| Type | Deposited Protein | Clinical Associations |
| Systemic | Immunoglobulin lambda chains | Multiple myeloma |
| Transthyretin | Familial amyloidosis, senile cardiac amyloidosis | |
| Amyloidosis – A protein | Inflammation-associated amyloidosis | |
| Beta2 -microglobulin | Dialysis-related amyloidosis | |
| Immunoglobulin heavy chains | Systemic amyloidosis | |
| Hereditary | Fibrinogen alpha chain, Apo A1 and Apo A2 | Familial systemic amyloidosis |
| Lysozyme | Familial systemic amyloidosis | |
| Central nervous system | Beta protein precursor | Alzheimer's |
| Prion protein | Creutzfeldt-Jakob disease, Gerstmann-Strãussler-Scheinker disease, fatal familial insomnia | |
| Cystatin C | hereditary cerebral hemorrhage with amyloidosis (Icelandic) | |
| Ocular | Gelsolin | Familial amyloidosis (Finnish) |
| Lactoferrin | Familial corneal amyloidosis | |
| Keratoepithelin | Familial corneal dystrophies | |
| Localized | Calcitonin | Thyroid Carcinoma (Medullary) |
| Amylin | Insulinoma, type 2 diabetes | |
| Atrial natriuretic factor amyloid | Isolated atrial amyloidosis | |
| Prolactin | Pituitary amyloid | |
| Keratin | Cutaneous amyloidosis | |
| Medin | Aortic amyloidosis in elderly people |
Showing posts with label Immunolgy. Show all posts
Showing posts with label Immunolgy. Show all posts
Primary Amyloidosis- Types, proteins and clinical associations
Churg-Strauss Syndrome (CSS)
CSS was first described in 1951 by Jacob Churg and Lotte Strauss as a symptom complex with asthma, eosinophilia and vasculitis. CSS resembles polyarteritis nodosa (PAN) in many aspects. CSS however has characteristic granulomas while PAN does not.(Hence also called allergic granulomatosis).
CSS occurs in middle aged individuals. They present with new or worse asthma. Asthma can start many years before the other symptoms.
Marked by eosinophilia then follows. After that there is the phase vasculitis. Vasculitis can involve the lungs, skin, nerves, kidneys and sometimes other body organs.
CSS occurs in middle aged individuals. They present with new or worse asthma. Asthma can start many years before the other symptoms.
Marked by eosinophilia then follows. After that there is the phase vasculitis. Vasculitis can involve the lungs, skin, nerves, kidneys and sometimes other body organs.
The Indirect Coombs Test or Indirect Antiglobulin Test
Indirect Coombs test (also known as the indirect antiglobulin test or IAT) is used to detect very low concentrations of antibodies present in a patient's plasma/serum prior to a blood transfusion.
The IAT is a two-stage test.
Stage 1 : Test RBCs washed and incubated with a test serum. If the serum contains antibodies to antigens on the RBC surface, the antibodies will bind onto the surface of the RBCs.
Stage 2: RBCs are then washed well with saline and then incubated with antihuman globulin. If antibodies have bound to RBC surface antigens in the first stage, RBCs will agglutinate when incubated with the antihuman globulin and the indirect Coombs test will be positive. By diluting the serum different titrations can be measured.
Most common uses of the IAT are : 1) To screen pregnant women for antibodies that may cause hemolytic disease of the newborn. 2) To test donor blood for compatibility.
The IAT is a two-stage test.
Stage 1 : Test RBCs washed and incubated with a test serum. If the serum contains antibodies to antigens on the RBC surface, the antibodies will bind onto the surface of the RBCs.
Stage 2: RBCs are then washed well with saline and then incubated with antihuman globulin. If antibodies have bound to RBC surface antigens in the first stage, RBCs will agglutinate when incubated with the antihuman globulin and the indirect Coombs test will be positive. By diluting the serum different titrations can be measured.
Most common uses of the IAT are : 1) To screen pregnant women for antibodies that may cause hemolytic disease of the newborn. 2) To test donor blood for compatibility.
Direct Coombs Test Or Direct Antiglobulin Test
The direct Coombs test (also known as the direct antiglobulin test) is used to detect antibodies on the RBC surface. A positive Coombs test indicates that an immune mechanism is attacking the patient's own RBC's. This mechanism could be autoimmunity, alloimmunity or a drug-induced immune-mediated mechanism.
The patient's RBCs are washed and then incubated with antihuman globulin. If immunoglobulin or complement factors have been fixed on to the RBC surface in-vivo, the antihuman globulin will agglutinate the RBCs and the direct Coombs test will be positive.
Examples of diseases that give a positive DAT: Rh isoiimunization, ABO disease, autoimmune hemolytic anemis (warm and cold type) and drug induced hemolysis.
The patient's RBCs are washed and then incubated with antihuman globulin. If immunoglobulin or complement factors have been fixed on to the RBC surface in-vivo, the antihuman globulin will agglutinate the RBCs and the direct Coombs test will be positive.
Examples of diseases that give a positive DAT: Rh isoiimunization, ABO disease, autoimmune hemolytic anemis (warm and cold type) and drug induced hemolysis.
SCID (Severe Combined Immunodeficiency)
Adenosine Deaminase Deficiency causes accumulation of adenosine in T-lymphocytes causing them to malfunction and lead to of the types of an immunodeficiency condition called SCID (Severe Combined Immunodeficiency).
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