![A 76-year-old woman presented generalized weakness and easy bruising. Her laboratory values showed a white blood cell count of 15.1 × 109/L, hemoglobin 90 g/L, and platelets 19 × 109/L with 85% circulating abnormal promyelocytes, many of which with Chediak-Higashi like giant granules (panels A,B: Wright stain, original magnification ×1000 for both panels). The promyelocytes were uniformly and strongly positive for cytochemical myeloperoxidase stain (panel C: original magnification ×1000). No Auer rods were seen. Other laboratory values were fibrinogen 1.58 g/L and d-dimer >20 mg/L. Bone marrow revealed 71% promyelocytes with similar morphological features (panels D,E: Giemsa-Wright stain, original magnification ×1000 for both panels; panel F: myeloperoxidase with counterstain, original magnification ×1000). Flow cytometry study demonstrated a population of aberrant myeloid precursors, positive for CD13, CD33, CD34 (subset), CD38, CD64, CD117 (subset), and CD123, and negative for HLA-DR, and T- and B-lineage markers. Fluorescence in situ hybridization study revealed a clone with PML/RARA rearrangement, whereas molecular analysis detected the PML-RARA bcr3/short fusion transcript, establishing the diagnosis of acute promyelocytic leukemia with PML-RARA. Cytogenetics detected 46, XX; t(15;17)(q22;q21)[18]. Patient was treated with all trans retinoid acid, arsenic trioxide, and idarubicin, and a morphological remission was achieved in the bone marrow.](https://ash.silverchair-cdn.com/ash/content_public/journal/blood/139/1/10.1182_blood.2021013975/4/m_bloodbld2021013975f1.png?Expires=1737701649&Signature=SwEcfg-YF5rkDzcwK-Ol1A0tH0zIWs2Yqf~TxOFgC-RzFyp~d7qwAI4vq0rP0jwXIgNmA3n71xSRRdEwBKI3vmbbw~ItSVqu7pQvsIBu-DJuHeWre9kVJwFxKO-jQylcSgguK4EbtcgGqKAslZIugaKiXkl-gJOeyZo0BRXutJr-MLfosUb2T6NpNgVlmuitkSo35CK918L3a0Kh8DZ2e3xtM5YJQPNQGciw8T04aEDZnYvb9f6n9wVoTwSop0whS4mprcxa2E3PmKbx92u6xUI-tjsmcguWJl23vuCz8ND~CFiQpivOBKklUMm2beKw9jYbJiXmcuUEXTX6SXc63w__&Key-Pair-Id=APKAIE5G5CRDK6RD3PGA)
A 76-year-old woman presented generalized weakness and easy bruising. Her laboratory values showed a white blood cell count of 15.1 × 109/L, hemoglobin 90 g/L, and platelets 19 × 109/L with 85% circulating abnormal promyelocytes, many of which with Chediak-Higashi like giant granules (panels A,B: Wright stain, original magnification ×1000 for both panels). The promyelocytes were uniformly and strongly positive for cytochemical myeloperoxidase stain (panel C: original magnification ×1000). No Auer rods were seen. Other laboratory values were fibrinogen 1.58 g/L and d-dimer >20 mg/L. Bone marrow revealed 71% promyelocytes with similar morphological features (panels D,E: Giemsa-Wright stain, original magnification ×1000 for both panels; panel F: myeloperoxidase with counterstain, original magnification ×1000). Flow cytometry study demonstrated a population of aberrant myeloid precursors, positive for CD13, CD33, CD34 (subset), CD38, CD64, CD117 (subset), and CD123, and negative for HLA-DR, and T- and B-lineage markers. Fluorescence in situ hybridization study revealed a clone with PML/RARA rearrangement, whereas molecular analysis detected the PML-RARA bcr3/short fusion transcript, establishing the diagnosis of acute promyelocytic leukemia with PML-RARA. Cytogenetics detected 46, XX; t(15;17)(q22;q21)[18]. Patient was treated with all trans retinoid acid, arsenic trioxide, and idarubicin, and a morphological remission was achieved in the bone marrow.
Awareness of this rare morphological variant of acute promyelocytic leukemia will help promptly establish the diagnosis.
A 76-year-old woman presented generalized weakness and easy bruising. Her laboratory values showed a white blood cell count of 15.1 × 109/L, hemoglobin 90 g/L, and platelets 19 × 109/L with 85% circulating abnormal promyelocytes, many of which with Chediak-Higashi like giant granules (panels A,B: Wright stain, original magnification ×1000 for both panels). The promyelocytes were uniformly and strongly positive for cytochemical myeloperoxidase stain (panel C: original magnification ×1000). No Auer rods were seen. Other laboratory values were fibrinogen 1.58 g/L and d-dimer >20 mg/L. Bone marrow revealed 71% promyelocytes with similar morphological features (panels D,E: Giemsa-Wright stain, original magnification ×1000 for both panels; panel F: myeloperoxidase with counterstain, original magnification ×1000). Flow cytometry study demonstrated a population of aberrant myeloid precursors, positive for CD13, CD33, CD34 (subset), CD38, CD64, CD117 (subset), and CD123, and negative for HLA-DR, and T- and B-lineage markers. Fluorescence in situ hybridization study revealed a clone with PML/RARA rearrangement, whereas molecular analysis detected the PML-RARA bcr3/short fusion transcript, establishing the diagnosis of acute promyelocytic leukemia with PML-RARA. Cytogenetics detected 46, XX; t(15;17)(q22;q21)[18]. Patient was treated with all trans retinoid acid, arsenic trioxide, and idarubicin, and a morphological remission was achieved in the bone marrow.
Awareness of this rare morphological variant of acute promyelocytic leukemia will help promptly establish the diagnosis.
For additional images, visit the ASH Image Bank, a reference and teaching tool that is continually updated with new atlas and case study images. For more information, visit http://imagebank.hematology.org.
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