Pathoma - WBC Disorders - Plasma Cell Disorders, Histiocytosis Flashcards
What is the major neoplastic cell in multiple myeloma
Malignant proliferation of plasma cells (mature B cells) in the bone marrow
What is the interleukin often seen in multiple myeloma
High serum IL-6
Why are lytic lesions seen in multiple myeloma
Plasma cells produce osteoclast activating factor (RANK), leading to bone destruction and thus bone pain with hypercalcemia
Why do you see elevated serum protein in multiple myeloma
Plasma cells produce immunoglobulin
M spike (usually IgG or IgA)
Why is there increased risk of infection in multiple myeloma
Even though plasma cells produced immunoglobulin it is monoclonal antibody (all the same type)
What do you see under microscopy in multiple myeloma
Rouleaux formation of RBCs (stacked) Because increased serum protein decreases charge between RBCs
What are the results of plasma cells producing increased light chain in multiple myeloma
Primary AL amyloidosis (light chains depositing in tissue)
Proteinuria (light chains excreted as Bence Jones protein)
Risk of renal failure (light chains depositing in kidney)
What is Monoclonal Gammopathy of Undetermined Significance (MGUS)
Increased serum protein with M spike with no other features of multiple myeloma 1% will progress to multiple myeloma
What is Waldenstrom macroglobulinemia
B-cell lymphoma with monoclonal IgM production
IgM is big, hence MACROglobulinemia
What is the clinical presentation of Waldenstrom macroglobulinemia
Increased serum protein with M spike
LAD with absent lytic bone lesions
IgM leads to serum hyperviscosity - visual and neuro deficits
What are Langerhans cells and what do they do?
Specialized dendritic cells found predominantly in the skin
Derived from bone marrow monocytes
Present antigen to naive T-cells
Describe the appearance and positive markers of Langerhan cells
Birbeck granules (tennis racket) CD1a+ and S100+
