intraocular histiocytosis masquerading as uveitis
TRANSCRIPT
Intraocular Histiocytosis masquerading as Uveitis
Mayur R. Moreker
Tanuj R. SharmaKeyuri PatelB. K. SmrutiAjay Dudani
Taparia Institute of OphthalmologyBombay Hospital Institute of Medical Sciences
Dr. Mayur R. MorekerTaparia Institute of OphthalmologyBombay Hospital Institute of Medical Sciences , Mumbai.
PurposeTo describe "Isolated Intraocular Histiocytosis", a rare entity masquerading clinically as various uveitis presentations in the same patient at different visits
MethodsRetrospective chart review of 2 cases with biopsy proven "Intraocular Histiocytosis"
Dr. Mayur R. MorekerTaparia Institute of OphthalmologyBombay Hospital Institute of Medical Sciences , Mumbai.
MethodsA 25 year old gentleman with bilateral chronic uveitis presented to various uveitis clinics over past 2 years at different points of time with typical clinical features of :
• Presumed Ocular Tuberculosis (Granulomatous Intermediate Uveitis with Positive Quantiferron TB Gold test)
Dr. Mayur R. MorekerTaparia Institute of OphthalmologyBombay Hospital Institute of Medical Sciences , Mumbai. Masquerading as Intermediate Uveitis
MethodsA 25 year old gentleman with bilateral chronic uveitis presented to various uveitis clinics over past 2 years at different points of time with typical clinical features of :
• Toxoplasma Chorioretinitis (Headlight in fog appearance)
Dr. Mayur R. MorekerTaparia Institute of OphthalmologyBombay Hospital Institute of Medical Sciences , Mumbai. Masquerading as Toxoplasmosis
MethodsA 25 year old gentleman with bilateral chronic uveitis presented to various uveitis clinics over past 2 years at different points of time with typical clinical features of :
• Behcet’s Disease (Hypopyon with peripheral retinal vasculitis)
He was HIV negative and was extensively investigated (CBC/ESR, Mantoux Test, Serum ACE, TORCH titers, HLA B5, HLA B51, ANA, ANCA, MRI Brain with Orbit - All inconclusive). He was treated with anti-Toxoplasma drugs, oral steroids, immunosuppressives and anti-Koch’s drugs with only partial response
MethodsA vitreous biopsy showed Histiocyte predominance on cytology (confirmed to be of non-Langerhans type with immunohistochemistry, CD 68 highlighting Histiocytes, but CD 1a and S - 100 protein not expressed) with negative bacterial, fungal and Koch’s cultures.
Patient was then screened by an oncologist to rule out other organ involvement and put on chemotherapy for Histiocytosis with Vinblastine to show complete response.
Dr. Mayur R. MorekerTaparia Institute of OphthalmologyBombay Hospital Institute of Medical Sciences , Mumbai. Resolution with Vinblastine
MethodsA 32 year old lady presented to various uveitis clinics over past 6 months with unilateral chronic uveitis with typical clinical features of:
• Presumed Ocular Tuberculosis (Mutton fat keratic precipitates, Bussaca Nodules and positive Mantoux test) and treated accordingly with no response and relapsing hypopyon uveitis
Dr. Mayur R. MorekerTaparia Institute of OphthalmologyBombay Hospital Institute of Medical Sciences , Mumbai. Masquerading as Tuberculosis
MethodsPatient was HIV Negative and showed marked peripheral Eosinophilia during relapses.
Extensive further investigations (CBC/ESR, Serum ACE, Quantiferron TB Gold Test, ANA, ANA Blot, ANCA, HLA B51, HLA B5) to rule out Koch’s Disease, Sarcoidosis, Churg Strauss Syndrome (in view of eosinophilia) were to no avail.
Dr. Mayur R. MorekerTaparia Institute of OphthalmologyBombay Hospital Institute of Medical Sciences , Mumbai.
MethodsA vitreous biopsy showed Histiocyte predominance on cytology further complicated with a culture proven sensitive growth of Sphingomonas Paucimobilis.
Patient was then screened by an oncologist for other organ involvement.
Dr. Mayur R. MorekerTaparia Institute of OphthalmologyBombay Hospital Institute of Medical Sciences , Mumbai.
Histiocyte predominance on cytology Reniform nuclei and Longitudnal grooves
MethodsShe was treated first with oral Azathioprine and oral Ciprofloxacin with an immediate good response but relapses. Patient was then screened by an oncologist to rule out other organ involvement and put on chemotherapy for Histiocytosis with Vinblastine to show complete response.
Dr. Mayur R. MorekerTaparia Institute of OphthalmologyBombay Hospital Institute of Medical Sciences , Mumbai. Resolution with Vinblastine
BCVA with aphakic correction - 20/20, N6
Isolated ocular Histiocytosis without other systemic manifestations is extremely rare, and when it does occur, it is characterized by recurrent uveitis with a tumorous intraocular growth.
Most patients respond poorly to systemic corticosteroid therapy and require chemotherapy.
Sikic J, Vukojevic N, Popovic-Suic S, Katusic D. Intraocular Histiocytosis in a 12-Year –Old Girl Without Systemic Disease. Coll. Antropol. 29 (2005) Suppl. 1: 119 - 121
Discussion
Dr. Mayur R. MorekerTaparia Institute of OphthalmologyBombay Hospital Institute of Medical Sciences , Mumbai.
Conclusion• "Intraocular Histiocytosis" is a rarely reported clinical
entity which masquerades as "Hypopyon Uveitis" and clinical features typical of other forms of uveitis.
• Diagnosis is achieved by cytological sampling of the vitreous biopsy and treatment is with steroids and immunosuppressive chemotherapy.
Dr. Mayur R. MorekerTaparia Institute of OphthalmologyBombay Hospital Institute of Medical Sciences , Mumbai.