Abnormalities of the peripheral blood as a presenting feature of immunodeficiency

Victor S. Blanchette, Jospeh J. Hallett, J. Michael Hemphill, Jerry A. Winkelstein, William H. Zinkham

Research output: Contribution to journalArticlepeer-review

4 Scopus citations


The predisposition of immunodeficient patients to the development of peripheral blood abnormalities is well documented. Less often recognized is the presence of immune defects in patients who initially present with “idiopathic” forms of thrombocytopenic purpura or autoimmune hemolytic anemia in the absence of symptomatology indicative of an immunodeficiency. In this report we describe two children, each of whom had intermittent and at times severe hemolysis and thrombocytopenia secondary to formation of autoantibodies. Although the initial clinical impression was the presence of an “idiopathic” form of platelet or red cell destructive phenomenon, subsequent observations demonstrated abnormalities of the immune system which affected both B and T lymphocytes. In one of the patients there was a marked deficiency of IgA with a moderate but progressive decrease in IgM and IgG. The other patient had a moderate decrease in IgA and a failure to sensitize to dinitrochlorobenzene. Both exhibited dysmorphic features, including small stature, abnormal dentition, hyperelasticity of the skin, and hyperextensibility of the joints. Neither had significant problems with bacterial, fungal, or viral infections. These observations highlight the importance of evaluating the immune system in children with “idiopathic” forms of hemolytic anemia or thrombocytopenia. Demonstration of these abnormalities can have important therapeutic implications and may also provide information regarding the etiology of the blood abnormalities.

Original languageEnglish (US)
Pages (from-to)87-92
Number of pages6
JournalAmerican Journal of Hematology
Issue number1
StatePublished - 1978


  • autoimmune hemolytic anemia
  • immunodeficiency
  • thrombocytopenic purpura

ASJC Scopus subject areas

  • Hematology


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