Acute Promyelocytic Leukemia in a Woman with Thalassemia Intermedia: Case Report and Review of Literature on Hematological Malignancies in β-Thalassemia Patients

Abstract

Patients affected by transfusion-dependent β-thalassemia are prone to developing several clinical complications, mostly related to the iron overload. We report the case of a patient affected by transfusion-dependent β-thalassemia (TDT) developing acute promyelocytic leukemia (APL). In our case, the therapeutic management was significantly complicated not only by myocardial dysfunction, but also by the occurrence of the differentiation syndrome following all-trans retinoic acid (ATRA) administration. We carried out a careful revision of the current literature on the occurrence of hematological malignancies in β-thalassemia patients to investigate the major complications so far described. APL occurrence in β-thalassemia patients has been very rarely reported, and our experience suggests that TDT patients suffering pre-existing comorbidities may develop a potentially fatal complication during ATRA therapy.

Keywords: ATRA differentiation syndrome; acute promyelocytic leukemia; hematological malignancies; retinoic acid; β-thalassemia.

Figure 1

(A) Upper picture: peripheral blood smear, showing anisopoikilocytosis, microcytic hypochromic red cells, and numerous target cells. In the center, a leukemic promyelocyte (black arrow). (B) Lower picture: bone marrow blood smear, showing promyelocytes (black arrows) and marked dyserythropoiesis (red arrow).

Figure 2

Gantt graph displaying patient’s clinical course during the treatment of acute promyelocytic leukemia (Office Timeline.Version 6.00.06, 2021 Office Timeline Inc., Bellevue WA 98004, USA). AF: atrial fibrillation; ATRA: all trans-retinoid acid; VM: Ventimask.

Figure 3

Chest X-ray and TC scan documenting bilateral pulmonary infiltrates and pleural effusion during ATRA differentiation syndrome (Carestream Vue Motion Version 12.1.5.9529 © Carestream Health, Inc., Rochester, NY, USA, 2021). D: RIGHT side.