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Chronic Idiopathic Thrombocytopenic Purpura: Title and subTitle BreakA Familial Immunodeficiency Syndrome? FREE

Marie J. Stuart, MB, BS; Russell H. Tomar, MD; Merrill L. Miller, MD; Frederick R. Davey, MD
[+] Author Affiliations

Reprint requests to Department of Pediatrics, State University of New York, Upstate Medical Center, 750 E Adams St, Syracuse, NY 13210 (Dr Stuart).


JAMA. 1978;239(10):939-942. doi:10.1001/jama.1978.03280370035020
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We studied three children with chronic idiopathic thrombocytopenic purpura (ITP) and their immediate families. All three patients and 10/13 family members manifested at least one immunologic defect, eg, decreased numbers of T lymphocytes (1/3, 5/13), diminished in vitro response to phytohemagglutinin (2/3, 6/13), dysgammaglobulinemia (2/3, 4/13), altered autoantibodies (1/3, 5/13), and decreased serum properdin levels (3/3,2/13). In addition, one parent and two asymptomatic siblings of two of the propositi had shortened platelet life spans with normal platelet counts. The HLA antigens A3 and B7 were identified in all three families. There also appeared to be an association between a familial haplotype and the immunologic defects. Chronic ITP appears to occur in families with underlying immunologic defects, which are genetically related.

(JAMA 239:939-942, 1978)

REFERENCES

Clancy RL, Muller HK, Ward HA:  Immunodeficiency and grouping with thyrogastric autominmune disease in patients with chronic idiopathic thrombocytopenic purpura . Aust NZ J Med 4:243-246, 1974;.
Khalifa AS, Lusher JM, Cejka J, et al:  Immunoglobulins in idiopathic thrombocytopenic purpura in childhood . Acta Haematol 56:205-211, 1976;.
Stuart MJ, Murphy S, Oski FA:  A simple nonradioisotope technique for the determination of platelet life span . N Engl J Med 292:1310-1313, 1975;.
Bach JF:  Evaluation of T cells and thymic serum factors in man using the rosette technique . Transplant Rev 16:196-217, 1973;.
Bianco C, Patrick R, Nussenzweig UJ:  A population of lymphocytes bearing a membrane receptor for antigen-antibody-complement complexes . J Exp Med 132:702-720, 1970;.
Oppenheim JJ, Schecter B:  Lymphocyte transformation , in Rose NR, Friedman H (eds): Manual of Clinical Immunology . Washington, DC, American Society for Microbiology, 1976;, pp 81-94.
Bach FH, Voynow NK:  One way stimulation mixed leukocyte cultures . Science 153:545-547, 1966;.
Lightsey AL, McMillan R, Koenig HM, et al:  In vitro production of platelet-binding IgG in childhood idiopathic thrombocytopenic purpura . J Pediatr 88:414-418, 1976;.
McClure PD:  Idiopathic thrombocytopenic purpura in children: Diagnosis and management . Pediatrics 55:68-74, 1975;.

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Clancy RL, Muller HK, Ward HA:  Immunodeficiency and grouping with thyrogastric autominmune disease in patients with chronic idiopathic thrombocytopenic purpura . Aust NZ J Med 4:243-246, 1974;.
Khalifa AS, Lusher JM, Cejka J, et al:  Immunoglobulins in idiopathic thrombocytopenic purpura in childhood . Acta Haematol 56:205-211, 1976;.
Stuart MJ, Murphy S, Oski FA:  A simple nonradioisotope technique for the determination of platelet life span . N Engl J Med 292:1310-1313, 1975;.
Bach JF:  Evaluation of T cells and thymic serum factors in man using the rosette technique . Transplant Rev 16:196-217, 1973;.
Bianco C, Patrick R, Nussenzweig UJ:  A population of lymphocytes bearing a membrane receptor for antigen-antibody-complement complexes . J Exp Med 132:702-720, 1970;.
Oppenheim JJ, Schecter B:  Lymphocyte transformation , in Rose NR, Friedman H (eds): Manual of Clinical Immunology . Washington, DC, American Society for Microbiology, 1976;, pp 81-94.
Bach FH, Voynow NK:  One way stimulation mixed leukocyte cultures . Science 153:545-547, 1966;.
Lightsey AL, McMillan R, Koenig HM, et al:  In vitro production of platelet-binding IgG in childhood idiopathic thrombocytopenic purpura . J Pediatr 88:414-418, 1976;.
McClure PD:  Idiopathic thrombocytopenic purpura in children: Diagnosis and management . Pediatrics 55:68-74, 1975;.
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