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10 Cards in this Set

  • Front
  • Back

AA amyloidosis

Chronic inflammatory disease eg RA, bronchiectasis, FMF, leprosy


Hepatosplenomegaly


Malabsorption


CCF with low QRS voltage and exquisite sensitivity to digitalis


Proteinuria


Amyloid deposit on the skin


Peripheral neuropathy


Amyloid-induced synovitis



Diagnose: abdominal fat biopsy or rectal biopsy (positive Congo red stain)

Hyperviscosity syndrome

Bleeding + visual disturbance + neurological symptoms



Waldenstroom macroglobulinemia


Polycythemia rubra Vera


Multiple myeloma


Leukaemia

Hb electrophoresis

Cannot detect alpha thalassemia (associated with hydrops fetalis)

Multiple myeloma

When to treat:


- paraprotein IgG >30


- any of C R A B (skeletal survey)



Treatment: thalidomide + dexamethasone + bortezomib

Wiskott Aldrich syndrome

X-linked


g


Reduced B cells and IgthrombocytopeniaEczema


thrombocytopenia


Eczema

Iron overload

Grey skin


Early heart failure


Diabetes



SE of desferrioxamine: high frequency deafness and retinopathy

Pentad of TTP

Confusion


Pyrexia


Kidney disease


Thrombocytopenia


Microangiopathic haemolytic anaemia



Plasma exchange


CONTRAINDICATION: platelet transfusion

PNH

Budd chiari syndrome


Pancytopenia (platelets tend to agglutinate)


Red urine in the morning



Flow cytometry CD55 and CD59

Von Willebrand disease

Desmopressin can be used to stimulate vWF release from endothelial cells

Anaphylaxis in blood transfusion

IgA deficiency - therefore developed anti-IgA antibodies