IHO Disease Flashcards
HLA DR3/Dr4
increased risk of DM1
Heinz body -
what is it ?
what is it seen in (2)?
oxidized hemogobin w/in RBC
seen in G6P dehydrogenase deficiency and thallasemia
Red urine w/out RBC
hemoglobinuria
nuclear elements in RBC
Holly Jolly bodies, seen post splenectomy
Seronegative Spondyloarthropathies - Arthritis w/out rheumatoid factor - no anti Ig Ab (4)
PAIR - HLA B27 associated
Psoriatic Arthritis - joint pain w/psoriasis
Ankylosing spondylitis - bamboo spine
Inflammatory Bowl Disease - Crohns and UC
Reactive Arthritis(Reiter Syndrome) - Conjunctivitis, Urethritis, Arthritis
Primary Amyloidosis - seen and associated w/
AL systemic deposition
-Ig light chain derived
Plasma cell dyscrasias - multiple myelinoma
Secondary Amyloidosis - seen and associated w/
SAA -> AA systemic deposition
-acute phase reactant increased in chronic inflam states and Familal Mediterranium Fever
Nephrotic syndrome - kidney most common
Restrictive cardiomyopathy
Tounge enlargement, malabsorbtion, arrhythmia
Senile Cardiac amyloidosis
serum non-mutated transthyretin in heart, usually asymptomatic
>80 yrs
Familal amyloid cardiomyopathy
serum mutated transthyretin deposits -> restrictive cardiomyopathy
African americans
Alzheimer disease amyloid type and who has higher risk?
A beta
depostis in brain forming plaques
Beta APP on chromosome 21 making down at increased risk
Dialysis associated
beta2 microglobulin
not filtered well and builds
Medullary Carcinoma
calcitonin deposits within tumor
tumor cell in amyloid background
hemolytic anemia, pancytopenia and venous thrombosis think of?
More often seen?
Paroxysmal nocturnal hemoglobinuria
Seen in the morning
Paroxysmal nocturnal hemoglobinuria due to ?
increased complement mediated RBC lysis
-missing decay accelerating factors (CD55/59)
or
- decreased synth of GPI anchor (PIG-A)