Secondary immune deficiencies and HIV-1 infection Flashcards

1
Q

What are causes of secondary immune deficiencies?

A

Malnutrition

Infectious Diseases e.g. HIV

Environmental stress

Age Extremes: Prematurity and old age

Surgery and trauma splenectomy

Immunosuppressive drugs

Genetic and metabolic diseases

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2
Q

What are signs and symptoms of secondary immune deficiencies?

A

Infections: Severe, persistent, recurrent, unusual

Autoimmune conditions (cytopaenias) and allergic disease

Persistent inflammation

Cancer (viral associated EBV, HHV-8)

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3
Q

Which drugs can cause immune deficiencies?

A

Small molecules:

  • Glucocorticoids and mineralocorticoids
  • Cytotoxic agents: Methotrexate, mycophenolate, cyclophosphamide
  • and azathioprine.
  • Calcineurin inhibitors: Cyclosporine and tacrolimus
  • Antiepileptic drugs (phenytoin, carbamazepine, levetiracetam)
  • DMARD (sulphasalazine, leflunomide)

JAK inhibitors:

  • Tofacitinib
  • Upadacitinib
  • Ruxolitinib
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4
Q

Which drug class can cause TB reoccurance?

A

Anti-TNF agents

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5
Q

Which cancers are common with secondary immune deficiencies?

A

B and plasma cell cancers can present with immune deficiency (antibody deficiency syndromes are most common).

Chemotherapy, biological agents, and radiotherapy can leads to loss of immune cells and immune deficiency.

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6
Q

Which specific B-cell lymphoproliferative disorders can arise from secondary immune deficiencies?

A

Multiple myeloma

Chronic lymphocytic leukaemia

Non Hodgkin’s lymphoma

Monoclonal gammopathy of uncertain significance

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7
Q

What is Good’s syndrome?

A

Thymoma and antibody deficiency

Combined T and B cell (absent) defect

CMV PJP and muco-cutaneous candida

Autoimmune disease (Pure red cell aplasia, Myasthenia gravis, Lichen planus)

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8
Q

What are appropriate investigations for secondary immune deficiencies?

A

Full Blood count

Hb < 10g/L

Neutrophil count

Lymphocyte count

Platelet count

Immunoglobulins (IgG, IgA, IgM, IgE )

Serum complement (C3, C4)

HIV test (18-80 years)

Strategy will pick up to 85% of all immune defects

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9
Q

What is serum protein electrophoresis?

A

SPE separation of serum proteins by charge

Detection of discrete bands: monoclonal identified by immunofixation with labelled IgG, IgA, IgM anti-sera

SPE can miss free light chain disease which is seen 20% multiple myeloma cases): hence measurement of free light chains is essential for work up of B cell LPD.

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10
Q

What are second line investigations for secondary immune deficiencies?

A

Measure concentration of vaccine antibodies:

  • Tetanus toxoid: Protein antigen
  • Pneumovax vaccine: Carbohydrate antigen ( all 23 serotypes or to individual pneumococcal serotypes)
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11
Q

What are third line investigations for secondary immune deficiencies?

A

Analysis of naïve and memory T and B cell subsets

Assessment of IgG subclasses

Determination of anti-cytokine and complement antibodies:

  • Anti-Type 1 interferon antibodies (IFN-α and IFN-ω) in SARS-CoV-2 infection
  • Anti Type 2 interferon antibodies (IFN-γ) in disseminated NTM infection
  • Anti-GM-CSF antibodies disseminated in cryptococcal infection
  • Anti-C1 inhibitor antibodies and acquired late onset angioedema ( B cell LPD and SLE)

Genetics (whole exome or whole genome sequencing in cases where it is difficult to work if you are dealing with primary or secondary immune defect

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12
Q

What is the management of secondary immune deficiencies?

A

Treat underlying cause

Advise on measures to reduce exposure to infection

Immunisation against respiratory viruses and bacteria and offer vaccines to household contacts

Education to treat bacterial infections promptly: May require higher and longer therapies courses (co-amoxiclav 625mg TDS for 10-14 days rather then 375mg for 5-7 days)

Prophylactic antibiotics for confirmed recurrent bacterial infection

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