1. Grand Rounds Conference Eric Downing MD University of Louisville Department of Ophthalmology and Visual Sciences 9/4/2015

2. Subjective CC/HPI: 55M with complaint of floater and blurry vision just temporally OD x 5 days. POH: none PMH: liver cirrhosis and ascites 2/2 EtOH abuse, anemia and thrombocytopenia, DM2, HTN, morbid obesity Eye Meds: none Meds: Insulin, Metoprolol, Lisinopril, Lasix, Albumin

3. Objective OD OS OD OS VA: 20/25-20/20 Pupils: 3→23→2, no rAPD IOP:1412 EOM:fullfull CVF:fullfull

4. Objective SLE: E/L/L:WNL OU C/S:White, quiet OU K:Clear OU AC:D&Q OU I/L:1+NS OU Vit:no vitritis DFE:

5. Clinical photos

6. Differential Diagnosis Subacute bacterial endocarditis Subacute bacterial endocarditis Anemia Anemia Leukemias Leukemias Diabetes Diabetes HTN HTN Sepsis Sepsis

7. Labs WBC: 17.1 WBC: 17.1 Hgb/Hct: 6.7/20.4 Hgb/Hct: 6.7/20.4 Plt: 29 Plt: 29 Blood culture: negative Blood culture: negative Blood smear: many immature granulocytes and blast cells Blood smear: many immature granulocytes and blast cells TEE with no vegetations TEE with no vegetations

8. Assessment 55M with multiple retinal hemorrhages and Roth spots OU. 55M with multiple retinal hemorrhages and Roth spots OU. Elevated WBC with elevated blast ratio Elevated WBC with elevated blast ratio Anemia/thrombocytopenia Anemia/thrombocytopenia Dx: Acute Myelogenous Leukemia with Leukemic retinopathy Dx: Acute Myelogenous Leukemia with Leukemic retinopathy

9. Plan Chemo not initiated due to comorbidities Chemo not initiated due to comorbidities Palliative 5 day course of Decitabine initiated Palliative 5 day course of Decitabine initiated

10. Roth Spot Originally described as Retinitis Septica by Moritz Roth in 1872 Originally described as Retinitis Septica by Moritz Roth in 1872 In 1878 Litten termed them the Roth spot In 1878 Litten termed them the Roth spot Now the term is used to describe any white- centered hemorrhage Now the term is used to describe any white- centered hemorrhage

11. Histopathology Retinal capillary rupture Retinal capillary rupture Extrusion of whole blood Extrusion of whole blood Platelet adhesion and activation Platelet adhesion and activation Coagulation cascade Coagulation cascade Platelet-fibrin thrombus Platelet-fibrin thrombus

12. Subacute Bacterial Endocarditis History: fever, night sweats, weight loss, dental work History: fever, night sweats, weight loss, dental work Fever (90%), heart murmur (85%), petechiae, splinter hemorrhages, Osler’s nodes Fever (90%), heart murmur (85%), petechiae, splinter hemorrhages, Osler’s nodes Polymorphous or mononuclear cells with a surrounding hemorrhage Polymorphous or mononuclear cells with a surrounding hemorrhage Roth spots only observed in 5% of these patients Roth spots only observed in 5% of these patients Litten’s sign: CWSs associated with SBE Litten’s sign: CWSs associated with SBE

13. Leukemias Ocular findings in about 40% Ocular findings in about 40% Positive correlation between levels of anemia and/or thrombocytopenia Positive correlation between levels of anemia and/or thrombocytopenia Exam Exam Hemorrhages involving any/all layers of retina Hemorrhages involving any/all layers of retina Perivascular sheathing Perivascular sheathing Pale-swelling of the optic nerve Pale-swelling of the optic nerve Vascular tortuosity possibly related to hyperviscosity Vascular tortuosity possibly related to hyperviscosity CWS are poor prognostic factor CWS are poor prognostic factor

14. Anemia Central fibrin collection with heme Central fibrin collection with heme Retinopathy observed in ~28% of pts, especially those with Hgb <8 and plt <50 Retinopathy observed in ~28% of pts, especially those with Hgb <8 and plt <50 75% also have conjunctival pallor 75% also have conjunctival pallor

15. Prognostic Importance of Retinopathy in Acute Leukemia Prospective study of 54 patients with ALL or AML Prospective study of 54 patients with ALL or AML 35% had retinopathy at diagnosis 35% had retinopathy at diagnosis In general, those with retinopathy fared worse In general, those with retinopathy fared worse Patients with CWSs and Roth spots had a mortality rate 8 times higher than those without Patients with CWSs and Roth spots had a mortality rate 8 times higher than those without Age >40 incurred a 7 times higher mortality risk Age >40 incurred a 7 times higher mortality risk

16. References 1. Ling R, James B. White-centered retinal hemorrhages (Roth spots). Postgrad Med J. 1998 Oct; 74(876):581-582. 2. Guyer DR, Schachat AP, Vitale S, et al. Leukaemic retinopathy. Relationship between fundus lesions and haematologic parameters at diagnosis. Ophthalmology. 1980;87:66-9 3. Abu el-Asrar AM, al-Mornen AK, Kangave D, Harakati MS. Prognostic importance of retinopahy in acute leukemia. Doc Ophthalmol. 1995;91(3):273- 81. 4. Carraro MC, Rossetti L, Gerli GC. Prevalence of retinopathy in patients with anemia or thrombocytopenia. Eur J Haematol. 2001 Oct;67(4):238-44. 5. BCSC Ophthalmologic Pathology and Intraocular Tumors. pp160-161 6. Macauley M, Nag S. Roth spots in pernicious anemia. BMJ Case Reports. 2011;doi:10.