Abstract :
B/T Mixed phenotype acute leukemias (MPAL) presenting with Acute promyelocytic leukemia (APML) like catastrophic bleeding manifestations is extremely rare. Our patient was a six years male who presented with fever, bleeding gums and skin bleeds for three days. Physical examination revealed pallor, multiple ecchymotic patches and splenomegaly. He had pancytopenia with 2% blasts with folded nuclei on the peripheral smear and deranged coagulation parameters. In view of the above, APML was suspected and patient was administered ATRA and FISH for PML-RARA on peripheral blood was sent. Throat swab for RT-PCR was positive for COVID-19 and X-ray chest showed bilateral ground glass opacities. In view of worsening saturation, he was put on Bilevel Positive Airway Pressure and later mechanical ventilation. He was managed with antibiotics, blood components and inotropes after an episode of hematemesis induced hypovolemic shock. However, patient deteriorated and succumbed to his illness. Post-mortem bone marrow showed 88% blasts which were positive for CD19, cyto CD79a, CD10, cyto CD3, CD34 and TdT. RT-PCR for PML-RARA (Promyelocytic leukemia/ retinoic acid receptor alpha) and break apart FISH for RARA (retinoic acid receptor alpha) were negative. A final diagnosis of B/T MPAL, NOS (Not otherwise specified) was made post-mortem. The aim of this case report is to highlight the extremely rare presentation of a rare leukemia in children and blasts with convoluted nuclei leading to a diagnostic dilemma. Our patient had a tumultuous course aggravated by the COVID19 pneumonia leading to fatal outcome.
Keyword :
Bleeding, Mixed phenotype acute leukemias, Acute promyelocytic leukemia.