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Letters to the Editor should order IgG4 serology and histological staining in suspected cases. Increasing awareness of this entity may help address clinical ambiguity surrounding the labels ‘idiopathic orbital inflammation’ and ‘reactive hyperplasia of lymph nodes’.
Rajan S Patheja MBBS(Hons), Mahendra Singh FRCPA, James A Bennett MBBS and Timothy J Sullivan FRANZCO Department of Ophthalmology, Royal Brisbane and Women’s Hospital, Brisbane, Queensland, Australia Received 12 June 2015; accepted 23 June 2015.
REFERENCES 1. Sato Y, Inoue D, Asano N et al. Association between IgG4-related disease and progressively transformed germinal centres of lymph nodes. Mod Pathol 2012; 25: 956–67. 2. McNab AA, McKelvie P. IgG4-related ophthalmic disease. Part 1: background and pathology. Ophthal Plast Reconstr Surg 2015; 31: 83–8. 3. Kojima M, Nakamura N, Sakamoto K et al. Progressive transformation of the germinal centre of extranodal organs: a clinicopathological, immunohistochemical, and genotypic study of 14 cases. Pathol Res Pract 2010; 206: 235–40. 4. Ferry JA. IgG4-related lymphadenopathy and IgG4related lymphoma: moving targets. Diagn Histopathol 2013; 19: 128–39. 5. Seki N, Yamazaki N, Koizumi J et al. A case of progressively transformed germinal centre-type IgG4-related lymphadenopathy. Auris Nasus Larynx 2015; e-pub. http://dx.doi.org/10.1016/j.anl.2015.02.010
Acute dacryocystitis as a presenting sign of chronic lymphocytic leukaemia Acute dacryocystitis is characterized by rapid onset of pain and erythematous swelling predominantly below the medial canthus resulting mainly from an acute infection of the lacrimal sac and perisac tissues. Although rare, involvement of the lacrimal drainage system with leukaemic infiltrates were reported and believed to be facilitated by the presence of lacrimal drainage-associated lymphoid tissue (LDALT).1–5 To the best of the authors knowledge, acute dacryocystitis as a presenting feature of a yet to be diagnosed chronic lymphocytic leukaemia (CLL) has been reported once before but with unclear details. We report the second such case which clearly shows acute dacryocystitis as a presenting feature of a CLL. Competing/conflicts of interest: None.
A 59-year-old otherwise healthy female presented with swelling and pain on the inner side of left eye of 5 days duration, associated with discharge and epiphora from the left eye. Constitutional symptoms were absent. There was no history of epiphora, trauma or surgery. On examination, there was tender swelling over the left lacrimal sac area with surrounding induration and preseptal cellulitis (Fig. 1a). Ocular examination was otherwise normal except for bilateral aphakia following cataract surgery. Microbiological cultures were negative. The patient was started on systemic broad spectrum antibiotic and antiinflammatory agents. A week later, there was a suboptimal response (Fig. 1b). Endoscopic evaluation of the nasal cavity was within normal limits (Fig. 1c), and the patient was scheduled for an endoscopic dacryocystorhinostomy. Routine pre-surgical blood counts showed marked leukocytosis (Total leucocyte count – 73 100/u˛l) with lymphocytosis without any anaemia or thrombocytopenia. A peripheral blood smear showed similar picture (Fig. 1d). Bone marrow biopsy showed marked lymphocytosis with predominantly monotonous population of small lymphocytes (Fig. 2a). Flow cytometric analysis showed 60% of atypical B-lymphocytes with bright expression of CD19 and HLADR with moderate expression of CD23, CD200 and CD45. The CD19-gated cells were 0.4% positive for CD38 and 1.9% positive for ZAP-70. The scatter parameters and antigenic expression profile by flow cytometry (Fig. 2b) were suggestive of B-cell chronic lymphocytic leukaemia (B-CLL). Systemic examination did not reveal any lymphadenopathy, hepatomegaly or splenomegaly. The B-CLL was staged as Rai Stage 0 and Binet Stage I with a favourable prognosis. In