ABSTRACT
De novo B-cell prolymphocytic leukemia (B-PLL) is a distinct clinicopathologic entity that was first described in 1974 by Galton et al. B-PLL is characterized by marked lymphocytosis with predominance of prolymphocytes, often massive splenomegaly, minimal lymphadenopathy, often aggressive clinical course and frequently poor prognosis.We experienced a case of B-PLL associated with false-positive direct antiglobulin test. The patient was 52 year-old man who presented with marked leukocytosis (160.2x103/(mu)L) and 60% of characteristic prolymphocytes in the peripheral blood. The bone marrow aspirate showed dry tap and the PAS stain of peripheral blood smear showed positivity of prolymphocytes. The immunophenotyping of the leukemic prolymphocytes revealed the positivity of surface immunoglobulin (IgM, lambda type), HLA-DR, CD19 and CD5.
Subject(s)
Humans , Middle Aged , Bone Marrow , Coombs Test , HLA-DR Antigens , Immunoglobulins , Immunophenotyping , Leukemia , Leukemia, Prolymphocytic , Leukemia, Prolymphocytic, B-Cell , Leukocytosis , Lymphatic Diseases , Lymphocytosis , SplenomegalyABSTRACT
Autoimmune neutropenia is characterized by severe neutropenia with cell-bound neutrophil antibodies or circulating antibodies for neutrophils. Diagnosis of disease is made of the basis of the presence of antibodies for neutrophil. Corticosteroid or rhG-CSF have been reported to be effective in some patients. Recently we experienced one case of autoimmune neutropenia patient who was admitted to our hospital in 1997 because of stomach cancer and degenerative joint disease. She had severe neutropenia without underlying autoimmune disease. And cell-bound neutrophil antibodies were detected by indirect immunofluorescence test. Treatments with rhG-CSF and steroid result in transient improvement of neutropenia and subtotal gastrectomy was done successfully. We herein report one case of autoimmune neutro- penia patient, to our best knowledge, the first report in Korea, with a brief review of literature.