CVID Flashcards
1- confirm onset history by :
2- antibody levels as — , — , — , and — challenge Levels Pre and 4weeks post Pneumovax 23
(Pneumococcal polysacharide Vaccine)
site , onset , frequency , severity , response to antibiotics , unusual paganism and hospital admission
hypogammaglobin:
Primary Immunodeficiency
- — is the most common cause
Secondary Causes
- — Production- more prone to — !
- — Malignancy eg CLL, Myeloma, Lymphoma & Rx
- — eg — & – Depletion (rituximab)
Protein Losses:
- — , —
Decreased production associated with —
CVID:
A Diagnosis of Exclusion
* Low — plus Low– or— + Impaired Specific — Response
* Exclude secondary causes as above- including medications, Lymphoid- Lymphoma & Thymoma
Left untreated CVID may lead to severe and life-threatening infections
Non-Infective Complications
Immune dysregulation, Auto-immunity, Inflammatory Disorders, Malignancy
(lymphoproliferative)
CVID
decreased
infection
haematological
medication as immunosprewssion n b cell depletion
GI and renal
infection
IgG
IgA or IgM
antibody
we use HRCT for — brocheitasis
CVID management:
* Patient education
* —- (remember that serology difficult to interpret)
* Prophylactic antibiotics if required e.g. – , — , —
* Manage — and —
* Prompt treatment of —, (Culture; required longer course of
antibiotics)
* Chest physio if bronchiectasis present
* Regular follow up - monitor infection diary; pulmonary function; imaging
* Occupational advice, incl Vaccines (avoid Live vaccines)
* Genetic considerations
- Immunoglobulin Replacement Therapy (IgRT)
* — Product
* Derived from pooled human plasma from several thousand donors
* Highly purified (generally > — percent) polyvalent IgG with opsonizing and complement-fixing activities
* Treated to inactivate or remove — pathogens
* In hypogammaglobulinemia - provides antibodies against a broad range of pathogens by providing — immunity.
early
imunoglobin replacement
septrin , doxycycline and azirthomycin
rhitinitis and asthma
infection
blood product
>95
blood born
passive
- Patients with severely impaired antibody mediated require lifelong —
- Without adequate IgRT, patients experience recurrent — which
frequently require hospitalisation. - Prompt diagnosis and treatment prior to the onset of complications is compatible with near normal life expectancy.
- choice of administration: iv , subcutaneous , facilitated subcutaneous or push
- frequency: daily push weekly fortmiglty , every 3 weeks
- location : immunology centre , hospital , home
- Remains flexible during different stages of life
and it requires on-going assessment in
partnership with the patient.
IgRT ( replacement therapy )
infection
fSCIG – allows large
volumes of immunoglobulin — to be administered with the pre medication of— enzyme to – the subcutaneous capacity – infusion can be given every – weeks instead of –
subcutaneously
Hyaluronidase
increase
3 weeks instead of weekly
causes of abnormal bleeding:
- –
- —
- —
platelet abnormalities:
* — Production- Bone Marrow, megakaryocytes
* Increased – – Destruction or Consumption
* Platelet —
vascular tissue
platelet
clothing factors
decreased
loss
dysfunction
causes of thrombocytopenia:
- Pseudothrombocytopenia
- Haematological Malignancy
- Infections, including Sepsis/ DIC
- Immune- Inflammatory, autoimmune, immunodeficiency
- Genetic conditions
- Medications
- Alcohol, Liver Disease, Splenomegaly
- Nutritional deficiencies
- Pregnancy associated
- Post Transfusion
in bleeding history we take:
summary:
* Immunodeficiency may present with recurrent common infections in the
community rather than unusual pathogens
* Need to consider pattern of infections – not just the current one
* Frequent delays in Diagnosis of CVID- Bronchiectasis may have been
preventable, Infections can be life threatening
* Immune dysregulation associated with Immunodeficiency leads to the
association with Autoimmunity
* The importance of individualised patient care and MDT input
- Site, Severity, Duration, Spontaneous or Traumatic
