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Ocular pathology of uncommon hematologic malignancies: a case series.

Head JE, Shen D, Santiago-Maysonet M, Bishop RJ, Chan CC - J Med Case Rep (2007)

Bottom Line: In the second case, a 51-year-old man with chronic myelomonocytic leukemia died after a waxing and waning clinical course.Post-mortem studies revealed the presence of atypical monocytes in the choroidal and subretinal spaces, consistent with his previous diagnosis.We herein emphasize novel findings in each case, including conjunctival involvement as the first sign of recurrent lymphomatoid granulomatosis and the combination of subretinal and choroidal myelomonocytic leukemic infiltration.

View Article: PubMed Central - HTML - PubMed

Affiliation: Immunopathology Section, National Institutes of Health, Bethesda, MD, USA. chanc@nei.nih.gov.

ABSTRACT

Introduction: In general, ocular complications of hematologic malignancies such as leukemia are well documented. However, reports of ocular involvement in such diseases as lymphomatoid granulomatosis and chronic myelomonocytic leukemia are uncommon. Here we present cases of these two relatively rare hematologic malignancies demonstrating clinical and subclinical ocular involvement.

Case presentation: In the first case, a 54-year-old man with a previous diagnosis of lymphomatoid granulomatosis presented with a new-onset conjunctival lesion while his systemic disease was thought to be in remission. A biopsy was taken that revealed heavy infiltrates of B and T cells at the site of the lesion. Molecular analysis confirmed that these cells were positive for both Epstein-Barr viral DNA and immunoglobulin heavy chain gene rearrangement, consistent with a manifestation of his systemic disease. In the second case, a 51-year-old man with chronic myelomonocytic leukemia died after a waxing and waning clinical course. Post-mortem studies revealed the presence of atypical monocytes in the choroidal and subretinal spaces, consistent with his previous diagnosis.

Conclusion: While ocular involvement in hematologic malignancies is not uncommon, these two cases describe involvement of the eye by two relatively rare neoplasms. We herein emphasize novel findings in each case, including conjunctival involvement as the first sign of recurrent lymphomatoid granulomatosis and the combination of subretinal and choroidal myelomonocytic leukemic infiltration. With the evolution of new antineoplastic therapies that may prolong life, these cases exemplify the importance of eye care in patients diagnosed with hematologic malignancies.

No MeSH data available.


Related in: MedlinePlus

Case 1. (A) Generalized conjunctival injection, chemosis and a prominent lesion with elevated, polygonal, hyperemic mounds and small hemorrhages are seen in the superior bulbar conjunctiva. (B) A dense infiltration of cells and several foci of necrosis are present, as is hemorrhage into the conjunctival parenchyma. The inset demonstrates pleomorphism and prominence of nucleoli. (C) Immunohistochemistry demonstrates strongly immunopositive staining for B-cell marker, CD20. (D) Gel electrophoresis reveals: (lane 1) positive bands are indicative of IgH gene rearrangements for B-cell lymphoma. EBV DNA is detected in the lymphoma cells. (Lane 3 = negative control, lane 4 = positive control). (B, hematoxylin & eosin, original magnification × 100; B (inset), original magnification × 400; C, avidin-biotin-complex immunoperoxidase, original magnification × 200).
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Figure 1: Case 1. (A) Generalized conjunctival injection, chemosis and a prominent lesion with elevated, polygonal, hyperemic mounds and small hemorrhages are seen in the superior bulbar conjunctiva. (B) A dense infiltration of cells and several foci of necrosis are present, as is hemorrhage into the conjunctival parenchyma. The inset demonstrates pleomorphism and prominence of nucleoli. (C) Immunohistochemistry demonstrates strongly immunopositive staining for B-cell marker, CD20. (D) Gel electrophoresis reveals: (lane 1) positive bands are indicative of IgH gene rearrangements for B-cell lymphoma. EBV DNA is detected in the lymphoma cells. (Lane 3 = negative control, lane 4 = positive control). (B, hematoxylin & eosin, original magnification × 100; B (inset), original magnification × 400; C, avidin-biotin-complex immunoperoxidase, original magnification × 200).

Mentions: A 54 year-old man with a previous diagnosis of lymphomatoid granulomatosis (LYG) presented with a left conjunctival growth consisting of clusters of papillae with focal hemorrhages of several weeks' duration (Figure 1A), during which time the systemic disease was thought to be in remission. The lesion was subsequently biopsied (Figure 1B). Given the patient's clinical history and the dense infiltrate of pleomorphic lymphoid cells in the tissue, immunohistochemistry was performed using antibodies against B-cell (CD20; Figure 1C), T-cell (CD45R0), and macrophage (CD68) markers. B-cell monoclonality was demonstrated using primer pairs FR3A, FR2A, and CDR3 for immunoglobulin heavy chain (IgH) regions [3], while PCR showed the presence of Epstein-Barr virus (EBV) DNA in the cells comprising the inflammatory infiltrate (Figure 1D). Based on the predominance of B- and T-cell infiltrates in the lesion, positive IgH rearrangement, and the presence of EBV DNA by PCR analysis, the patient's underlying disease was considered as a potential cause. He received palliative radiotherapy to the orbit.