view of the early stage of the disease, the patient is being closely monitored on a 2-weekly basis. The patient complained of increasing pain on the left side again after 2 weeks and underwent a dacryocystectomy. Surgery was uneventful except for increased intraoperative bleeding. At the last follow-up (3 months following the surgery), the patient was asymptomatic with resolution of pain and swelling. Histopathological examination revealed dense subepithelial lymphocytic infiltrates (Fig. 2c). Immunohistochemistry showed the cells to be positive to CD20 (Fig. 2d), CD5 (Fig. 2e) and CD23 (Fig. 2f). The histological features were consistent with predominantly B-lymphocytic infiltrate of the lacrimal mucosal tissues. Chronic lymphocytic leukaemia is a common form of leukaemia in adults usually suspected when blood counts show marked lymphocytosis and is confirmed by flow cytometry and target gene analysis. Lacrimal drainage obstructions and subsequent acute or chronic dacryocystitis are believed to be secondary to either a direct leukaemic infiltration or an abnormal stimulation and subsequent overgrowth of pre-existing lymphoid elements.1,4 Wirostko et al.2 documented acute dacryocystitis as a presenting sign of acute leukaemia in pediatric age group, which was successfully managed with antibiotics and chemotherapy. Stokes1 reported a case of bilateral chronic dacryocystitis with a fistula in a 68-year-old woman, where CLL was diagnosed on histological examinations of
Funding sources: None.
© 2015 Royal Australian and New Zealand College of Ophthalmologists
68 Figure 1. External photograph of the patient showing gross leftsided medial canthal swelling with preseptal cellulitis (a). Suboptimal clinical response at 1 week (b). Endoscopic view of the left nasal cavity showing normal lateral wall (c). Microphotograph of the peripheral smear showing lymphocytosis (Lieshman x100, d).
Figure 2. Microphotograph of the bone marrow aspiration showing marked lymphocytosis (Giemsa x400, a). Scatter parameters and antigenic expression profile of flow cytometry (b). Microphotograph of the lacrimal sac showing dense sub-epithelial lymphocytic infiltrates (H&E x100, c). Immunohistochemistry microphotographs of the lacrimal sac showing positivity to CD20 (CD20 x100, d), CD5 (CD5 x100, Fig. 2e) and CD23 (CD23 x400, f).
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© 2015 Royal Australian and New Zealand College of Ophthalmologists
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Letters to the Editor lacrimal sac following a dacryocystectomy. However, this patient had a history of previous repeated attacks of acute dacryocystitis and lacrimal abscess. One case of acute dacryocystitis in a 69-year-old man, following eight cycles of chemotherapy for CLL was reported by Karesh et al.3 The patient was treated by antibiotics followed by a dacryocystorhinostomy (DCR). Chemotherapy was initiated after surgery and the patient was symptom free at 17 months follow-up. Yip et al.4 reported a case of 84-year-old woman, primarily treated by chemotherapy for CLL and presented with acute dacryocystitis 2 years later, which was successfully managed with a dacryocystorhinostomy. Management options for acute dacryocystitis in a leukaemic patient include systemic antibiotics, chemotherapy when indicated, dacryocystorhinostomy or a dacryocystectomy. DCR is a preferred surgical option. Yip et al.4 in their series of CLL, showed successful outcomes in seven of the nine patients (one had acute dacryocystitis). However, restenosis secondary to lymphocytic infiltrations after successful DCR surgeries and dry eyes secondary to graft versus host disease have also been reported.5 Dacryocystectomy can be a viable option if the patient is systemically unwell or unwilling for a DCR. In conclusion, although rare, CLL can present initially as acute dacryocystitis. Suspicion should be entertained where the severity of leucocytosis does not correlate with the infection.
