PMID- 16011589
OWN - NLM
STAT- MEDLINE
DA  - 20050713
DCOM- 20051114
LR  - 20091021
IS  - 1351-8216 (Print)
IS  - 1351-8216 (Linking)
VI  - 11
IP  - 4
DP  - 2005 Jul
TI  - The effect of clotting factor concentrates on the immune system in HIV-negative
      haemophilics.
PG  - 366-70
AB  - Immune abnormalities have been reported in patients with haemophilia. Although
      infections with HIV and hepatitis viruses contribute to these abnormalities,
      chronic exposure to extraneous proteins in clotting factor concentrates (CFC) may
      also play a role. A number of studies suggest that the degree of immunological
      abnormalities correlates with the amount of intermediate purity CFC administered 
      over time. The purpose of this study was to investigate whether there were
      cellular and humoral immunological abnormalities in haemophilics receiving
      intensive factor replacement therapy with intermediate purity CFC. For this
      purpose 48 severe haemophilics and 33 healthy controls were enrolled in this
      study. T and B lymphocytes, CD4+ and CD8+ cell counts, CD4/CD8 ratio, natural
      killer cells, active T cells were studied in prophylaxis group, on-demand therapy
      group and healthy controls. In the percentages and absolute counts of lymphocyte 
      subgroups, no significant difference was found between three groups. We also
      investigated serum antitetanus IgG levels in these 48 haemophilics and the
      controls to evaluate the specific antibody response. Antitetanus IgG levels were 
      significantly lower in haemophilics compared to healthy controls (P < 0.001).
      Additionally we evaluated the response to tuberculin skin test in 45 of 48
      haemophilics vaccinated with BCG. The response to PPD test was significantly
      lower in haemophilics compared to the controls (P = 0.037). There was no response
      to tuberculin test, which is the best marker of delayed type hypersensitivity
      (DTH) reactions in 24% of haemophilics. In conclusion, although there was no
      significant change in the ratio of CD4/CD8 and lymphocyte subgroups, specific
      antibody responses and DTH tests were partially impaired in haemophilic patients 
      receiving intermediate purity CFC.
AD  - Department of Pediatric Hematology, Ege University School of Medicine, Izmir,
      Turkey. cbalkan@med.ege.edu.tr
FAU - Balkan, C
AU  - Balkan C
FAU - Kavakli, K
AU  - Kavakli K
FAU - Kutukculer, N
AU  - Kutukculer N
FAU - Aksu, G
AU  - Aksu G
FAU - Yilmaz, D
AU  - Yilmaz D
FAU - Aydinok, Y
AU  - Aydinok Y
LA  - eng
PT  - Journal Article
PL  - England
TA  - Haemophilia
JT  - Haemophilia : the official journal of the World Federation of Hemophilia
JID - 9442916
RN  - 0 (BCG Vaccine)
RN  - 0 (Blood Coagulation Factors)
RN  - 0 (Immunoglobulin G)
SB  - IM
MH  - Adolescent
MH  - Antibody Formation/immunology
MH  - BCG Vaccine/immunology
MH  - Blood Coagulation Factors/*adverse effects
MH  - HIV Seronegativity/*immunology
MH  - Hemophilia A/drug therapy/*immunology
MH  - Hemophilia B/drug therapy/*immunology
MH  - Humans
MH  - Hypersensitivity, Delayed/immunology
MH  - Immunity, Cellular/immunology
MH  - Immunoglobulin G/blood
MH  - Lymphocyte Count/methods
MH  - Tetanus/immunology
MH  - Tuberculin Test/methods
EDAT- 2005/07/14 09:00
MHDA- 2005/11/15 09:00
CRDT- 2005/07/14 09:00
AID - HAE1110 [pii]
AID - 10.1111/j.1365-2516.2005.01110.x [doi]
PST - ppublish
SO  - Haemophilia. 2005 Jul;11(4):366-70.