Ocular pathology of uncommon hematologic malignancies: a case series.

Head JE, Shen D, Santiago-Maysonet M, Bishop RJ, Chan CC - J Med Case Rep (2007)

Case 1. (A) Generalized conjunctival injection, chemosis and a prominent lesion with elevated, polygonal, hyperemic mounds and small hemorrhages are seen in the superior bulbar conjunctiva. (B) A dense infiltration of cells and several foci of necrosis are present, as is hemorrhage into the conjunctival parenchyma. The inset demonstrates pleomorphism and prominence of nucleoli. (C) Immunohistochemistry demonstrates strongly immunopositive staining for B-cell marker, CD20. (D) Gel electrophoresis reveals: (lane 1) positive bands are indicative of IgH gene rearrangements for B-cell lymphoma. EBV DNA is detected in the lymphoma cells. (Lane 3 = negative control, lane 4 = positive control). (B, hematoxylin & eosin, original magnification × 100; B (inset), original magnification × 400; C, avidin-biotin-complex immunoperoxidase, original magnification × 200).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2211492&req=5

Figure 1: Case 1. (A) Generalized conjunctival injection, chemosis and a prominent lesion with elevated, polygonal, hyperemic mounds and small hemorrhages are seen in the superior bulbar conjunctiva. (B) A dense infiltration of cells and several foci of necrosis are present, as is hemorrhage into the conjunctival parenchyma. The inset demonstrates pleomorphism and prominence of nucleoli. (C) Immunohistochemistry demonstrates strongly immunopositive staining for B-cell marker, CD20. (D) Gel electrophoresis reveals: (lane 1) positive bands are indicative of IgH gene rearrangements for B-cell lymphoma. EBV DNA is detected in the lymphoma cells. (Lane 3 = negative control, lane 4 = positive control). (B, hematoxylin & eosin, original magnification × 100; B (inset), original magnification × 400; C, avidin-biotin-complex immunoperoxidase, original magnification × 200).
Mentions: A 54 year-old man with a previous diagnosis of lymphomatoid granulomatosis (LYG) presented with a left conjunctival growth consisting of clusters of papillae with focal hemorrhages of several weeks' duration (Figure 1A), during which time the systemic disease was thought to be in remission. The lesion was subsequently biopsied (Figure 1B). Given the patient's clinical history and the dense infiltrate of pleomorphic lymphoid cells in the tissue, immunohistochemistry was performed using antibodies against B-cell (CD20; Figure 1C), T-cell (CD45R0), and macrophage (CD68) markers. B-cell monoclonality was demonstrated using primer pairs FR3A, FR2A, and CDR3 for immunoglobulin heavy chain (IgH) regions [3], while PCR showed the presence of Epstein-Barr virus (EBV) DNA in the cells comprising the inflammatory infiltrate (Figure 1D). Based on the predominance of B- and T-cell infiltrates in the lesion, positive IgH rearrangement, and the presence of EBV DNA by PCR analysis, the patient's underlying disease was considered as a potential cause. He received palliative radiotherapy to the orbit.

Bottom Line: In the second case, a 51-year-old man with chronic myelomonocytic leukemia died after a waxing and waning clinical course.Post-mortem studies revealed the presence of atypical monocytes in the choroidal and subretinal spaces, consistent with his previous diagnosis.We herein emphasize novel findings in each case, including conjunctival involvement as the first sign of recurrent lymphomatoid granulomatosis and the combination of subretinal and choroidal myelomonocytic leukemic infiltration.

View Article: PubMed Central - HTML - PubMed

Affiliation: Immunopathology Section, National Institutes of Health, Bethesda, MD, USA. chanc@nei.nih.gov.

ABSTRACT

Introduction: In general, ocular complications of hematologic malignancies such as leukemia are well documented. However, reports of ocular involvement in such diseases as lymphomatoid granulomatosis and chronic myelomonocytic leukemia are uncommon. Here we present cases of these two relatively rare hematologic malignancies demonstrating clinical and subclinical ocular involvement.

Case presentation: In the first case, a 54-year-old man with a previous diagnosis of lymphomatoid granulomatosis presented with a new-onset conjunctival lesion while his systemic disease was thought to be in remission. A biopsy was taken that revealed heavy infiltrates of B and T cells at the site of the lesion. Molecular analysis confirmed that these cells were positive for both Epstein-Barr viral DNA and immunoglobulin heavy chain gene rearrangement, consistent with a manifestation of his systemic disease. In the second case, a 51-year-old man with chronic myelomonocytic leukemia died after a waxing and waning clinical course. Post-mortem studies revealed the presence of atypical monocytes in the choroidal and subretinal spaces, consistent with his previous diagnosis.

Conclusion: While ocular involvement in hematologic malignancies is not uncommon, these two cases describe involvement of the eye by two relatively rare neoplasms. We herein emphasize novel findings in each case, including conjunctival involvement as the first sign of recurrent lymphomatoid granulomatosis and the combination of subretinal and choroidal myelomonocytic leukemic infiltration. With the evolution of new antineoplastic therapies that may prolong life, these cases exemplify the importance of eye care in patients diagnosed with hematologic malignancies.

No MeSH data available.


Related in: MedlinePlus