ACKNOWLEDGEMENT The authors wish to acknowledge Mr. B. Sreedhar and Mr. G. Chenchu Naidu for their technical help with histopathology.
Dilip K Mishra MD,1 Mohammad J Ali FRCS,2 Archana Bhargava MD3 and Milind N Naik MD2 1 Ocular Pathology Service, 2Dacryology Service, and 3 Department of Internal Medicine, L.V.Prasad Eye Institute, Hyderabad, India Received 26 June 2015; accepted 10 July 2015.
REFERENCES 1. Stokes WH. Dacryocystitis in lymphatic leukemia. Arch Ophthalmol 1938; 20: 85–7. 2. Wirostko WJ, Garcia GH, Cory S, Harris GJ. Acute dacryocystitis as the presenting sign of pediatric leukemia. Am J Ophthalmol 1999; 127: 734–6. 3. Karesh JW, Perman KI, Rodriguez MM. Dacryocystitis associated with malignant lymphoma of the lacrimal sac. Ophthalmology 1992; 100: 669–73. 4. Yip C, Bartley GB, Habermann TM, Garrity JA. Involvement of the lacrimal drainage system by leukemia or lymphoma. Ophthal Plast Reconstr Surg 2002; 18: 242–6. 5. Campbell AA, Jakobiec FA, Rashid A et al. Bilateral sequential dacryocystitis in a patient with graft versus host disease. Ophthal Plast Reconstr Surg 2014; [Epub ahead of print].
Choroidal neovascularization, outer retinal tubulation and fundus autofluorescence findings in a patient with enhanced S-cone syndrome Enhanced S-cone syndrome is a rare hereditary dystrophy, which usually is present with night blindness, variable loss of visual acuity and visual field abnormalities. Adults with the disorder characteristically show nummular pigmentary deposition at the level of the retinal pigment epithelium outside of the vascular arcades. These patients usually exhibit retinal schisis. New imaging techniques, such as spectral-domain optical coherence tomography and fundus autofluorescence, support us to identify and localize retinal lesions, such as retinal schisis or the presence of a hyperautofluorescence ring found in several dystrophies, such as retinitis pigmentosa. We present a case of a 16-year-old woman who came to our service due to nyctalopia. She presented subtle pigmentary changes (Fig. 1a) with foveolar schisis in both eyes (Fig. 1b). Additionally, in the left eye exists an involuted neovascular membrane along with foveal schisis and outer retinal tubulations (Figs 1c,2a). Electroretinography was performed. It displayed a severe reduction in rod function and an enhanced function of the short-wave-length-sensitive cones. Fundus autofluorescence was executed in both eyes. Several fine foci of increased autofluorescence were observed, except in the macular zone. In the left eye appears a hypoautofluorescence in the macula, in which margin multiple fine foci were found generating a ring (Fig. 2b,c). The patient undertook a genetic study exposing a heterozygotic mutation in NR2E3 gene. In the case we present, the left eye demonstrates subretinal fibrosis, a retino-choroidal anastomosis and outer retinal tubulation. These three signs show us that a neovascular membrane occurred and that nowadays it is involuted. Choroidal neovascularization in patients with enhanced S-cone syndrome has been previously reported.1 Outer retinal tubulations are structures generated by photoreceptors. They exist in choroidal neovascularization, subretinal fibrosis or in other entities that produce a severe damage of photoreceptors and pigmented epithelial layers.2 Outer retinal tubulations have been primarily described in age-related macular degeneration. However, they might be observed in retinal dystrophies, such as pattern dystrophy, acute zonal occult outer retinopathy, retinitis pigmentosa or Stargardt disease.3 In regard to fundus autofluorescence findings, we observe several autofluorescent foci. These foci have been formerly described in patients with normal fundus appearance. In the case we present, the lesions found by fundus Conflict of interest: No stated conflict of interest. Funding sources: No stated funding sources.
