A Case of Common Variable Immunodeficiency with Autoimmune Hemolytic Anemia

Common variable immunodeficiency (CVID) is a heterogeneous collection of disorders with hypogammaglobulinemia with recurrent bacterial infections and high incidence of autoimmune disorders as its hallmark. We report a 7-year-old girl suffering from CVID with Coombs' test positive hemolytic anemia. She had been relatively well until 23-months old when she was admitted to Taejon St. Mary's Hospital with pneumonia 5 years ago. Afterwards, she had suffered from recurrent otitis media, paranasal sinusitis, bronchitis and pneumonia, experiencing 13 admissions. She was diagnosed as autoimmune hemolytic anemia at 4-years old and had been treated with prednisolone. Laboratory finidings showed hypogammaglobulinemia(gamma-globulin in immunoelectrophoresis 0.04g/dL, IgG 170mg/dL, IgA 31mg/dL, IgM 27.5mg/dL) which was previously within normal limits checked at the age of 3- and 5-years old. Isohemmagglutinins (Anti-A,-B IgM and IgG) and anti-measles IgG, anti-mumps IgG, anti-rubella IgG and anti-HBs antibody along with PPD skin test were all negative. Peripheral lymphocyte subsets revealed as follows : pan T cells (CD3+) 48.6% (normal values : 60-85%), pan B cells (CD19+) 36.7% (8-20%), CD4+ T cells 24.4% (28+/-8%), CD8+ T cells 15.3% (5+/-10%), and CD4/CD8 ratio of 1.6 (0.6-2.8). Proliferations of peripheral blood mononuclear cells induced by various T cell stimulants were all markedly depressed. Chronic paranasal sinusitis and lung parenchymal damages were revealed on computerized tomography and lung scan, and a monthly intravenous immunoglobulin therapy was started.

A Case of Acute Drug-Induced Agranulocytosis Treated with Recombinant Granulocyte Colony-Stimulating Factor(G-CSF)

Drug-induced agranulocytosis is a potentially lethal disorder characterized by selective neutropenia. G-CSF has been utilized for its treatment. We report a case of acute agranulocytosis probably associated with injection of sulpyrine(dipyrone). A three-year old girl was admitted to Taejon St. Mary's Hospital following five days of fever and two days of chills and prostration. During this period, she had been treated at local clinics with oral acetaminophen, ibuprofen, and miokamycin. Two days before admission, she was administered an intramuscular injection of dipyrone as antipyretics. She had a past history of previous sensitization of dipyrone. CBC revealed profound netropenia(total WBC 900/mm3, with 1% neutrophils, 88% lymphocytes, 10% atypical lymphocytes, 1% monocytes), but normal RBC and platelet count. Bone marrow examination showed hypocellularity(20%), decreased myeloid precusors, and M:E ratio of 1 : 2.5. The girl received subcutaneous G-CSF once daily for 3 days. G-CSF therapy resulted in a steep increase of neutrophil count, which was faster than the spontaneous recovery reported in the literature. G-CSF may be considered useful in the management of drug-induced agranulocytosis.

A Case of Acute Drug-Induced Agranulocytosis Treated with Recombinant Granulocyte Colony-Stimulating Factor(G-CSF)

Drug-induced agranulocytosis is a potentially lethal disorder characterized by selective neutropenia. G-CSF has been utilized for its treatment. We report a case of acute agranulocytosis probably associated with injection of sulpyrine(dipyrone). A three-year old girl was admitted to Taejon St. Mary's Hospital following five days of fever and two days of chills and prostration. During this period, she had been treated at local clinics with oral acetaminophen, ibuprofen, and miokamycin. Two days before admission, she was administered an intramuscular injection of dipyrone as antipyretics. She had a past history of previous sensitization of dipyrone. CBC revealed profound netropenia(total WBC 900/mm3, with 1% neutrophils, 88% lymphocytes, 10% atypical lymphocytes, 1% monocytes), but normal RBC and platelet count. Bone marrow examination showed hypocellularity(20%), decreased myeloid precusors, and M:E ratio of 1 : 2.5. The girl received subcutaneous G-CSF once daily for 3 days. G-CSF therapy resulted in a steep increase of neutrophil count, which was faster than the spontaneous recovery reported in the literature. G-CSF may be considered useful in the management of drug-induced agranulocytosis.