Chapter 21 Immunodeficiencies Flashcards

1
Q

What is immunodeficiency?

A

A rare disorder of the immune system that results in failure to build protection against pathogens and predisposes to recurrent or fatal infections.

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2
Q

What clinical manifestations should alert for the possibility of an immunodeficiency disorder?

A

Recurrent fevers and infections.

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3
Q

What are common clinical signs of immunodeficiency?

A

Fever, inflammatory blood work, system-related inflammatory signs, recurrent pneumonia, sinus infections, pyodermatitis, deep skin or organ abscesses, meningitis, osteomyelitis, prolonged antibiotic therapy, opportunistic infections, failure to gain weight, and familial history of immunodeficiency.

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4
Q

What types of infections are most common in immunodeficient horses?

A

Bacterial infections.

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5
Q

What might accompany immunodeficiency reflecting an imbalance of the immune system?

A

Autoimmunity and neoplasia.

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6
Q

What are primary immunodeficiencies?

A

Congenital processes associated with a genetic hereditary defect, often manifesting in young horses.

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7
Q

What are secondary immunodeficiencies?

A

Acquired disruptions in immune function due to conditions like immunosuppressive treatment, infectious diseases, cancer, metabolic/endocrine diseases, age, and nutritional deficiencies.

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8
Q

What are the five groups of immunodeficiencies based on affected immune system components?

A

Humoral disorders (B cell deficiency), cytotoxic and helper cell deficiencies (T cell deficiency), combined B and T cell deficiencies, phagocytic deficiency, and complement deficiency.

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9
Q

What is transient hypogammaglobulinemia of the young?

A

A delay in endogenous immunoglobulin production by foals, resulting in susceptibility to bacterial infections.

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10
Q

What are typical clinical signs of transient hypogammaglobulinemia in foals?

A

Recurrent infections at 2-3 months of age, requiring antibiotic therapy.

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11
Q

What is agammaglobulinemia in horses?

A

A very rare and fatal immunodeficiency caused by failure in B lymphopoiesis, leading to impaired immunoglobulin production.

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12
Q

What is selective IgM deficiency?

A

A condition where serum IgM concentration is significantly below normal, affecting foals and sometimes adults, leading to recurrent infections.

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13
Q

What is selective IgA deficiency?

A

A common primary immunodeficiency in humans, often asymptomatic, with a possible higher incidence in certain horse cohorts like endurance horses.

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14
Q

What is Foal Immunodeficiency Syndrome (FIS)?

A

An inheritable and fatal condition characterized by profound anemia and septicemia in breeds like the Fell Pony and Dales Pony.

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15
Q

What is common variable immunodeficiency (CVID) in horses?

A

A rare, late-onset, fatal immunologic disorder of B cell depletion and inadequate antibody production, manifesting in adult horses.

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16
Q

What are common clinical signs of CVID?

A

Recurrent infections, pneumonia, sinusitis, meningitis, abnormal gait, peritonitis, gingivitis, weight loss, muscle atrophy, and low serum IgG concentrations.

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17
Q

What is the role of plasma transfusions in managing humoral deficiencies in horses?

A

They help through life-threatening infectious phases, particularly in foals with transient immunodeficiencies.

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18
Q

What is severe combined immunodeficiency (SCID)?

A

An autosomal recessive hereditary condition affecting B and T cell development in Arabian foals, leading to severe immunodeficiency.

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19
Q

What is the typical lifespan of SCID foals?

A

They generally die before five months of age due to severe respiratory infections.

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20
Q

What are common findings in SCID foals?

A

Severe peripheral blood lymphopenia, IgM and IgA deficiency, and low serum IgG concentration reflecting colostrum-derived antibodies.

21
Q

What is the cause of SCID in Arabian foals?

A

A frame-shift mutation in the gene encoding DNA-dependent protein kinase catalytic subunit (DNA-PKc), leading to defective B and T cell receptor formation.

22
Q

What is the treatment for SCID foals?

A

Experimental replacement of B and T cells via bone marrow and thymus transplantation, though challenging and not commonly successful.

23
Q

What are phagocytic deficiencies in horses?

A

Inherited forms are not described, but acquired neutrophil dysfunction can occur during sepsis in neonate foals.

24
Q

What is Pelger-Huët anomaly?

A

A benign, dominantly inherited defect of neutrophil differentiation, presenting dumbbell-shaped bilobed nuclei and a reduced number of nuclear segments.