© 2015 Royal Australian and New Zealand College of Ophthalmologists
67
Letters to the Editor should order IgG4 serology and histological staining in suspected cases. Increasing awareness of this entity may help address clinical ambiguity surrounding the labels ‘idiopathic orbital inflammation’ and ‘reactive hyperplasia of lymph nodes’.
Rajan S Patheja MBBS(Hons), Mahendra Singh FRCPA, James A Bennett MBBS and Timothy J Sullivan FRANZCO Department of Ophthalmology, Royal Brisbane and Women’s Hospital, Brisbane, Queensland, Australia Received 12 June 2015; accepted 23 June 2015.
REFERENCES 1. Sato Y, Inoue D, Asano N et al. Association between IgG4-related disease and progressively transformed germinal centres of lymph nodes. Mod Pathol 2012; 25: 956–67. 2. McNab AA, McKelvie P. IgG4-related ophthalmic disease. Part 1: background and pathology. Ophthal Plast Reconstr Surg 2015; 31: 83–8. 3. Kojima M, Nakamura N, Sakamoto K et al. Progressive transformation of the germinal centre of extranodal organs: a clinicopathological, immunohistochemical, and genotypic study of 14 cases. Pathol Res Pract 2010; 206: 235–40. 4. Ferry JA. IgG4-related lymphadenopathy and IgG4related lymphoma: moving targets. Diagn Histopathol 2013; 19: 128–39. 5. Seki N, Yamazaki N, Koizumi J et al. A case of progressively transformed germinal centre-type IgG4-related lymphadenopathy. Auris Nasus Larynx 2015; e-pub. http://dx.doi.org/10.1016/j.anl.2015.02.010
Acute dacryocystitis as a presenting sign of chronic lymphocytic leukaemia Acute dacryocystitis is characterized by rapid onset of pain and erythematous swelling predominantly below the medial canthus resulting mainly from an acute infection of the lacrimal sac and perisac tissues. Although rare, involvement of the lacrimal drainage system with leukaemic infiltrates were reported and believed to be facilitated by the presence of lacrimal drainage-associated lymphoid tissue (LDALT).1–5 To the best of the authors knowledge, acute dacryocystitis as a presenting feature of a yet to be diagnosed chronic lymphocytic leukaemia (CLL) has been reported once before but with unclear details. We report the second such case which clearly shows acute dacryocystitis as a presenting feature of a CLL. Competing/conflicts of interest: None.
A 59-year-old otherwise healthy female presented with swelling and pain on the inner side of left eye of 5 days duration, associated with discharge and epiphora from the left eye. Constitutional symptoms were absent. There was no history of epiphora, trauma or surgery. On examination, there was tender swelling over the left lacrimal sac area with surrounding induration and preseptal cellulitis (Fig. 1a). Ocular examination was otherwise normal except for bilateral aphakia following cataract surgery. Microbiological cultures were negative. The patient was started on systemic broad spectrum antibiotic and antiinflammatory agents. A week later, there was a suboptimal response (Fig. 1b). Endoscopic evaluation of the nasal cavity was within normal limits (Fig. 1c), and the patient was scheduled for an endoscopic dacryocystorhinostomy. Routine pre-surgical blood counts showed marked leukocytosis (Total leucocyte count – 73 100/u˛l) with lymphocytosis without any anaemia or thrombocytopenia. A peripheral blood smear showed similar picture (Fig. 1d). Bone marrow biopsy showed marked lymphocytosis with predominantly monotonous population of small lymphocytes (Fig. 2a). Flow cytometric analysis showed 60% of atypical B-lymphocytes with bright expression of CD19 and HLADR with moderate expression of CD23, CD200 and CD45. The CD19-gated cells were 0.4% positive for CD38 and 1.9% positive for ZAP-70. The scatter parameters and antigenic expression profile by flow cytometry (Fig. 2b) were suggestive of B-cell chronic lymphocytic leukaemia (B-CLL). Systemic examination did not reveal any lymphadenopathy, hepatomegaly or splenomegaly. The B-CLL was staged as Rai