Natural killer cell lymphoproliferative disorder of granular lymphocytes presenting as hemolytic anemia: case report and review of the literature.
Lymphoproliferative disorders of granular lymphocytes (LDGLs) have recently been hypothetically defined based on their immunophenotype, function, and clonality. The majority (129/161) of LDGLs are of the T-cell subset, which is typically associated with neutropenia and occasionally with anemia. The remainder (32/161) are of natural killer cell origin. This subset is associated with neutropenia (52% of cases) and anemia (60% of cases). The anemia most often has no immediately apparent cause. The Coombs test was negative in all cases reported. There is only one reported case of an associated Coombs-negative hemolytic anemia. We report an unusual case of natural killer cell LDGL, defined by morphology and immunophenotypic analysis, presenting as a de novo Coombs-negative hemolytic anemia. The anemia was resistant to steroid therapy. Therapeutic splenectomy resulted in a temporary resolution of the hemolytic process. The splenic pathology is also described.
Dunphy
C H
CH
Department of Pathology, St. Louis University School of Medicine, Missouri 63104, USA.
Velasquez
W S
WS
Morris
R W
RW
Smith
J
J
D000368:Aged; D000369:Aged, 80 and over; D000743:Anemia, Hemolytic / Q000150:complications* / Q000175:diagnosis; D001853:Bone Marrow / Q000473:pathology; D003298:Coombs Test; D003594:Cytoplasmic Granules; D004273:DNA, Neoplasm / Q000235:genetics; D005434:Flow Cytometry; D015321:Gene Rearrangement; D006098:Granulocytes; D006801:Humans; D007694:Killer Cells, Natural / Q000166:cytology* / Q000276:immunology; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000145:classification* / Q000150:complications* / Q000276:immunology; D008297:Male; D008853:Microscopy; D013154:Spleen / Q000473:pathology; D013156:Splenectomy; D044465:White People
D004273:DNA, Neoplasm