Abnormalities of the peripheral blood as a presenting feature of immunodeficiency

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

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

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
Volume4
Issue number1
DOIs
StatePublished - Jan 1 1978

Fingerprint

Thrombocytopenia
Immune System
IgA Deficiency
Dinitrochlorobenzene
Autoimmune Hemolytic Anemia
Idiopathic Thrombocytopenic Purpura
Dentition
Mycoses
Hemolytic Anemia
Virus Diseases
Hemolysis
Bacterial Infections
Autoantibodies
Immunoglobulin A
Immunoglobulin M
B-Lymphocytes
Blood Platelets
Immunoglobulin G
Joints
T-Lymphocytes

Keywords

  • autoimmune hemolytic anemia
  • immunodeficiency
  • thrombocytopenic purpura

ASJC Scopus subject areas

  • Hematology

Cite this

Blanchette, V. S., Hallett, J. J., Hemphill, J. M., Winkelstein, J. A., & Zinkham, W. H. (1978). Abnormalities of the peripheral blood as a presenting feature of immunodeficiency. American Journal of Hematology, 4(1), 87-92. https://doi.org/10.1002/ajh.2830040111

Abnormalities of the peripheral blood as a presenting feature of immunodeficiency. / Blanchette, Victor S.; Hallett, Jospeh J.; Hemphill, John Michael; Winkelstein, Jerry A.; Zinkham, William H.

In: American Journal of Hematology, Vol. 4, No. 1, 01.01.1978, p. 87-92.

Research output: Contribution to journalArticle

Blanchette, VS, Hallett, JJ, Hemphill, JM, Winkelstein, JA & Zinkham, WH 1978, 'Abnormalities of the peripheral blood as a presenting feature of immunodeficiency', American Journal of Hematology, vol. 4, no. 1, pp. 87-92. https://doi.org/10.1002/ajh.2830040111
Blanchette, Victor S. ; Hallett, Jospeh J. ; Hemphill, John Michael ; Winkelstein, Jerry A. ; Zinkham, William H. / Abnormalities of the peripheral blood as a presenting feature of immunodeficiency. In: American Journal of Hematology. 1978 ; Vol. 4, No. 1. pp. 87-92.
@article{a8b2bec5bc1e4eec9f4fdcb80c6db631,
title = "Abnormalities of the peripheral blood as a presenting feature of immunodeficiency",
abstract = "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.",
keywords = "autoimmune hemolytic anemia, immunodeficiency, thrombocytopenic purpura",
author = "Blanchette, {Victor S.} and Hallett, {Jospeh J.} and Hemphill, {John Michael} and Winkelstein, {Jerry A.} and Zinkham, {William H.}",
year = "1978",
month = "1",
day = "1",
doi = "10.1002/ajh.2830040111",
language = "English (US)",
volume = "4",
pages = "87--92",
journal = "American Journal of Hematology",
issn = "0361-8609",
publisher = "Wiley-Liss Inc.",
number = "1",

}

TY - JOUR

T1 - Abnormalities of the peripheral blood as a presenting feature of immunodeficiency

AU - Blanchette, Victor S.

AU - Hallett, Jospeh J.

AU - Hemphill, John Michael

AU - Winkelstein, Jerry A.

AU - Zinkham, William H.

PY - 1978/1/1

Y1 - 1978/1/1

N2 - 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.

AB - 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.

KW - autoimmune hemolytic anemia

KW - immunodeficiency

KW - thrombocytopenic purpura

UR - http://www.scopus.com/inward/record.url?scp=0018140215&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0018140215&partnerID=8YFLogxK

U2 - 10.1002/ajh.2830040111

DO - 10.1002/ajh.2830040111

M3 - Article

VL - 4

SP - 87

EP - 92

JO - American Journal of Hematology

JF - American Journal of Hematology

SN - 0361-8609

IS - 1

ER -