Bilateral Endogenous Bacterial Endophthalmitis and Bacteraemia as the presenting manifestation of Multiple Myeloma. Peter Cikatricis Peter Cikatricis 1, 3 Korina Theodoraki Korina Theodoraki 1 Yit C.Yang Yit C.Yang 3, 4 Alastair K.O. Denniston Alastair K.O. Denniston 1, 2, 3 1 Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom 2 Centre for Translational Inflammation Research, University of Birmingham, Birmingham, United Kingdom 3 Wolverhampton Eye Infirmary, Royal Wolverhampton Hospitals NHS Trust, Wolverhampton, United Kingdom 4 Aston University, Birmingham, United Kingdom

Ocular History  59-year old Caucasian male  12/ day history of painless decrease of vision in both eyes (L > R)  Headache, Fever, Nausea and Vomiting  POcHx: Left strabismic amblyopia (20/40 BCVA)  PMHx: Dental work 2 weeks prior, ex-smoker  DHx: Nil

First Clinical Presentation  Bilateral asymmetric vitritis with left panuveitis  Fundus infiltrative lesions (OS and ?OD)  Systemic signs of infection

Examination at First Presentation Fever 39.5 °C (103.1°F) Tachycardia 122 bpm Auscultation Grade 4/6 Pan-systolic murmur EYERightLeftBCVA20/40HM A/S & IOP Unremarkable, 12 Ciliary injection, 14 A/C Cells +, Flare + Cells 2-3+, Hypopyon Pupil Reactive/No RAPD PS++ LensClearClear

Vitreous Few Cells Fundus Roth spots? minimal exudate Right Eye Vitreous Marked vitritis (Grade 4 haze) Fundus Very limited view Left Eye

Differential Diagnoses Infection:  Bacterial  Viral  HIV/Syphilis  Toxoplasmosis  Fungal Inflammation:  Atypical Sarcoidosis  Severe HLA- B27-ass. Masquerade:  Lymphoma  Other blood malignancies  Paraneoplastic

Initial Investigations  Bloodwork (CBC, biochemistry, ESR, CRP, ACE, Ca 2+, ANA, ANCA, TPHA, HIV, Toxoplasma, Borrelia, TB T-Spot)  Blood cultures  CT/MRI of head and chest  Trans-oesophageal Echocardiogram requested

Lab Results  ESR – 62 mm/h (<30)  CRP – 246 mg/L (<10)  white blood cell count – 14.2 x10 9 /L ( )  neutrophils – 12.1 x10 9 /L (2.5–7.5)  all serology negative but…  Streptococcus Pneumoniae (Serotype 23B) in blood cultures, possible sources:  recent dental work  bacterial endocarditis

 Transoesophageal echocardiogram: mobile mass at the mitral valve (central on the video below)  severe, posteriorly directed jet of mitral regurgitation (light blue flow below)  normal Transoesophageal echocardiogram

Diagnosis  Pneumococcus endogenous endophthalmitis  Caused by Pneumococcal bacteraemia from endocarditis

Initial Treatment IV Vancomycin 1g STAT IV Meropenem 1g STAT Intravitreal Vancomycin 2mg – R & L Hospitalised for intravenous antibiotics: IV Vancomycin 1g BD Meropenem 1g TDS In 3 weeks prepared for therapeutic mitral valve replacement – 31mm valve implanted Right Eye Left Eye 20/15  NAD  Resolution 20/120  Reduced hypopyon  Vitritis - Grade 3 Vitreous haze

However the story continued...  Screening for possible underlying immunosuppression was negative for HIV but..  Elevated paraproteins – 33.2 g/L (>30 g/L)  diagnostic of multiple myeloma  bone marrow biopsy confirmed:  IgG-Multiple Myeloma

Bone Marrow Biopsy  Histopathology  Skeletal Survey  Low power view of bone marrow  High power view of plasma cells  CD138 immuno- histochemistry staining of plasma cells  Lucencies seen in the proximal femoral shafts, within L5, in the mid-humeral shaft

Further Treatment  4 weeks after initial presentation discharged from hospital  Good cardiac and systemic recovery  VA at discharge:  20/15 OD  20/40 OS (recovery of his normal level of vision in amblyopic eye )

Control after 8 weeks  Left eye  retinal detachment  treated by ppvitrectomy/cryo/gas  final visual outcome at 4 months:  20/15 OD  20/80 OS (due for cataract surgery)

Final Diagnosis  Bilateral endogenous pneumococcal endophthalmitis, caused by endocarditis  Multiple Myeloma predisposed the patient to develop bacterial endocarditis  first case described in the literature

Conclusion  The onset of Multiple Myeloma is often insidious  It is of utmost importance to ascertain underlying diagnosis of bilateral endophthalmitis in timely fashion in order to deliver effective treatment  Haematological malignancies should be considered as one of the causes of acquired immunosuppression in cases of endogenous endophthalmitis