-DR, and cytoplasmic-MPO on the immunophenotyping study.The diagnosis was confirmed by cytogenetic and molecular studies.To distinguish variant APL cases from classical APL cases, regardless of whether morphologically the findings are consistent with those of classical APL, combining morphologic, immunophenotypic, cytogenetic, and molecular studies before chemotherapy is very important.

View Article: PubMed Central - PubMed

Affiliation: Department of Laboratory Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.

ABSTRACT
A subgroup of acute leukemia with morphology resembling acute promyelocytic leukemia (APL) shows variant translocations involving RARA and has a different morphology from that of classical APL. The variant APL with t(11;17)(q23;q12); ZBTB16-RARA subgroup has been reported to have leukemic cells with regular nuclei, many granules, absence of Auer rods, an increased number of Pelgeroid neutrophils, strong myeloperoxidase (MPO) activity, and all-trans-retinoic-acid (ATRA) resistance. Here, we report a case of variant APL with t(11;17)(q23;q12); ZBTB16-RARA showing typical morphological features of classical APL, including numerous Auer rods and faggot cells. The leukemic cells expressed CD13, CD33, CD117, human leukocyte antigen (HLA)-DR, and cytoplasmic-MPO on the immunophenotyping study. The diagnosis was confirmed by cytogenetic and molecular studies. To distinguish variant APL cases from classical APL cases, regardless of whether morphologically the findings are consistent with those of classical APL, combining morphologic, immunophenotypic, cytogenetic, and molecular studies before chemotherapy is very important.

No MeSH data available.


Related in: MedlinePlus