Plasma cell disorders (dyscrasias) Flashcards
Which cell proliferates in multiple myeloma?
plasma cells in the bone marrow
What may be found in the serum of someone with multiple myeloma?
IL6 which stimulates plasma cell growth and immunoglobulin production
Clinical features of multiple myeloma
Bone pain with hypercalcemia Elevated serum protein Increased risk of infection Rouleaux formation Primary AL amyloidosis Proteinuria
Pathophys of bone pain in multiple myeloma?
the neoplastic plasma cells activate RNK receptor on osteoclasts –> bone destruction, punched out bone lesions, increased risk of fracture
What will you see on XRAY of someone with multiple myeloma?
punched out lesions of bone
What will you see on serum electrophoresis of someone with multiple myeloma and why?
M spike -represents monoclonal IgG or igA produced by neoplastic plasma cells
Why will someone with multiple myeloma have increased risk of susceptibility to infection?
Monoclonal antibody production lacks antigenic diversity
What will you see on blood smear of someone with multiple myeloma and why?
Rouleaux formation of RBCs - increased protein that decreases charge of RBCs
Why may someone have primary AL amyloidosis in multiple myeloma?
free light chains circulate in serum and deposit in tissue
What effect will multiple myeloma potentially have on the kidney?
free light chains excreted in urine as benze jones protein may deposit in tubules and lead to risk of renal faliure
Explain monoclonal gammopathy of undetermined significant (MGUS)
increased serum protein with M spike without other features of multiple myeloma
Who is the common population to get MGUS
elderly
complication of MGUS
May progress to multiple myeloma
What will you see on electrophoresis of MGUS?
M spike
What is over produced in Waldenstrom macroglobulinemia?
igM
Clinical features of waldenstrom macroglobulinemia
Generalized lymphadenopathy
Increased serum IgM (M spike)
Visual and neurologic deficits – from hyperviscosity
Bleeding -viscous serum causes defective platelet aggregation
How to treat acute complications waldenstrom macroglobulinemia?
plasmapheresis which removes IgM
What are the types of langherhans cell histiocytosis?
Letterer-siwe disease
Eosinophilic granuloma
Hand-Schuller-Christian disease
Where are langherhans cells primarily found?
the skin
What is the characteristic finding in langherhans cells histiocytosis on EM?
Birbeck granules
What do the cells of langherhans cells histiocytosis stain positive for?
CD1a+ and S-100+
Explain letterer-siwe disease
Malignant proliferation of langherhans clls
Classically presents as skin rash and cystic skeletal defects, rapidly fatal
children <2 years old
Explain eosinophilic granuloma
Benign proliferation of langerhans cells in bone
Classic presentation is pathological fracture (does not involve skin)
Explain Hand-Schuller-Christian disease
Malignant proliferation of langherhans cells that presents with scalp rash, lytic skull defects, diabetes insipidus and exopthalamus in child >3
