Chronic Leukemias and Plasma Cell Disorders Flashcards
What are the 3 main chronic leukemias?
Chronic Lymphocytic Leukemia
Hairy cell leukemia
Adult T cell Leukemia/Lymphoma
Chronic Lymphocytic Leukemia (CLL)
In what parts of the body does it happen?
Involves blood, bone marrow, and lymph nodes
Chronic Lymphocytic Leukemia (CLL)
What patients does this tend to happen in?
Elderly patients
May have immune dysfunction and hypogammaglobulinemia
Chronic Lymphocytic Leukemia (CLL)
Pathogenesis
You get a ton of abnormal B cells that are useless in fighting infection. They suppress normal B cell proliferation.
May also lead to autoimmune hemolytic anemia
Chronic Lymphocytic Leukemia (CLL)
How can you assess prognosis?
Look for mutations in the IGH gene
Those with IGH mutations have a favorable outcome
Chronic Lymphocytic Leukemia (CLL)
What do the cells look like?
Mostly small round lymphocytes with condensed chromatin
May also see Smudge cells due to increased fragility (their appearance is an artifact of the blood smear)
Hairy Cell Leukemia
How do the cells appear?
They have cytoplasmic projections (hair-like)
Hairy Cell Leukemia
What are some usual presenting symptoms?
Pancytopenia
Splenomegaly (red pulp expansion)
Hepatomegaly
Infections
Hairy Cell Leukemia
What cytochemical stain should you use for diagnosis?
TRAP
Tartrate Resistant Acid Phosphatase
Hairy Cell Leukemia
Prognosis
Excellent response to gentle chemo
Adult T Cell Leukemia/Lymphoma
What type of cells tend to be involved? What characteristic appearance will they maintain?
CD4+ T cells (they will have convoluted nuclei with a floret shape)
Adult T Cell Leukemia/Lymphoma
What infection is it commonly associated with?
HTLV-1 infection
What is the most common plasma cell disorder?
MGUS
Monoclonal Gammopathy of Undertermined Significance
Multiple Myeloma
What is the basic pathogenesis?
Monoclonal plasma cell proliferation involving the bone marrow and typically the skeleton at many sites
Multiple Myeloma
What drives the proliferation of plasma cells?
Cytokines, like IL-6
Multiple Myeloma
Diagnostic Criteria
M protein - monoclonal protein in serum or urine (IgG or IgA)
Plasmacytoma (tissue based tumor of plasma cells)
CRAB Hypercalcemia Renal insufficiency Anemia Bone lesions (lytic)
Multiple Myeloma
What lab tests would you run to assess? What would you see in normal? Multiple myeloma?
Serum protein electrophoresis
Normal - smear due to many Abs of many sizes
Multiple Myeloma- one sharp band due to monoclonal expansion
You may run a second step with antibody binding to see the isotype of the heavy and light chains.
Multiple Myeloma
Why does the patient get hypercalcemia?
Bone resorption (also leads to more fractures)
Multiple Myeloma
Why does the patient get infections?
Recurrent infections occur because they are only making one type of Ab
Multiple Myeloma
What is Bence-Jones proteinuria?
Light chains are toxic to the tubular epithelium, eventually lead to proteinuria
Monoclonal Gammopathy of Undertermined Significance (MGUS)
How should you treat them?
Don’t treat them, just follow them over time and analyze with serum protein electrophoresis and renal function assessment. You should begin treatment once the monoclonal protein levels start to increase
