Diagnostic Criteria and Laboratory Analysis of Primary Immunodeficiency Conditions

Clinical cases of primary immunodeficiency are similar in symptoms and syndromes in the sense that they all feature characteristic anomalies or deficiencies in the immune system in one way or another. Basic diagnostic criteria include suggestive T cell defects, B cell defects, B cell deficiency and T cells (combined), defects without clearly implicating damage to T or B cells and more. Careful diagnostic analysis and laboratory tests are needed to accurately distinguish the causes and types of immunodeficiency conditions. Although complicated but very interesting.

B cell defects are suggestive

A. repeatedly proven bacterial pneumonia, sepsis or meningitis

b. nodular lymphoid hyperplasia

Lack of B cells and suggestive cells (combined immunodeficiency disease - CID)

A. All features mentioned above except chronic mucocutanoeus condadiasis and nodular lymphoid hyperplasia.

b. features of Wiskott-Aldrich syndrome (ear dries, thrombocytopenia and eczema)

c. ataxia-telangiectasia feature

Immunodeficiency is suggestive without clearly involving T or B cell defects

A. Pneumocytis carini pneumonia

b. Eczema is unbearable

c. Ulcerative colitis in infants less than 1 year old

d. Unresolved diarrhea

e. Unexplained haematological deficiency (RBC, WBC, Platelet)

f. Severe general seborrheic dermatitis (Leiner disease) shows C5 deficiency, as often as in combination immunodeficiency diseases.

g. Repeated pyogenic infections are seen in C3 deficiency

Suggestive biochemical defects

A. features combination immunodeficiency with typical bone lesions (adenosine deaminase deficiency)

b. Features of Blackfan-Diamond splastic anemia (nucleoside phosphorylase deficiency)

Suggestive Polymorphonuclear Leukocyte Disorders

A. primary skin infection (if associated with asthma, eczema and rough faeces, think of Buckey's syndrome)

b. Chronic osteomyelitis caused by species of klebsiella or serratia, drain lymph nodes idexx indonesia (chronic granulomatous disease).

Suggestive secondary deficiency

A. Concurrent or previous infection

b. Lymphoid malignancy (chronic lymphatic leukemia, Hodgkin's disease, myeloma).

Laboratory analysis

a.) Genealogy history

b.) General blood analysis (not only the total but absolute number of different leukocytes)

c.) Investigating the relationship of immunity numbers

- concentration of gammaglobulin

- immune serum globulin by Manchini

- Serum protein immunoelectrophoresis

- Different antibody titers, blood group, liter of isohemagglutinin

- secretory immunoglobulins

- Immunoglobulin surface antiserum lymphocytes, characterized by fluoroscein

- EAC-rosella.

d.) Investigation of cellular immunity

- E-rosela:

-reaction of explosive transformation (in non-specific stimulation with FHA, stimulation with antigens, in mixed lymphocyte cultures)

- depression of macrophage migration

- delayed type hypersensitivity reaction (intracutaneous test with 2,4-dinitroftorbenzol, streptokinase, odoriase, antigen, shieck reaction).

e.) special investigation:

- T-helper and T-press functions:

- histochemical determination of adenosine-deaminase activity

- transcobalamin content

f.) Chest x-ray including side position and mediastinal Tomogram to reveal thymus hte

g.) Lymph node biopsy using hysterological and histochemical methods

h.) investigation of the complement system (total praise, factors)

i.) investigation of the function of phagocytosis (killing of opsonized bacteria, tests with blue tetrazolium, cytochemical methods of determining enzyme activity, etc.).

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