Acute Promyelocytic Leukemia Presenting with Bilateral Vitreous Hemorrhage and Retinal Detachment: A Rare Clinical Presentation

Abstract

Acute Promyelocytic Leukemia (APL) is a unique and highly aggressive form of AML that has the potential to cause severe bleeding disorders and is a life-threatening disease unless diagnosed in a timely fashion. The ocular features of leukemia are well known, but bilateral VH with ED as the presenting sign of APL is very unusual. Unique eye findings can cause the diagnosis to be delayed if systemic causes are not entertained early. A previously healthy female aged 36 years came to the clinic with sudden onset of bilateral loss of vision over four days, which was accompanied by headache, exertional dyspnea, palpitation, weakness and fatigue. A complete eye examination disclosed greatly decreased vision in both eyes, and a fundoscopy was performed, which showed that the retina was detached from the back of the eye and that there was a bleeding into the eye in both eyes. General physical examination revealed severe pallor and no icterus, cyanosis, lymphadenopathy or edema. Hematological results included anemia (Hb 9–10 g/dL), extreme thrombocytopenia (34,000–35,000/µL) and increasing leukocytosis. Normocytic normochromic anemia with almost 80% of abnormal promyelocytes was noted on peripheral blood smear. Bone marrow biopsy revealed hypercellular marrow with predominance of abnormal promyelocytes confirming the diagnosis of APL. The patient also had pulmonary infection with right sided consolidation on hospital stay. Initial treatment: platelet transfusion for severe thrombocytopenia, packed red blood cell (PRBC) transfusion for symptomatic anemia. Bilateral VH and RD were recommended to be conservatively managed. Intravenous antibiotics were started for pulmonary infection. Immediate consultation was arranged with hematology and definitive treatment for leukemia was started after confirmation of APL diagnosis on bone marrow. The other systemic and hemorrhagic complications were prevented by early multidisciplinary intervention. Bilateral vitreous hemorrhage with exudative retinal detachment is a rare, but important presenting sign of APL and can be the initial sign of a life-threatening hematological malignancy. Early diagnosis and prompt joint activity between ophthalmologists and hematologists are key to diagnosis and treatment of eye disease. Early treatment may be both sight-saving and life-saving.