The spectrum of human immunodeficiency virus infection in patients with factor IX deficiency (Christmas Disease)

Early reports suggested that hemophiliacs with factor IX deficiency (Christmas Disease) may be at less risk for developing the acquired immunodeficiency syndrome (AIDS) than patients with classic hemophilia. We evaluated 12 factor IX deficient patients for clinical and immunologic abnormalities related to infection with the human immunodeficiency virus (HIV). Antibody to HIV was not detected in these patients prior to 1982. By 1985, 66 percent (eight of 12) patients were seropositive. All three concentrates available commercially before 1985 were associated with seropositivity. Furthermore, seropositive hemophiliacs had received on average significantly more factor IX concentrate than seronegative hemophiliacs (27,825 ± 17,976 (S.D.) versus 1,250 ± 1,500 factor units/year, (p < 0.02). Half of the seropositive individuals had generalized lymphadenopathy with splenomegaly. Two seropositive patients have developed AIDS, one with cryptococcal meningitis and another with a large cell immunoblastic lymphoma. Infection with HIV has occurred with high frequency in hemophiliacs who received unmodified factor IX concentrates.

[1]  M. Buti,et al.  HTLV-III ANTIBODIES AND CHRISTMAS DISEASE , 1986, The Lancet.

[2]  J. Goudsmit,et al.  SEROCONVERSION TO HTLV-III IN HAEMOPHILIAC GIVEN HEAT-TREATED FACTOR VIII CONCENTRATE , 1986, The Lancet.

[3]  R. Redfield,et al.  Defective regulation of Epstein-Barr virus infection in patients with acquired immunodeficiency syndrome (AIDS) or AIDS-related disorders. , 1986, The New England journal of medicine.

[4]  D. Bolognesi,et al.  HTLV-III SEROCONVERSION ASSOCIATED WITH HEAT-TREATED FACTOR VIII CONCENTRATE , 1986, The Lancet.

[5]  J. Levy,et al.  AIDS retrovirus antibodies in hemophiliacs treated with factor VIII or factor IX concentrates, cryoprecipitate, or fresh frozen plasma: prevalence, seroconversion rate, and clinical correlations. , 1986, Blood.

[6]  H. Prince,et al.  Human T cell leukemia virus type III antibody, lymphadenopathy, and acquired immune deficiency syndrome in hemophiliac subjects. Results of a prospective study. , 1986, The American journal of medicine.

[7]  H. Ioachim,et al.  LYMPHOMAS OF AIDS , 1986, The Lancet.

[8]  M. Ollero,et al.  Antibodies to human T-cell leukemia virus type III in hemophiliacs from Spain. , 1985, Blood.

[9]  J. Lewis,et al.  Lymphoma presenting as a traumatic hematoma in an HTLV-III antibody-positive hemophiliac. , 1985, The New England journal of medicine.

[10]  B. Evatt,et al.  Thermal inactivation of the acquired immunodeficiency syndrome virus, human T lymphotropic virus-III/lymphadenopathy-associated virus, with special reference to antihemophilic factor. , 1985, The Journal of clinical investigation.

[11]  R. Holman,et al.  Human T-Lymphotropic Retrovirus Type III / Lymphadenopathy-Associated Virus Antibody: Association With Hemophiliacs' Immune Status and Blood Component Usage , 1985 .

[12]  G. Wood,et al.  The varied histopathology of lymphadenopathy in the homosexual male. , 1985 .

[13]  B. Evatt,et al.  Coincidental appearance of LAV/HTLV-III antibodies in hemophiliacs and the onset of the AIDS epidemic. , 1985, The New England journal of medicine.

[14]  Wormser Gp,et al.  Seborrheic dermatitis and butterfly rash in AIDS. , 1984 .

[15]  B. Safai,et al.  SEROEPIDEMIOLOGICAL STUDIES OF HUMAN T-LYMPHOTROPIC RETROVIRUS TYPE III IN ACQUIRED IMMUNODEFICIENCY SYNDROME , 1984, The Lancet.

[16]  D. Lawrence,et al.  The acquired immunodeficiency syndrome in patients with hemophilia. , 1984, Annals of internal medicine.

[17]  J. Chermann,et al.  Isolation of a T-lymphotropic retrovirus from a patient at risk for acquired immune deficiency syndrome (AIDS). , 1983, Science.

[18]  B. A. Lond,et al.  Andrew Moynihan Claye. , 1977, Lancet.

[19]  Miller Ne,et al.  Letter: High-density lipoprotein and atherosclerosis. , 1975, Lancet.

[20]  C. Karakousis.  CANCER : FAILURE OF LOCALISATION OF THE IMMUNE SYSTEM? , 1975, The Lancet.

[21]  Yasargil Ec.  Prescribing in General Practice , 1899 .

[22]  V. Tsang,et al.  Enzyme-linked immunoelectrotransfer blot techniques (EITB) for studying the specificities of antigens and antibodies separated by gel electrophoresis. , 1983, Methods in enzymology.