Stage 0 and Binet Stage I with a favourable prognosis. In view of the early stage of the disease, the patient is being closely monitored on a 2-weekly basis. The patient complained of increasing pain on the left side again after 2 weeks and underwent a dacryocystectomy. Surgery was uneventful except for increased intraoperative bleeding. At the last follow-up (3 months following the surgery), the patient was asymptomatic with resolution of pain and swelling. Histopathological examination revealed dense subepithelial lymphocytic infiltrates (Fig. 2c). Immunohistochemistry showed the cells to be positive to CD20 (Fig. 2d), CD5 (Fig. 2e) and CD23 (Fig. 2f). The histological features were consistent with predominantly B-lymphocytic infiltrate of the lacrimal mucosal tissues. Chronic lymphocytic leukaemia is a common form of leukaemia in adults usually suspected when blood counts show marked lymphocytosis and is confirmed by flow cytometry and target gene analysis. Lacrimal drainage obstructions and subsequent acute or chronic dacryocystitis are believed to be secondary to either a direct leukaemic infiltration or an abnormal stimulation and subsequent overgrowth of pre-existing lymphoid elements.1,4 Wirostko et al.2 documented acute dacryocystitis as a presenting sign of acute leukaemia in pediatric age group, which was successfully managed with antibiotics and chemotherapy. Stokes1 reported a case of bilateral chronic dacryocystitis with a fistula in a 68-year-old woman, where CLL was diagnosed on histological examinations of
Funding sources: None.
© 2015 Royal Australian and New Zealand College of Ophthalmologists
68 Figure 1. External photograph of the patient showing gross leftsided medial canthal swelling with preseptal cellulitis (a). Suboptimal clinical response at 1 week (b). Endoscopic view of the left nasal cavity showing normal lateral wall (c). Microphotograph of the peripheral smear showing lymphocytosis (Lieshman x100, d).
Figure 2. Microphotograph of the bone marrow aspiration showing marked lymphocytosis (Giemsa x400, a). Scatter parameters and antigenic expression profile of flow cytometry (b). Microphotograph of the lacrimal sac showing dense sub-epithelial lymphocytic infiltrates (H&E x100, c). Immunohistochemistry microphotographs of the lacrimal sac showing positivity to CD20 (CD20 x100, d), CD5 (CD5 x100, Fig. 2e) and CD23 (CD23 x400, f).
Letters to the Editor
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Letters to the Editor lacrimal sac following a dacryocystectomy. However, this patient had a history of previous repeated attacks of acute dacryocystitis and lacrimal abscess. One case of acute dacryocystitis in a 69-year-old man, following eight cycles of chemotherapy for CLL was reported by Karesh et al.3 The patient was treated by antibiotics followed by a dacryocystorhinostomy (DCR). Chemotherapy was initiated after surgery and the patient was symptom free at 17 months follow-up. Yip et al.4 reported a case of 84-year-old woman, primarily treated by chemotherapy for CLL and presented with acute dacryocystitis 2 years later, which was successfully managed with a dacryocystorhinostomy. Management options for acute dacryocystitis in a leukaemic patient include systemic antibiotics, chemotherapy when indicated, dacryocystorhinostomy or a dacryocystectomy. DCR is a preferred surgical option. Yip et al.4 in their series of CLL, showed successful outcomes in seven of the nine patients (one had acute dacryocystitis). However, restenosis secondary to lymphocytic infiltrations after successful DCR surgeries and dry eyes secondary to graft versus host disease have also been reported.5 Dacryocystectomy can be a viable option if the patient is systemically unwell or unwilling for a DCR. In conclusion, although rare, CLL can present initially as acute dacryocystitis. Suspicion should be entertained where the severity of leucocytosis does not correlate with the infection.
