A case of de novo B - cell prolymphocytic leukemia associated with false - positive direct antiglobulin test / 대한내과학회지

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.

A Case of Dyskeratosis Congenita with Myelodysplastic Syndrome / 대한혈액학회지

Dyskeratosis congenita is a rare form of ectodermal dysplasia consisting of dystrophic nails, reticular hyperpigmentation and leukoplakia, that is often associated with aplastic anemia. We have experienced a 17 year-old-man who had reticular pigmentation of the skin and dystrophic changes of the fingers and toe nails. The tongue was smooth and lingual papillae disappeared with formation of adherent white leukoplakic patches. Laboratory data revealed pancytopenia. Bone marrow study showed mild hypocellular marrow with dyserythropoiesis, suggesting the refractory anemia of myelodysplastic syndrome. We report one case of dyskeratosis congenita with myelodysplastic syndrome with a review of literature.

A case of autoimmune neutropenia / 대한내과학회지

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.

The Effect of Halofantrine in the Treatment of 14 Cases of Chloroquine Resistant Imported Malaria / 대한내과학회지

OBJECTIVES: The prevalence of malaria is increasing in recent years and also multidrug resistant malaria is increasing around the world and there is an increasing concern about imported malaria in nonendemic areas. Now many drugs are tried to find out effect on multidrug resistant malaria. We performed this study to investigate the thrapeutic effect of halofantrine in the treatment of chloroquine resistant imported malaria. METHODS: From Feb. 1992 to May 1995, we experienced 35 patients infected with malaria and treated 14 patients among 35 patients with halofantrine. RESULTS: 1) All 14 patients were sailor with a mean age of 39.4 years and infected with malaria. 2) The majority of patients were infected with malaria at Africa. 3) 10 patients were infected with Plasmodium falciparum and the remainder were undetermined. 4) In the 11 cases of chloroquine resistant malaria treated with quinine plus tetracycline combination therapy or Fansidar, 4 cases could not be tolerable due to side effects and resistance to the therapy, we substituted halofantrine for above regimens. 5) In the 10 cases, treated after May 1994, halofantrine was the first choice of treatment because they were the cases of malaria infected in the mid-Africa where the prevalence of chloroquine resistant malaria is high. 6) With halofantrine, all 14 cases were treated with minimal side effects suc4 as nausea, vomiting, anorexia, abdominal pain and fatigue. CONCLUSION: We think halofantrine is a simple and effective regimen against chloroquine resistant malaria and consider this agent as an alternative therapeutic regimen on chloroquine resistant malaria.