A Prospective Study of HIV-Infected Haemophilic Boys and the Prognostic Significance of Immune and Haematological Abnormalities

Summary

Fifty-seven haemophilic boys have been studied prospectively determining T lymphocyte subsets and haematological parameters over a period of 26 months. Clinical follow up of these patients has continued for a further 41 months. At the beginning of the study 21 boys were HIV antibody seropositive and seroconversion was documented in a further 9 during the study period, giving a 52.6% incidence of HIV seropositivity. It has been possible to define a group of patients with persistently falling T4 values and these have shown clinical evidence of HIV infection. Although persistent generalized lymphadenopathy has been observed in 30% of HIV antibody positive boys and some boys have had unexplained fevers, only one boy has progressed to have the acquired immune deficiency syndrome (AIDS) some 5 years and 3 months from HIV seroconversion. This boy had a persistently falling T4 value. The importance of these findings is discussed. To date the abnormalities found in these haemophilic boys irrespective of their age are similar to those already reported in adult haemophiliacs.

Key words

Haemophilia - T lymphocytes - HIV-III infection

• References

• 1 Centers of Disease Control. Pneumocystis carinii pneumonia amongst persons with Hemophilia A. MMWR 1982; 31: 365-367
• 2 Gottlieb MS, Schroff R, Schanker HM, Weisman JD, Fan PT, Wolf RA, Saxon A. Pneumocystis carinii pneumonia and mucosal candidiasis in previously healthy homosexual men: evidence of a new acquired cellular immunodeficiency. N Engl J Med 1981; 305: 1425-1431
• 3 Masur H, Michelis MA, Greene JB, Onorato I, Van de Stoune RA, Holzman RS, Wormser G, Brettman L, Lange M, Murray HW, Cunningham-Rundles S. An outbreak of community-acquired Pneumocystis carinii pneumonia: initial manifestation of cellular immune dysfunction. N Engl J Med 1981; 305: 1431-1438
• 4 Blattner WA, Biggar RJ, Weiss SH, Melbye M, Goedert JJ. Epidemiology of human T-lymphotropic virus type III and the risk of the Acquired Immuno Deficiency Syndrome. Ann Intern Med 1985; 103: 665-670
• 5 Eyster ME, Gail MH, Ballard JO, Al-Mondhiry H, Goedert JJ. Natural history of human immunodeficiency virus infections in hemophiliacs: effects of T-cell subsets, platelet counts and age. Ann Intern Med 1987; 107: 1-6
• 6 Ekert H. AIDS incubation period. Nature 1987; 329: 494
• 7 Beddall AC, Hill FG H, George RH, Williams MD, Al-Rubei K. Unusually high incidence of tuberculosis among boys with haemophilia during an outbreak of the disease in hospital. J Clin Pathol 1985; 38: 1163-1165
• 8 Gallo RC, Salahuddin SZ, Popovic M, Shearer GM, Kaplan M, Haynes BF, Palker TJ, Redfield R, Oleske J, Safai B, White G, Foster P, Markham PD. Frequent detection and isolation of cytopathic retroviruses (HTLV-III) from patients with AIDS and at risk for AIDS. Science 1984; 224: 500-503
• 9 Centers for Disease Control. Classification system for Human-T-Lymphotropic Virus type III/Lymphadenopathy-associated virus infections. MMWR 1986; 35: 334-339
• 10 Ledermann MM, Ratnoff OD, Scillian JJ, Jones PK, Schacter B. Impaired cell-mediated immunity in patients with classic hemophilia. N Engl J Med 1983; 308: 79-83
• 11 Carr R, Veitch SE, Edmond E, Peutherer JF, Prescott RJ, Steel CM, Ludiam CA. Abnormalities of circulating lymphocyte subsets in haemophiliacs in an AIDS-free population. Lancet 1984; i: 1431-1434
• 12 Melbye M, Froebel KS, Madhok R, Biggar RJ, Sarin PS, Stenbjerg S, Lowe GD O, Forbes CD, Goedert JJ, Gallo RC, Ebbesen P. HTLV-III seropositivity in European haemophiliacs exposed to factor VIII concentrate imported from the USA. Lancet 1984; ii: 1444-1446
• 13 Jason J, McDougal JS, Holman RC, Stein SF, Lawrence DN, Nicholson JK A, Dixon G, Doxey M, Evatt BL. And theHaemophilia/ AIDS Collaborative Study Group. Human-T-Lymphotropic retrovirus type III/Lymphadenopathy-associated virus antibody Association with hemophiliacs’ immune status and blood component usage JAMA 1985; 253: 3409-3415
• 14 Stein SF, Evatt BL, McDougal JS, Lawrence DN, Holman RC, Ramsey RB, Spira TJ. A longitudinal study of patients with hemophilia: immunologic correlates of infection with HTLV-III/LAV and other viruses. Blood 1985; 66: 973-979
• 15 de Shazo RD, Daul CB, Andes WA, Bozelka BE. A longitudinal immunologic evaluation of hemophilic patients. Blood 1985; 66: 993-998
• 16 Klatzmann D, Barre-Sinoussi F, Nugeyre MT, Dauguet C, Vilmer E, Griscelli C, Brun-Vezinet F, Rouzioux C, Gluckman JC, Chermann JC, Montagnier L. Selective tropism of lymphadenopathy associated virus (LAV) for helper-induced T lymphocytes. Science 1984; 225: 59-63
• 17 Mathur-Wagh U, Mildvan D, Senie RT. Follow up at 4½ years on homosexual men with generalized lymphadenopathy. N Engl J Med 1985; 313: 1542-1543
• 18 Laurence J, Brun-Vezinet F, Schutzer SE, Rouzioux C, Klatzmann D, Barre-Sinoussi F, Chermann JC, Montagnier L. Lymph-adenopathy-associated viral antibody in AIDS. Immune correlations and definition of a carrier state N Engl J Med 1984; 311: 1269-1273
• 19 Mathur-Wagh U, Enlow RW, Spigland I, Winchester RJ, Sacks HS, Rorat E, Yancovitz SR, Klein MJ, William DC, Mildvan D. Longitudinal study of persistent generalized lymphadenopathy in homosexual men: relation to acquired immune deficiency syndrome. Lancet 1984; i: 1033-1038