ACKNOWLEDGEMENT The authors wish to acknowledge Mr. B. Sreedhar and Mr. G. Chenchu Naidu for their technical help with histopathology.
Dilip K Mishra MD,1 Mohammad J Ali FRCS,2 Archana Bhargava MD3 and Milind N Naik MD2 1 Ocular Pathology Service, 2Dacryology Service, and 3 Department of Internal Medicine, L.V.Prasad Eye Institute, Hyderabad, India Received 26 June 2015; accepted 10 July 2015.
REFERENCES 1. Stokes WH. Dacryocystitis in lymphatic leukemia. Arch Ophthalmol 1938; 20: 85–7. 2. Wirostko WJ, Garcia GH, Cory S, Harris GJ. Acute dacryocystitis as the presenting sign of pediatric leukemia. Am J Ophthalmol 1999; 127: 734–6. 3. Karesh JW, Perman KI, Rodriguez MM. Dacryocystitis associated with malignant lymphoma of the lacrimal sac. Ophthalmology 1992; 100: 669–73. 4. Yip C, Bartley GB, Habermann TM, Garrity JA. Involvement of the lacrimal drainage system by leukemia or lymphoma. Ophthal Plast Reconstr Surg 2002; 18: 242–6. 5. Campbell AA, Jakobiec FA, Rashid A et al. Bilateral sequential dacryocystitis in a patient with graft versus host disease. Ophthal Plast Reconstr Surg 2014; [Epub ahead of print].
Choroidal neovascularization, outer retinal tubulation and fundus autofluorescence findings in a patient with enhanced S-cone syndrome Enhanced S-cone syndrome is a rare hereditary dystrophy, which usually is present with night blindness, variable loss of visual acuity and visual field abnormalities. Adults with the disorder characteristically show nummular pigmentary deposition at the level of the retinal pigment epithelium outside of the vascular arcades. These patients usually exhibit retinal schisis. New imaging techniques, such as spectral-domain optical coherence tomography and fundus autofluorescence, support us to identify and localize retinal lesions, such as retinal schisis or the presence of a hyperautofluorescence ring found in several dystrophies, such as retinitis pigmentosa. We present a case of a 16-year-old woman who came to our service due to nyctalopia. She presented subtle pigmentary changes (Fig. 1a) with foveolar schisis in both eyes (Fig. 1b). Additionally, in the left eye exists an involuted neovascular membrane along with foveal schisis and outer retinal tubulations (Figs 1c,2a). Electroretinography was performed. It displayed a severe reduction in rod function and an enhanced function of the short-wave-length-sensitive cones. Fundus autofluorescence was executed in both eyes. Several fine foci of increased autofluorescence were observed, except in the macular zone. In the left eye appears a hypoautofluorescence in the macula, in which margin multiple fine foci were found generating a ring (Fig. 2b,c). The patient undertook a genetic study exposing a heterozygotic mutation in NR2E3 gene. In the case we present, the left eye demonstrates subretinal fibrosis, a retino-choroidal anastomosis and outer retinal tubulation. These three signs show us that a neovascular membrane occurred and that nowadays it is involuted. Choroidal neovascularization in patients with enhanced S-cone syndrome has been previously reported.1 Outer retinal tubulations are structures generated by photoreceptors. They exist in choroidal neovascularization, subretinal fibrosis or in other entities that produce a severe damage of photoreceptors and pigmented epithelial layers.2 Outer retinal tubulations have been primarily described in age-related macular degeneration. However, they might be observed in retinal dystrophies, such as pattern dystrophy, acute zonal occult outer retinopathy, retinitis pigmentosa or Stargardt disease.3 In regard to fundus autofluorescence findings, we observe several autofluorescent foci. These foci have been formerly described in patients with normal fundus appearance. In the case we present, the lesions found by fundus Conflict of interest: No stated conflict of interest. Funding sources: No stated funding sources.
© 2015 Royal Australian and New Zealand College of Ophthalmologists
