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Morphologic heterogeneity of acute promyelocytic leukemia (APL)

Morphologic heterogeneity of acute promyelocytic leukemia (APL)

Journal of Clinical Oncology 23(16_Suppl): 6741-6741

NlmCategory="UNASSIGNED">6741 Background: Three morphological forms of APL recognized; hypergranular, hypogranular and hyperbasophilic forms. Other subtypes were rare, however, have been described. We describe a case of AML with PML-RARα fusion gene without above morphology. A 67-year-old caucasian male was hospitalized with diverticulits. He received radioactive seed-implants for prostate cancer 1.5 years ago. CBC showed WBC 9,000/cmm, Hemoglobin 12.4g/dl, platelet 25,000/cmm, neutrophil 5%, band 3%, lymphocyte 30%, monocyte 20%, metamyelocyte 2%, myelocyte 15%, promyelocyte 15%, myeloblast 10%. Clotting profile was normal except INR 1.6 and elevated D-dimers. Bone marrow biopsy was consistent with AML-M2. Induction chemotherapy was initiated resulting in worsening DIC. This raised the suspicion for morphologic variant of APL. The cytogenetic study revealed no diving cells. FISH and PCR studies were positive for PML-RARα fusion gene. He was then started on all-trans retinoic acid. Currently he has received 2 cycles of consolidation chemotherapy. The literature was reviewed for atypical morphologic presentations of APL on MEDLINE. In a review of the literature of t(15:17) in AML subtypes other than M3, there have been 16 reported cases including our case. Male: Female:Unknown- 5:8:3; Subtypes: M1- 6, M2- 5, M4- 1, acute esosinophilic leukemia- 1, M7- 2, undifferentiated L2/M1- 1; DIC: Yes- 4, No-4, unknown- 8; Treatment with ATRA: Yes- 7, No- 6, unknown- 3; Outcomes: Complete hematologic remission - Yes- 7, Died - 3, unknown- 6. Molecular responses: Yes- 1, No- 6, unknown- 9. Patients with t(15;17) are amenable to treatment with ATRA making this most curable AML. The diagnosis of APL can be made from morphology and flow cytometry, but our case and the literature illustrate there are occasions where APL may present with atypical morphology. All efforts must be made rule out PML-RARα gene rearrangement in AML patients, especially with DIC. [Table: see text] No significant financial relationships to disclose.

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Accession: 058348061

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PMID: 27944782

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