Conjunctival involvement with T-cell prolymphocytic leukemia: report of a case and review of the literature.
T-cell prolymphocytic leukemia is a rare and highly aggressive hematological neoplasm. A patient with T-cell prolymphocytic leukemia presented with bilateral perilimbal conjunctival infiltrates. Conjunctival biopsy showed aggregates of large atypical lymphocytes in the substantia propria with a concentration of atypical cells in the perivascular areas. Immunophenotyping of the malignant cells identified an abnormal clonal T-cell population consistent with T-cell prolymphocytic leukemia. A literature review of all reports of conjunctival involvement with leukemia was performed. The three cases of ocular prolymphocytic leukemia, including the one case of ocular T-cell prolymphocytic leukemia, are discussed in detail as well as 14 reported clinical cases of biopsy-proven conjunctival leukemia. The majority of cases occurred in the setting of acute leukemia, and conjunctival involvement was frequently a presenting sign of the disease or signified disease relapse. Conjunctival involvement with leukemia was consistent with good visual acuity; however, it portended a poor prognosis.
National Eye Institute, NIH, Bethesda, Maryland 20892-1863, USA.
Susan S
SS
Lee
Michael R
MR
Robinson
John C
JC
Morris
Barry C
BC
Mirtsching
DeFen
D
Shen
Chi-Chao
CC
Chan
D000074323:Alemtuzumab; D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized; D000912:Antibodies, Neoplasm / Q000627:therapeutic use; D015703:Antigens, CD / Q000378:metabolism; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001706:Biopsy; D003228:Conjunctiva / Q000473:pathology*; D017809:Fatal Outcome; D006801:Humans; D015463:Leukemia, Prolymphocytic / Q000175:diagnosis* / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology; D015458:Leukemia, T-Cell / Q000175:diagnosis* / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D015649:Pentostatin / Q000627:therapeutic use; D019233:Retreatment
D000903:Antibiotics, Antineoplastic; D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D000912:Antibodies, Neoplasm; D015703:Antigens, CD; D000970:Antineoplastic Agents; D015649:Pentostatin; D000074323:Alemtuzumab