25
Q

What is the significance of complement deficiencies in horses?

A

Primary complement component diseases are rare, with foals born with low serum complement activity relying on endogenous production after birth.

26
Q

What is the clinical importance of identifying immunodeficiency?

A

Early identification can guide appropriate management and treatment, improving the quality of life and survival of affected horses.

27
Q

What is the significance of lymphocyte proliferation response in immunodeficient foals?

A

Normal lymphocyte proliferation in vitro suggests intact T cell function, despite other immune dysfunctions.

28
Q

What is the role of diagnostic immunologic assays in horses?

A

To improve detection and understanding of various immunodeficiencies, aiding in accurate diagnosis and treatment planning.

29
Q

What are common pathogens involved in infections in immunodeficient horses?

A

Staphylococcus spp., Streptococcus spp., Actinobacillus spp., and Klebsiella spp.

30
Q

What are the effects of delayed antibody production in foals?

A

Increased susceptibility to bacterial infections, requiring continuous or intermittent antibiotic therapy.

31
Q

What are typical laboratory findings in immunodeficient horses?

A

Neutrophilia, hyperfibrinogenemia, low serum IgG concentrations, and abnormal liver enzymes during septicemia.

32
Q

What is the role of genetic testing in managing hereditary immunodeficiencies?

A

Genetic tests can identify carriers and affected horses, guiding breeding decisions to reduce the incidence of these disorders.

33
Q

What are the clinical implications of hypogammaglobulinemia in horses?

A

Increased susceptibility to infections, requiring vigilant monitoring and management to prevent severe outcomes.

34
Q

What is the prognosis for horses with CVID?

A

Generally poor, with many horses being euthanized due to financial reasons or poor quality of life within six months of diagnosis.

35
Q

What is the role of bone marrow transplantation in treating immunodeficiencies?

A

While successful in experimental settings, it is not commonly used due to the complexity and cost of the procedure.

36
Q

What is the significance of low serum IgM concentrations in adult horses?

A

May indicate selective IgM deficiency, often secondary to conditions like lymphosarcoma or immunosuppressive therapy.

37
Q

What is the impact of early detection of immunodeficiency in foals?

A

Early detection allows for timely management and treatment, potentially improving outcomes and preventing severe infections.

38
Q

What are common management strategies for horses with immunodeficiency?

A

Routine physical examination, blood work, continuous or intermittent antibiotic therapy, and avoiding live vaccines.

39
Q

What is the significance of genetic diversity in managing hereditary immunodeficiencies?

A

Maintaining genetic diversity helps prevent the spread of hereditary immunodeficiencies, improving the overall health of the population.

40
Q

What are typical clinical signs of immunodeficiency in equine neonates?

A

Recurrent infections such as pneumonia, diarrhea, osteomyelitis, meningitis, and failure to gain weight or grow properly.

41
Q

What are the clinical challenges in diagnosing immunodeficiencies in horses?

A

Low incidence, poor understanding of clinical signs, and lack of routine diagnostic testing contribute to delayed or missed diagnoses.

42
Q

What is the significance of opportunistic infections in immunodeficient horses?

A

They indicate an impaired immune system, often requiring aggressive and prolonged treatment to manage.

43
Q

What are the long-term management options for immunodeficient horses?

A

Continuous monitoring, supportive care, and sometimes lifelong antibiotic therapy to manage recurrent infections.

44
Q

What is the role of inactivated vaccines in immunodeficient horses?

A

They can provide partial immunity without the risks associated with live vaccines, aiding in disease prevention.

45
Q

What are the clinical signs of severe combined immunodeficiency (SCID) in Arabian foals?

A

Bronchopneumonia non-responsive to antibiotics, Pneumocystis jiroveci pneumonia, adenovirus inclusions, and severe lymphopenia.

46
Q

What is the significance of complement component deficiencies in foals?

A

Low serum complement activity can lead to delayed opsonic capacity and increased susceptibility to infections.

47
Q

What are the implications of lymphocyte immunophenotyping in immunodeficient horses?

A

Helps identify specific immune cell deficiencies, guiding targeted treatment and management strategies.

48
Q

What is the clinical relevance of major histocompatibility complex (MHC) class II expression in foals?

A

Low expression indicates abnormal lymphocyte development and function, commonly seen in immunodeficient foals.

49
Q

What is the significance of persistent absolute lymphopenia in horses?

A

Suggests poor lymphocyte population proliferation and activation, often indicating underlying immunodeficiency.