Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disease in which antibodies directed against one’s own platelets cause their peripheral destruction and splenic sequestration, resulting in a low platelet count and, occasionally, bleeding complications. It is a relatively rare disorder, affecting approximately 1 in 10 000 in the general population.1 It is generally considered a benign disease, and most adult patients with ITP are in otherwise good health. Idiopathic thrombocytopenic purpura most often manifests with petechial skin rash, bruising, or mucosal bleeding. Ophthalmic involvement is exceptionally rare.2 We report a life-threatening case of ITP, initially manifesting with visual complaints secondary to massive subretinal hemorrhage.
A previously healthy, 26-year-old Asian woman was seen with a 5-day history of blurred vision in the left eye. She denied trauma, headache, or toxic exposure. Examination revealed visual acuity of 20/30 OD and counting fingers at 3 ft OS. There was no relative afferent pupillary defect, and ocular motility, intraocular pressures, and anterior segment were normal. Dilated fundus examination revealed bilateral subretinal and intraretinal hemorrhages, which were greater in the left eye (Figure 1).
Fundus photographs of the right eye (A) and left eye (B) at initial examination showing subretinal and intraretinal hemorrhage.
The patient was sent for laboratory testing to evaluate for hematologic dyscrasia. Two days later, she developed severe headache and confusion. She was seen in the emergency department, where she was found to be obtunded with epistaxis and petechial rashes on her lower extremities. The patient was intubated to protect the airway, and a complete blood cell count showed a hemoglobin level of 6.7 g/dL and a platelet count of 33× 103/μL with a normal prothrombin time, partial thromboplastin time, D-dimer test result, and fibrinogen level. A computed tomographic scan of the head revealed a large right frontal intracranial hemorrhage (Figure 2), and the patient underwent emergent neurosurgical evacuation of the hematoma.
Computed tomography demonstrating large right frontal intracerebral hemorrhage.
The patient was initially treated with blood and platelet transfusion, intravenous immunoglobulin, and dexamethasone. She required multiple subsequent transfusions until definitive treatment was achieved following laparoscopic splenectomy. A bone marrow biopsy specimen supported the diagnosis of ITP, showing increased megakaryopoiesis and erythropoiesis without other significant abnormalities. Three months later, after intensive occupational rehabilitation, the patient returned to near baseline functional status. Follow-up examination revealed a visual acuity of 20/20 OD and 20/100 OS with residual serous retinal detachment and exudates in the left macula (Figure 3).
Fundus photographs of the right eye (A) and left eye (B) 3 months after initial examination.
Ophthalmic manifestations of ITP are rarely encountered, and ITP initially manifesting with decreased visual acuity secondary to retinal hemorrhage has not, to our knowledge, been previously reported. Idiopathic thrombocytopenic purpura can rarely result in significant morbidity or even mortality from bleeding complications.3 Other ophthalmic manifestations associated with ITP include vitreous hemorrhage associated with intracranial bleeding in a Terson type phenomenon,4 hemorrhage within the optic tract,5 nonarteritic anterior ischemic optic neuropathy,6 and subconjunctival hemorrhage.7 Thrombocytopenia alone, even severe (a platelet count <50 000), is rarely sufficient to cause significant retinal hemorrhage.8 However, thrombocytopenia combined with anemia is a known risk factor,9 and retinal hemorrhages in association with ITP have only been reported to occur with concurrent anemia.2,8
Retinal hemorrhages are often an indication of an underlying blood dyscrasia, and their presence, in the absence of a known etiology, warrants evaluation. In addition to the ophthalmic examination, a thorough medical history, review of systems, and physical assessment of the skin and mucosal surfaces may aid the physician in assessing the urgency of hematologic evaluation. Patients exhibiting significant abnormalities may warrant urgent evaluation to initiate potentially life-saving interventions in rare instances of bleeding complications such as those reported herein.
Correspondence: Dr Shah, Department of Ophthalmology, California Pacific Medical Center, 2340 Clay St, Suite 500, San Francisco, CA 94115 (firstname.lastname@example.org).
Financial Disclosure: None.
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